Materiały źródłowe

• #1 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The right upper PV draining into the SVC is the commonest type of PAPVC, which is usually associated with ASD, most commonly sinus venosus type (SV-ASD). […] Amongst the spectrum of PAPVC, left-sided anomalous connections have been reported in nearly 18.2% of cases. […] Similarly to TAPVC, mixed connections are reported in the PAPVC as well, where at least one PV from each side drains into a different venous compartment.

• #2

  https://www.polradiol.com/Partial-anomalous-pulmonary-venous-connection-state-of-the-art-review-with-assessment,146849,0,2.html

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #3 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  PAPVC is also a relatively uncommon congenital anomaly, found in only 0.5-0.7% of the general population. […] Anomalous pulmonary venous connections are classified on the basis of the location of pulmonary venous drainage as one of four types: supracardiac, cardiac, infracardiac, or mixed. […] In case report 1, we describe an infracardiac pulmonary drainage known as scimitar syndrome, in which part of the right lung is drained by a pulmonary vein connecting to the inferior vena cava. […] Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic. Some authors have suggested that PAPVC becomes clinically significant only when 50% or more of the pulmonary blood flow returns anomalously. […] Imaging techniques, especially CT and MRI, have a pivotal role in the accurate characterization of these abnormalities, predicting outcomes and establishing appropriate preoperative planning.

• #4 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #5 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] The embryologic origin of left sided PAPVR typically starts around postnatal day 32, due to malregression of the primordial pulmonary trunk and the primitive posterior left atrium, along with concomitant maldevelopment of the sinus venosus, and the left common cardinal and right vitelline veins. […] Left sided partial anomalous pulmonary venous return of all three left pulmonary veins to the coronary sinus is a rare anatomic anomaly thoughtful surgical correction is required when treating these unique PAPVR lesions. […] Partial anomalous pulmonary venous return (PAPVR) of all left pulmonary veins draining into the coronary sinus is a very rare congenital lesion discovered in adulthood, and can be treated with simple repair by coronary sinus unroofing and patch closure of atrial septal closure.

• #6 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all (total form) or some (partial form) pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium. […] The development of the pulmonary veins starts early in embryonic life and involves several complicated processes. Pulmonary venous developmental anomalies occur if any of these processes fails to progress properly. Thus, abnormal venous pulmonary drainage may be partial (PAPVC) in cases where only part of the pulmonary venous anatomy is abnormal, or it can involve all the pulmonary veins (TAPVC), resulting in complete drainage of the pulmonary venous return into the systemic venous circulation. […] TAPVC, as in case report 3, accounts for approximately 1-5% of congenital cardiovascular anomalies. This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation.

• #7 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  We know that the pulmonary veins originate from the posterior aspect of the left atrium. Meanwhile, the lung buds that arise from the lung parenchyma canalize as a vessel and gradually connect to the developing pulmonary veins. Some theories say that the lung buds are initially enmeshed in the splanchnic plexus which drains into the cardinal and umbilical vitelline veins (systemic venous system). By week 4 of gestation, the pulmonary veins from the left atrium connects with the superior portion of the splanchnic plexus to form the pulmonary plexus and ultimately loses its connection with the splanchnic plexus. The pulmonary vein is then supposed to divide into 4 branches, 2 on right and 2 on left, each with an orifice at the left atrium. Failure of one or more of the pulmonary veins to separate from the systemic venous systemic results in PAPVC/TAPVC.

• #8 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  We know that the pulmonary veins originate from the posterior aspect of the left atrium. Meanwhile, the lung buds that arise from the lung parenchyma canalize as a vessel and gradually connect to the developing pulmonary veins. Some theories say that the lung buds are initially enmeshed in the splanchnic plexus which drains into the cardinal and umbilical vitelline veins (systemic venous system). By week 4 of gestation, the pulmonary veins from the left atrium connects with the superior portion of the splanchnic plexus to form the pulmonary plexus and ultimately loses its connection with the splanchnic plexus. The pulmonary vein is then supposed to divide into 4 branches, 2 on right and 2 on left, each with an orifice at the left atrium. Failure of one or more of the pulmonary veins to separate from the systemic venous systemic results in PAPVC/TAPVC.

• #9 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #10 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #11 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  In the occurrence of Partial Anomalous Pulmonary Venous Return (PAPVR), a failure of this separation process results in one or multiple pulmonary veins draining into an inappropriate structure. […] The most common presentation of PAPVR involves the right upper pulmonary vein and drains into the junction of the superior vena cava (SVC) and right atrium, creating a left-to-right shunt, and there is almost always an associated Atrial Septal Defect (ASD) present. […] The estimated overall incidence of PAPVR is 0.5 %. […] To further understand the etiology of PAPVR, the embryological development of the SVC must also be understood. […] The SVC is derived from the proximal portion of the right anterior cardinal vein and the right common cardinal vein at a point that is caudal to the transverse anastomosis within the embryo.

• #12 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  In the occurrence of Partial Anomalous Pulmonary Venous Return (PAPVR), a failure of this separation process results in one or multiple pulmonary veins draining into an inappropriate structure. […] The most common presentation of PAPVR involves the right upper pulmonary vein and drains into the junction of the superior vena cava (SVC) and right atrium, creating a left-to-right shunt, and there is almost always an associated Atrial Septal Defect (ASD) present. […] The estimated overall incidence of PAPVR is 0.5 %. […] To further understand the etiology of PAPVR, the embryological development of the SVC must also be understood. […] The SVC is derived from the proximal portion of the right anterior cardinal vein and the right common cardinal vein at a point that is caudal to the transverse anastomosis within the embryo.

• #13 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #14 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-connection-an-articulo-S2174204916302367

  TAPVC, as in case report 3, accounts for approximately 1-5% of congenital cardiovascular anomalies.2 This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation. […] PAPVC is also a relatively uncommon congenital anomaly, found in only 0.50.7% of the general population.4 Anomalies in veins from the right lung are twice as common as from the left lung. […] Anomalous pulmonary venous connections are classified on the basis of the location of pulmonary venous drainage as one of four types: supracardiac, cardiac, infracardiac, or mixed. […] In case report 1, we describe an infracardiac pulmonary drainage known as scimitar syndrome, in which part of the right lung is drained by a pulmonary vein connecting to the inferior vena cava.

• #15 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-connection-an-articulo-S2174204916302367

  TAPVC, as in case report 3, accounts for approximately 1-5% of congenital cardiovascular anomalies.2 This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation. […] PAPVC is also a relatively uncommon congenital anomaly, found in only 0.50.7% of the general population.4 Anomalies in veins from the right lung are twice as common as from the left lung. […] Anomalous pulmonary venous connections are classified on the basis of the location of pulmonary venous drainage as one of four types: supracardiac, cardiac, infracardiac, or mixed. […] In case report 1, we describe an infracardiac pulmonary drainage known as scimitar syndrome, in which part of the right lung is drained by a pulmonary vein connecting to the inferior vena cava.

• #16 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The right upper PV draining into the SVC is the commonest type of PAPVC, which is usually associated with ASD, most commonly sinus venosus type (SV-ASD). […] Amongst the spectrum of PAPVC, left-sided anomalous connections have been reported in nearly 18.2% of cases. […] Similarly to TAPVC, mixed connections are reported in the PAPVC as well, where at least one PV from each side drains into a different venous compartment.

• #17 Incidental radiological finding of partial anomalous pulmonary vein drainage | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-incidental-radiological-finding-partial-anomalous-articulo-S157921292100001X

  Partial anomalous pulmonary venous connection (PAPVC) is an unusual congenital vascular disease that is often diagnosed incidentally. It consists of the connection of one (or more) pulmonary veins to a systemic vein, causing left-to-right shunt. It is usually asymptomatic, but when associated with other malformations or if the shunt is significant, it may sometimes cause severe pulmonary hypertension. […] The most common form of PAPVC is drainage from the right upper lobe vein to the superior vena cava; this form is usually associated in 80% of cases with sinus venosus atrial septal defect (ASD). […] The CT scan showed a PAPVC involving drainage from the right upper lobe, the middle lobe, and some segments of the right lower lobe to the superior vena cava, which resulted in dilation of right heart cavities and signs of precapillary pulmonary hypertension. […] Our case is interesting because of the patient’s age at presentation (despite significant PAPVC) and the absence of concomitant venous sinus ASD.

• #18 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The right upper PV draining into the SVC is the commonest type of PAPVC, which is usually associated with ASD, most commonly sinus venosus type (SV-ASD). […] Amongst the spectrum of PAPVC, left-sided anomalous connections have been reported in nearly 18.2% of cases. […] Similarly to TAPVC, mixed connections are reported in the PAPVC as well, where at least one PV from each side drains into a different venous compartment.

• #19 Anomalous pulmonary venous drainage: a pictorial essay with a CT focus | Journal of Congenital Cardiology | Full Text

  https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-017-0008-4

  Partial anomalous pulmonary venous drainage (PAPVD) describes the connection of at least one pulmonary vein, but not all, to the systemic venous system or right atrium (RA). Drainage of all pulmonary veins outside the left atrium is termed total anomalous pulmonary venous drainage (TAPVD). […] The prevalence of PAPVD has been reported to be between 0.40.7%. It is more common on the right and results in the formation of a left-to-right shunt. […] Right-sided anomalous drainage may occur into any of the central veins. The most common form of PAPVD is the anomalous drainage of the RSPV into the superior vena cava (SVC). […] Up to 18.2% of PAPVD cases affect the left-sided pulmonary veins. The commonest pattern on the left is the LSPV connected to the left brachiocephalic vein. […] Careful scrutiny of the pulmonary veins is important and the identification of less than four pulmonary veins connected to the left atrium should alert the reader to a diagnosis of PAPVD.

• #20 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #21 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=staging.radpair.com&lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. […] The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen. […] More rarely it is seen with ostium primum defect, a subtype of atrioventricular defects.

• #22 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #23 Partial Anomalous Pulmonary Venous Return (PAPVR)

  https://my.clevelandclinic.org/health/diseases/24580-partial-anomalous-pulmonary-venous-return

  PAPVR creates a left-to-right cardiac shunt, or an atypical pattern of blood flow through your heart. Partial anomalous means some (but not all) of the oxygen-rich blood from your lungs doesn’t return to your heart in the normal way. […] This means two things are happening that shouldn’t. First, some oxygen-rich blood unnecessarily returns to your lungs, where it just came from. Second, your right ventricle has a higher load of blood it must pump to your lungs. This extra work can take a toll on your heart over time. […] PAPVR, like other forms of congenital heart disease, happens when a fetal heart doesn’t form properly in the uterus. […] Normally, during the early weeks of pregnancy, four pulmonary veins form out of a common pulmonary vein. These veins connect with the fetal left atrium. However, changes in this process cause the pulmonary veins to connect to the right atrium or nearby veins (like the superior vena cava or left brachiocephalic vein).

• #24 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. As the name suggests, in PAPVC, the blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium. Usually, a single pulmonary vein is anomalous. Rarely, all the veins from one lung are anomalous. Thus, some of the pulmonary venous flow enters the systemic venous circulation. […] Numerous factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs). The shunt magnitude, expressed as the Qp:Qs ratio, and other factors determine development of symptoms and complications in partial anomalous pulmonary venous connection (PAPVC). […] The most important factor is the number of pulmonary veins that drain into the systemic circulation. The more veins that anomalously drain, the more blood returns to the right side of the heart. Some authors have suggested that this defect becomes clinically significant when 50% or more of the pulmonary veins anomalously return.

• #25 Partial Anomalous Pulmonary Venous Return (PAPVR)

  https://my.clevelandclinic.org/health/diseases/24580-partial-anomalous-pulmonary-venous-return

  PAPVR creates a left-to-right cardiac shunt, or an atypical pattern of blood flow through your heart. Partial anomalous means some (but not all) of the oxygen-rich blood from your lungs doesn’t return to your heart in the normal way. […] This means two things are happening that shouldn’t. First, some oxygen-rich blood unnecessarily returns to your lungs, where it just came from. Second, your right ventricle has a higher load of blood it must pump to your lungs. This extra work can take a toll on your heart over time. […] PAPVR, like other forms of congenital heart disease, happens when a fetal heart doesn’t form properly in the uterus. […] Normally, during the early weeks of pregnancy, four pulmonary veins form out of a common pulmonary vein. These veins connect with the fetal left atrium. However, changes in this process cause the pulmonary veins to connect to the right atrium or nearby veins (like the superior vena cava or left brachiocephalic vein).

• #26 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #27 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The right upper PV draining into the SVC is the commonest type of PAPVC, which is usually associated with ASD, most commonly sinus venosus type (SV-ASD). […] Amongst the spectrum of PAPVC, left-sided anomalous connections have been reported in nearly 18.2% of cases. […] Similarly to TAPVC, mixed connections are reported in the PAPVC as well, where at least one PV from each side drains into a different venous compartment.

• #28 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #29 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. As the name suggests, in PAPVC, the blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium. Usually, a single pulmonary vein is anomalous. Rarely, all the veins from one lung are anomalous. Thus, some of the pulmonary venous flow enters the systemic venous circulation. […] Numerous factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs). The shunt magnitude, expressed as the Qp:Qs ratio, and other factors determine development of symptoms and complications in partial anomalous pulmonary venous connection (PAPVC). […] The most important factor is the number of pulmonary veins that drain into the systemic circulation. The more veins that anomalously drain, the more blood returns to the right side of the heart. Some authors have suggested that this defect becomes clinically significant when 50% or more of the pulmonary veins anomalously return.

• #30 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. If only a portion of the common pulmonary vein, either right or left, becomes atretic, persisting pulmonary-systemic venous connections form PAPVC. […] The main physiologic manifestation of PAPVC is similar to atrial septal defect (ASD), which is left to right shunt at the atrial level leading to recirculation of oxygenated blood through the pulmonary vasculature. The increased blood to the left side leads to dilatation of the right atrium and ventricle and increased pulmonary blood flow. The factors that determine the magnitude of left to right shunting include the number and size of the anomalous veins, the site of origin of the anomalous veins, and the size of ASD and the pulmonary vascular resistance.

• #31 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. As the name suggests, in PAPVC, the blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium. Usually, a single pulmonary vein is anomalous. Rarely, all the veins from one lung are anomalous. Thus, some of the pulmonary venous flow enters the systemic venous circulation. […] Numerous factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs). The shunt magnitude, expressed as the Qp:Qs ratio, and other factors determine development of symptoms and complications in partial anomalous pulmonary venous connection (PAPVC). […] The most important factor is the number of pulmonary veins that drain into the systemic circulation. The more veins that anomalously drain, the more blood returns to the right side of the heart. Some authors have suggested that this defect becomes clinically significant when 50% or more of the pulmonary veins anomalously return.

• #32

  https://www.polradiol.com/Partial-anomalous-pulmonary-venous-connection-state-of-the-art-review-with-assessment,146849,0,2.html

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #33 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-connection-an-articulo-S2174204916302367

  Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic. Some authors have suggested that PAPVC becomes clinically significant only when 50% or more of the pulmonary blood flow returns anomalously. […] Imaging techniques, especially CT and MRI, have a pivotal role in the accurate characterization of these abnormalities, predicting outcomes and establishing appropriate preoperative planning. […] Unlike PAPVC, no catheter-corrective treatment is possible for TAPVC, although atrial septostomy is used in some patients when corrective surgery is delayed. […] In pediatric patients, PAPVC is usually treated if they have Qp:Qs of 1:1.5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure,9 although this cutoff has not been subject to rigorous study. […] Thus, for patients with PAPVC who have already developed mild to moderate pulmonary hypertension, surgical repair is usually safe and effective, although catheter-guided and medical therapies may play an increasing role.

• #34 Anomalous pulmonary venous drainage: a pictorial essay with a CT focus | Journal of Congenital Cardiology | Full Text

  https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-017-0008-4

  The magnitude of the left-to-right shunt is dependent of the number PAPVD. In a series looking at single PAPVD, the shunt is modest (Qp:Qs ~ 1.31.6) associated with mild dilatation of the right ventricle. […] APVD is one of the treatable causes of pulmonary hypertension in adults. […] This rare condition refers to failure of incorporation of normal pulmonary veins into the left atrium and accounts for up to 0.1% of congenital heart disease. […] Cross-sectional imaging with CT and MRI is widely used for the diagnosis of APVD in the adult population. CT has superior spatial resolution and thus is useful in presurgical planning in complex lesions. However, MRI is often sufficient for diagnosis and has the advantages of providing functional assessment and shunt calculation. […] The pulmonary veins have been found to be an important site of such arrhythmogenic activity, with up to 96% of foci triggering paroxysmal episodes of AF arising from them. […] Radiofrequency ablation (RFA) has been successfully used to disconnect the electrical connection between the pulmonary veins and the left atrium, thereby treating AF in affected patients.

• #35 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  Because of the left-to-right shunt that is established by PAPVR, a significant enough unrepaired shunt can result in pulmonary vascular remodelling that leads to the development of Pulmonary Artery Hypertension (PAH). […] The cause of PAH and right ventricular hypertrophy is the result of an increase in blood volume being introduced to the right side of the heart, resulting in right-sided volume overload and leading to PAH and ventricular hypertrophy.

• #36 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Over many years, excessive pulmonary venous return to the right side of the heart causes right atrial and ventricular dilation. This has numerous consequences, including risk of arrhythmia development, right-sided heart failure, and development of pulmonary hypertension. […] A native PAPVC usually does not have any associated obstruction to venous drainage. However, obstruction may occur postoperatively due to baffle obstruction.

• #37 Partial Anomalous Pulmonary Venous Return (PAPVR)

  https://my.clevelandclinic.org/health/diseases/24580-partial-anomalous-pulmonary-venous-return

  Over time, uncorrected left-to-right shunts can reverse and cause Eisenmenger syndrome. […] Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. […] Providers only recommend surgery if it’s necessary to ease symptoms or prevent complications. […] Surgery can typically treat PAPVR successfully. It’s important to speak with your provider about the details of your surgery and what it will accomplish. […] Many people don’t need surgery. If your PAPVR is mild, your provider will keep an eye on the situation. If you develop symptoms or signs of complications, your provider may recommend surgery at that point.

• #38 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Over many years, excessive pulmonary venous return to the right side of the heart causes right atrial and ventricular dilation. This has numerous consequences, including risk of arrhythmia development, right-sided heart failure, and development of pulmonary hypertension. […] A native PAPVC usually does not have any associated obstruction to venous drainage. However, obstruction may occur postoperatively due to baffle obstruction.

• #39 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. Because isolated PAPVC is a clinically significant independent risk factor, it should be actively treated to prevent the development of pulmonary hypertension later in life, which may result in severe clinical consequences. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity. Isolated PAPVC is very rare, and 97% of patients with isolated PAPVC have a right upper lobe PAPVC to the SVC. […] We found a significant association between isolated PAPVC and right-sided volume overload (P = 0.02).

• #40 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  PAPVC can be associated with other cardiac congenital diseases. […] Irrespective of the type or spectrum, all PAPVCs cause increased volume load in the right heart with increased pulmonary blood flow due functional left to right shunts. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #41

  https://www.polradiol.com/Partial-anomalous-pulmonary-venous-connection-state-of-the-art-review-with-assessment,146849,0,2.html

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #42 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

• #43 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  PAPVC can be associated with other cardiac congenital diseases. […] Irrespective of the type or spectrum, all PAPVCs cause increased volume load in the right heart with increased pulmonary blood flow due functional left to right shunts. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #44 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #45 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=staging.radpair.com&lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. […] The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen. […] More rarely it is seen with ostium primum defect, a subtype of atrioventricular defects.

• #46 Incidental radiological finding of partial anomalous pulmonary vein drainage | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-incidental-radiological-finding-partial-anomalous-articulo-S157921292100001X

  Partial anomalous pulmonary venous connection (PAPVC) is an unusual congenital vascular disease that is often diagnosed incidentally. It consists of the connection of one (or more) pulmonary veins to a systemic vein, causing left-to-right shunt. It is usually asymptomatic, but when associated with other malformations or if the shunt is significant, it may sometimes cause severe pulmonary hypertension. […] The most common form of PAPVC is drainage from the right upper lobe vein to the superior vena cava; this form is usually associated in 80% of cases with sinus venosus atrial septal defect (ASD). […] The CT scan showed a PAPVC involving drainage from the right upper lobe, the middle lobe, and some segments of the right lower lobe to the superior vena cava, which resulted in dilation of right heart cavities and signs of precapillary pulmonary hypertension. […] Our case is interesting because of the patient’s age at presentation (despite significant PAPVC) and the absence of concomitant venous sinus ASD.

• #47 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a disease that is often asymptomatic in children and may not present until later in adulthood either symptomatically, or as an incidental finding on radiology or autopsy. […] The most common presentation of PAPVR involves the right upper pulmonary vein as it drains into the junction of the superior vena cava (SVC) and right atrium creating a left-to-right shunt which results in freshly oxygenated blood from the lungs being returned to, and mixed with, deoxygenated systemic blood in the right atrium. […] In most cases of PAPVR, the right upper pulmonary vein abnormally drains into the junction of the superior vena cava (SVC) and right atrium, creating a left-to-right shunt. […] There is almost always an associated Atrial Septal Defect (ASD) present in PAPVR.

• #48 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  In the occurrence of Partial Anomalous Pulmonary Venous Return (PAPVR), a failure of this separation process results in one or multiple pulmonary veins draining into an inappropriate structure. […] The most common presentation of PAPVR involves the right upper pulmonary vein and drains into the junction of the superior vena cava (SVC) and right atrium, creating a left-to-right shunt, and there is almost always an associated Atrial Septal Defect (ASD) present. […] The estimated overall incidence of PAPVR is 0.5 %. […] To further understand the etiology of PAPVR, the embryological development of the SVC must also be understood. […] The SVC is derived from the proximal portion of the right anterior cardinal vein and the right common cardinal vein at a point that is caudal to the transverse anastomosis within the embryo.

• #49 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=staging.radpair.com&lang=us

  Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the right heart or systemic venous system, rather than in the left atrium. […] Four types of PAPVR have been described: supra-cardiac, cardiac, infracardiac, and mixed. […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. […] It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. […] The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen. […] More rarely it is seen with ostium primum defect, a subtype of atrioventricular defects.

• #50 Partial Anomalous Pulmonary Venous Connection with Ventricular Septal Defect and Intact Atrial Septum – Journal of Cardiac Critical Care TSS

  https://jcardcritcare.org/partial-anomalous-pulmonary-venous-connection-with-ventricular-septal-defect-and-intact-atrial-septum/

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac abnormality in which the pulmonary veins drain into the right atrium directly or the superior vena cava 90% of PAPVC are accompanied by atrial septal defect. However, PAPVC with ventricular septal defect is a rare entity. […] PAPVC with isolated ventricular septal defect (VSD) is a rare congenital cardiac malformation. […] In the case of PAPVC, few PVs drain abnormally into RA or into SVC. In the case of PAPVC, it is unusual to see more than one vein draining into RA. […] It is seen that 0.40.7% of cases are diagnosed as PAPVC in an autopsy series. […] Usually, the right-sided PVs drain into RA, but, in this case, LSPV is seen draining into an innominate vein. […] PAPVC is associated with ASD in 90% of cases.

• #51 Partially anomalous pulmonary venous connection to the superior and inferior vena cavae. | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-13-05/

  Partially anomalous pulmonary venous connection to the superior and inferior vena cavae. […] This represented an unusual variant of partially anomalous pulmonary venous connection (PAPVC) in which the right pulmonary veins drained to both the IVC and SVC, and not to the left atrium. […] The discovery on CMR that the right pulmonary veins drained to the SVC as well as the IVC led to an important modification of the surgical plan. […] PAPVC is a congenital cardiac anomaly in which some but not all of the pulmonary veins connect to the systemic veins. […] The most common variants include the left upper pulmonary vein to innominate vein, right upper pulmonary vein to the superior vena cava, and right pulmonary veins to the inferior vena cava. […] Nonetheless, numerous variations of PAPVC have been described. […] Furthermore, PAPVC is associated with congenital conditions such as atrial septal defects, tetralogy of Fallot, pulmonary hypoplasia and pulmonary sequestration in the setting of scimitar syndrome, Noonan’s syndrome and Turner’s syndrome.

• #52 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-connection-an-articulo-S2174204916302367

  TAPVC, as in case report 3, accounts for approximately 1-5% of congenital cardiovascular anomalies.2 This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation. […] PAPVC is also a relatively uncommon congenital anomaly, found in only 0.50.7% of the general population.4 Anomalies in veins from the right lung are twice as common as from the left lung. […] Anomalous pulmonary venous connections are classified on the basis of the location of pulmonary venous drainage as one of four types: supracardiac, cardiac, infracardiac, or mixed. […] In case report 1, we describe an infracardiac pulmonary drainage known as scimitar syndrome, in which part of the right lung is drained by a pulmonary vein connecting to the inferior vena cava.

• #53 Partially anomalous pulmonary venous connection to the superior and inferior vena cavae. | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-13-05/

  Partially anomalous pulmonary venous connection to the superior and inferior vena cavae. […] This represented an unusual variant of partially anomalous pulmonary venous connection (PAPVC) in which the right pulmonary veins drained to both the IVC and SVC, and not to the left atrium. […] The discovery on CMR that the right pulmonary veins drained to the SVC as well as the IVC led to an important modification of the surgical plan. […] PAPVC is a congenital cardiac anomaly in which some but not all of the pulmonary veins connect to the systemic veins. […] The most common variants include the left upper pulmonary vein to innominate vein, right upper pulmonary vein to the superior vena cava, and right pulmonary veins to the inferior vena cava. […] Nonetheless, numerous variations of PAPVC have been described. […] Furthermore, PAPVC is associated with congenital conditions such as atrial septal defects, tetralogy of Fallot, pulmonary hypoplasia and pulmonary sequestration in the setting of scimitar syndrome, Noonan’s syndrome and Turner’s syndrome.

• #54 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #55 Anomalous pulmonary venous connection – Wikipedia

  https://en.wikipedia.org/wiki/Anomalous_pulmonary_venous_connection

  Partial anomalous pulmonary venous connection (PAPVR) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins. […] It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. […] The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. […] It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome. […] To understand the different types of anomalous pulmonary venous connection, it is important to establish the background in which normal fetal vasculature develops. […] During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus.

• #56 Partial anomalous pulmonary venous return – UpToDate

  https://www.uptodate.com/contents/partial-anomalous-pulmonary-venous-return

  Partial anomalous pulmonary venous return (PAPVR; also known as partial anomalous pulmonary venous connection [PAPVC]), encompasses a spectrum of congenital cardiovascular anomalies. Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect with the anomalous pulmonary vein. […] The anatomic abnormalities that result in PAPVR and the diagnosis and management of PAPVR will be reviewed here. Total anomalous pulmonary venous connection (TAPVC) is discussed separately. […] For congenital anomalies of the pulmonary vein, the terms „anomalous venous return,” „anomalous venous connection,” and „anomalous venous drainage” are often used synonymously and reflect the same physiology. […] However, in the purest anatomic sense, the term „anomalous venous return” is the more general description, while „anomalous venous connection” and „anomalous venous drainage” have distinct meanings, as discussed below.

• #57 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  In TAPVC, the entire oxygenated blood from the lungs returns to the right atrium. Therefore, communication between the left and right atrium is crucial for survival in TAPVC. Mixing of the oxygenated and deoxygenated blood occurs in the right atrium, which is then shunted right to the left at the level of atria. Therefore, the left atrium and aorta get mixed blood, which leads to cyanosis in patients with TAPVC.

• #58 Total anomalous pulmonary venous connection – UpToDate

  https://www.uptodate.com/contents/total-anomalous-pulmonary-venous-connection

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways. The anatomic variants of TAPVC are dependent upon which specific connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.

• #59 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  In TAPVC, the entire oxygenated blood from the lungs returns to the right atrium. Therefore, communication between the left and right atrium is crucial for survival in TAPVC. Mixing of the oxygenated and deoxygenated blood occurs in the right atrium, which is then shunted right to the left at the level of atria. Therefore, the left atrium and aorta get mixed blood, which leads to cyanosis in patients with TAPVC.

• #60 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-connection-an-articulo-S2174204916302367

  Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all (total form) or some (partial form) pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium. […] Anomalous pulmonary venous connections are a specific group of congenital heart defects caused by the abnormal drainage of a part or the entire lung to a systemic vein or the right atrium. The estimated incidence is 2/100000 births.1 Most frequently only a single pulmonary vein is anomalous. However, more than one vein can have abnormal drainage, and rarely all the pulmonary venous vessels can connect to the right side of the heart, a condition known as total anomalous pulmonary venous connection (TAPVC). […] Thus, abnormal venous pulmonary drainage may be partial (PAPVC) in cases where only part of the pulmonary venous anatomy is abnormal, or it can involve all the pulmonary veins (TAPVC), resulting in complete drainage of the pulmonary venous return into the systemic venous circulation.

• #61 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all (total form) or some (partial form) pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium. […] The development of the pulmonary veins starts early in embryonic life and involves several complicated processes. Pulmonary venous developmental anomalies occur if any of these processes fails to progress properly. Thus, abnormal venous pulmonary drainage may be partial (PAPVC) in cases where only part of the pulmonary venous anatomy is abnormal, or it can involve all the pulmonary veins (TAPVC), resulting in complete drainage of the pulmonary venous return into the systemic venous circulation. […] TAPVC, as in case report 3, accounts for approximately 1-5% of congenital cardiovascular anomalies. This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation.

• #62 Partial and Total Anomalous Pulmonary Venous Connections | Radiology Key

  https://radiologykey.com/partial-and-total-anomalous-pulmonary-venous-connections/

  Partial and total anomalous drainage are described as two distinct entities, as the classification, pathophysiology, and diagnosis are quite different. […] Partial anomalous drainage of a right pulmonary vein to superior or inferior vena cava is associated with a sinus venosus atrial septal defect. […] Partial anomalous drainage of a right pulmonary vein to right atrium can also be associated with a secundum atrial septal defect. […] Partial anomalous drainage of a left pulmonary vein(s) is usually into the left innominate vein or coronary sinus and often associated with a secundum atrial septal defect. […] Partial anomalous drainage is common in left isomerism. […] Depends on how much of the pulmonary circulation empties into the systemic circulation, presence of pulmonary venous obstruction, and associated cardiac anomalies.

• #63 Partial and Total Anomalous Pulmonary Venous Connections | Radiology Key

  https://radiologykey.com/partial-and-total-anomalous-pulmonary-venous-connections/

  Basically a left-to-right shunt increasing the pulmonary-to-systemic flow ratio proportional to the number of veins connected to the right-sided circulation and to the presence of an associated atrial septal defect. […] Mild physiologic abnormality with a hemodynamically relevant left-to-right shunt usually with complete return of all veins from one lung or with an associated atrial septal defect. […] Right atrium and ventricle dilate, leading to right heart volume overload. […] Unobstructed pulmonary venous return causes a left-to-right shunt, and with all blood bypassing the left atrium, an obligatory interatrial right-to-left shunt is necessary for survival. […] Complete mixing of systemic and pulmonary venous blood within the heart, resulting in identical oxygenation in all four chambers and produces cyanosis.

• #64 Anomalous pulmonary venous connection – Wikipedia

  https://en.wikipedia.org/wiki/Anomalous_pulmonary_venous_connection

  Partial anomalous pulmonary venous connection (PAPVR) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins. […] It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. […] The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. […] It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome. […] To understand the different types of anomalous pulmonary venous connection, it is important to establish the background in which normal fetal vasculature develops. […] During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus.

• #65 Anomalous pulmonary venous connection – Wikipedia

  https://en.wikipedia.org/wiki/Anomalous_pulmonary_venous_connection

  Partial anomalous pulmonary venous connection (PAPVR) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins. […] It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. […] The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. […] It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome. […] To understand the different types of anomalous pulmonary venous connection, it is important to establish the background in which normal fetal vasculature develops. […] During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus.

• #66 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Unlike PAPVC, no catheter-corrective treatment is possible for TAPVC, although atrial septostomy is used in some patients when corrective surgery is delayed. […] In pediatric patients, PAPVC is usually treated if they have Qp:Qs of 1:1.5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure, although this cutoff has not been subject to rigorous study. […] Thus, for patients with PAPVC who have already developed mild to moderate pulmonary hypertension, surgical repair is usually safe and effective, although catheter-guided and medical therapies may play an increasing role.

• #67

  https://journals.lww.com/10.4103/jmedsci.jmedsci_89_24

  Partial anomalous pulmonary venous connection (PAPVC) is a relatively rare congenital heart anomaly characterized by abnormal drainage of one or more pulmonary veins into a location other than the left atrium. […] The anomalous connection or drainage of pulmonary veins arises from the failure of one or more pulmonary veins to establish a connection with the left atrium during fetal development. Different levels of dilation in the right ventricle and right atrium are typically observed due to a persistent left-to-right shunt, leading to increased blood flow to the lungs and potential remodeling of the pulmonary vascular bed, which can result in pulmonary arterial hypertension. […] In contemporary times, the advancement of endovascular techniques allows for a less invasive approach to correct the anomaly. Some pediatric cases undergoing transcatheter occlusion for PAPVR have demonstrated fast recovery, shorter in-hospital stays, lower costs, and heightened patient satisfaction. […] In this case report, the transcatheter occlusion of the vertical vein in PAPVR has proven to be a viable alternative to surgical treatment. It is associated with effectiveness, safety, shorter hospital stays, and faster recovery times.

• #68 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #69 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #70 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  Changes in some genes have been linked to heart conditions at birth. […] Having rubella during pregnancy can cause changes in an unborn baby’s heart. […] Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. […] Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions. […] Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby. […] To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you don’t have symptoms, surgery may not be needed. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options.

• #71 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #72 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #73 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  Changes in some genes have been linked to heart conditions at birth. […] Having rubella during pregnancy can cause changes in an unborn baby’s heart. […] Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. […] Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions. […] Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby. […] To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you don’t have symptoms, surgery may not be needed. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options.

• #74 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  Changes in some genes have been linked to heart conditions at birth. […] Having rubella during pregnancy can cause changes in an unborn baby’s heart. […] Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. […] Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions. […] Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby. […] To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you don’t have symptoms, surgery may not be needed. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options.

• #75 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #76 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  Changes in some genes have been linked to heart conditions at birth. […] Having rubella during pregnancy can cause changes in an unborn baby’s heart. […] Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. […] Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions. […] Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby. […] To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you don’t have symptoms, surgery may not be needed. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options.

• #77 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  Changes in some genes have been linked to heart conditions at birth. […] Having rubella during pregnancy can cause changes in an unborn baby’s heart. […] Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. […] Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions. […] Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby. […] To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you don’t have symptoms, surgery may not be needed. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options.

• #78 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby’s heart is forming before birth. An unborn baby also is called a fetus. […] Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby. […] What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include: […] Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.

• #79 Unusual presentation of a patient with partial anomalous pulmonary venous connections without a septal defect: a case report and literature review | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-025-00809-3

  Although there is a trend toward transcatheter correction of the anomaly, surgical treatment remains the gold standard for treating PAPVC. […] The presentation of PAPVC is considerably nonspecific and depends on the magnitude of the intracardiac shunt and the anomalous drainage of the PV. Therefore, diagnosis is not straightforward and requires a thorough evaluation using multimodal imaging. […] However, regardless of the type of PAPVC, it causes left-to-right shunts in pre-tricuspid level, which result in recirculation of the oxygenated blood into the pulmonary circulation, increased pulmonary blood flow, and increased volume load on the right heart. […] In summary, it is important to note that aging and its associated effects, such as HTN and diastolic dysfunction, contribute to increased pressure in the LA compared to the RA. This, in turn, can lead to increased blood flow to the right heart and a greater left-to-right shunt.

• #80 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Over many years, excessive pulmonary venous return to the right side of the heart causes right atrial and ventricular dilation. This has numerous consequences, including risk of arrhythmia development, right-sided heart failure, and development of pulmonary hypertension. […] A native PAPVC usually does not have any associated obstruction to venous drainage. However, obstruction may occur postoperatively due to baffle obstruction.

• #81 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. Because isolated PAPVC is a clinically significant independent risk factor, it should be actively treated to prevent the development of pulmonary hypertension later in life, which may result in severe clinical consequences. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity. Isolated PAPVC is very rare, and 97% of patients with isolated PAPVC have a right upper lobe PAPVC to the SVC. […] We found a significant association between isolated PAPVC and right-sided volume overload (P = 0.02).

• #82 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

• #83 Partial and Total Anomalous Pulmonary Venous Connections | Radiology Key

  https://radiologykey.com/partial-and-total-anomalous-pulmonary-venous-connections/

  Obstructed pulmonary venous return results in pulmonary venous and arterial hypertension, pulmonary edema, and diminished pulmonary return, leading to low cardiac output. […] Increased right-sided pressure overload leads to right heart failure. […] Restriction to flow may result from an obstruction of the pulmonary venous circuit or across the atrial septum. […] Infracardiac type is almost always obstructed and is a result of a combination of factors. […] Supracardiac type, seldom obstructed. […] Cardiac type, rarely obstructed.

• #84 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  PAPVC can be associated with other cardiac congenital diseases. […] Irrespective of the type or spectrum, all PAPVCs cause increased volume load in the right heart with increased pulmonary blood flow due functional left to right shunts. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #85 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

• #86 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://www.acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  Because of the left-to-right shunt that is established by PAPVR, a significant enough unrepaired shunt can result in pulmonary vascular remodelling that leads to the development of Pulmonary Artery Hypertension (PAH). […] The cause of PAH and right ventricular hypertrophy is the result of an increase in blood volume being introduced to the right side of the heart, resulting in right-sided volume overload and leading to PAH and ventricular hypertrophy.

• #87 Case Study: Partial Anomalous Pulmonary Venous Return Of The Right Upper Pulmonary Vein Into The Cephalic Portion Of The SVC Causing A Left-To-Right Shunt In 56-Year-Old Asian Female

  https://acquaintpublications.com/article/case_study_partial_anomalous_pulmonary_venous_return_of_the_right_upper_pulmonary_vein_into_the_cephalic_portion_of_the_svc_causing_a_left_to_right_shunt_in_56_year_old_asian_female

  Because of the left-to-right shunt that is established by PAPVR, a significant enough unrepaired shunt can result in pulmonary vascular remodelling that leads to the development of Pulmonary Artery Hypertension (PAH). […] The cause of PAH and right ventricular hypertrophy is the result of an increase in blood volume being introduced to the right side of the heart, resulting in right-sided volume overload and leading to PAH and ventricular hypertrophy.

• #88 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

• #89 Partial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9673975/

  PAPVC can be associated with other cardiac congenital diseases. […] Irrespective of the type or spectrum, all PAPVCs cause increased volume load in the right heart with increased pulmonary blood flow due functional left to right shunts. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #90

  https://www.polradiol.com/Partial-anomalous-pulmonary-venous-connection-state-of-the-art-review-with-assessment,146849,0,2.html

  The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Some authors believe that at least 50% of the anomalous pulmonary venous flow in systemic circulation is required to present a clinically significant PAPVC. […] There are different types of anomalous venous connections forming a heterogenous morphological spectrum. […] The number of anomalous veins, the size and site of draining ostia, and the presence and size of septal defects are the most important determinants of the haemodynamic load and therefore the clinical presentation. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies.

• #91 Unusual presentation of a patient with partial anomalous pulmonary venous connections without a septal defect: a case report and literature review | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-025-00809-3

  Although there is a trend toward transcatheter correction of the anomaly, surgical treatment remains the gold standard for treating PAPVC. […] The presentation of PAPVC is considerably nonspecific and depends on the magnitude of the intracardiac shunt and the anomalous drainage of the PV. Therefore, diagnosis is not straightforward and requires a thorough evaluation using multimodal imaging. […] However, regardless of the type of PAPVC, it causes left-to-right shunts in pre-tricuspid level, which result in recirculation of the oxygenated blood into the pulmonary circulation, increased pulmonary blood flow, and increased volume load on the right heart. […] In summary, it is important to note that aging and its associated effects, such as HTN and diastolic dysfunction, contribute to increased pressure in the LA compared to the RA. This, in turn, can lead to increased blood flow to the right heart and a greater left-to-right shunt.

• #92 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. Because isolated PAPVC is a clinically significant independent risk factor, it should be actively treated to prevent the development of pulmonary hypertension later in life, which may result in severe clinical consequences. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity. Isolated PAPVC is very rare, and 97% of patients with isolated PAPVC have a right upper lobe PAPVC to the SVC. […] We found a significant association between isolated PAPVC and right-sided volume overload (P = 0.02).

• #93 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  Partial anomalous pulmonary venous return refers to anomalies in which one or more (but not all) of the pulmonary veins connects to a location other than the left atrium. This causes left to right shunting which may have hemodynamic and therefore clinical significance, warranting repair in some patients. […] Anatomically, partial anomalous pulmonary venous return refers to anomalies in which one or more (but not all) of the pulmonary veins connects to a location other than the left atrium. Often, this means one or more pulmonary veins empty into the right atrium or a systemic vein such as the superior vena cava (SVC) or inferior vena cava (IVC). Physiologically, this produces a left-to-right shunt, allowing for already-oxygenated blood to recirculate into the lungs and result in excessive pulmonary blood flow.

• #94 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  A surgical correction is recommended for patients with PAPVR when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (aka: ratio of pulmonary flow (Qp) to systemic flow (Qs) is 1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary venous resistance is less than one third of systemic venous resistance. Surgical repair involves intracaval baffling of the left atrium (Warden procedure) or direct reimplantation of the anomalous pulmonary vein into the left atrium.

• #95 Unusual presentation of a patient with partial anomalous pulmonary venous connections without a septal defect: a case report and literature review | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-025-00809-3

  The management of PAPVC depends on clinical findings, hemodynamic characteristics, and anatomical features. […] Corrective intervention is indicated in the following situations: the presence of significant symptoms related to this anomaly, a significant left-to-right shunt indicated by a Qp/Qs ratio greater than 2, or imaging evidence showing right ventricular (RV) dilation or RV volume overload. […] Open surgical correction remains the gold standard treatment for these patients. However, selected patients undergoing percutaneous transcatheter modification have demonstrated excellent outcomes. […] Although rare, PAPVC can be a potentially debilitating anomaly if it is not diagnosed on time. The presentation is nonspecific, and accurate diagnosis primarily relies on multimodal imaging techniques, especially PCTA or PMRA.

• #96 Unusual presentation of a patient with partial anomalous pulmonary venous connections without a septal defect: a case report and literature review | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-025-00809-3

  The management of PAPVC depends on clinical findings, hemodynamic characteristics, and anatomical features. […] Corrective intervention is indicated in the following situations: the presence of significant symptoms related to this anomaly, a significant left-to-right shunt indicated by a Qp/Qs ratio greater than 2, or imaging evidence showing right ventricular (RV) dilation or RV volume overload. […] Open surgical correction remains the gold standard treatment for these patients. However, selected patients undergoing percutaneous transcatheter modification have demonstrated excellent outcomes. […] Although rare, PAPVC can be a potentially debilitating anomaly if it is not diagnosed on time. The presentation is nonspecific, and accurate diagnosis primarily relies on multimodal imaging techniques, especially PCTA or PMRA.

• #97 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  A surgical correction is recommended for patients with PAPVR when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (aka: ratio of pulmonary flow (Qp) to systemic flow (Qs) is 1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary venous resistance is less than one third of systemic venous resistance. Surgical repair involves intracaval baffling of the left atrium (Warden procedure) or direct reimplantation of the anomalous pulmonary vein into the left atrium.

• #98 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

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