Materiały źródłowe

• #1 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. […] DIAGNOSIS […] Echocardiography […] Magnetic resonance imaging […] Computed tomography […] Cardiac catheterization.

• #2 Partial Anomalous Pulmonary Venous Return (PAPVR)

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

  Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disorder that causes abnormal blood flow from your lungs to your heart. One or more pulmonary veins return oxygen-rich blood to the wrong side of your heart. This blood recirculates through your lungs instead of out to your body. Symptoms include shortness of breath and fatigue. […] Healthcare providers diagnose PAPVR through a physical exam and testing. During an exam, your provider uses a stethoscope to listen to your heart. Some people with PAPVR have a heart murmur. If your provider suspects a problem, they’ll order further testing. […] Tests that help providers diagnose PAPVR include: Echocardiogram, especially a transesophageal echocardiogram (TEE). Heart MRI. Cardiac computed tomography (CT) scan. […] Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. Children diagnosed with PAPVR are more likely than adults to need surgery. This is because they often have additional heart disorders that require surgical treatment.

• #3 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. […] The overall prevalence of PAPVR is 0.4-0.7%. […] Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. […] Chest radiographic features are particular to each subtype of PAPVR. […] Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. It is considered the imaging modality of choice. […] Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

• #4 Partial anomalous pulmonary venous return – UpToDate

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

  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. […] The estimated prevalence of PAPVR is 0.2 to 0.7 percent. The true prevalence may be higher since mild cases may go unrecognized. PAPVR is more common in patients with Turner syndrome. […] Disclaimer: This generalized information is a limited summary of diagnosis, treatment, and/or medication information. It is not meant to be comprehensive and should be used as a tool to help the user understand and/or assess potential diagnostic and treatment options.

• #5 Partial anomalous pulmonary venous return – UpToDate

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

  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. […] The estimated prevalence of PAPVR is 0.2 to 0.7 percent. The true prevalence may be higher since mild cases may go unrecognized. PAPVR is more common in patients with Turner syndrome. […] Disclaimer: This generalized information is a limited summary of diagnosis, treatment, and/or medication information. It is not meant to be comprehensive and should be used as a tool to help the user understand and/or assess potential diagnostic and treatment options.

• #6 Partial Anomalous Pulmonary Venous Return (PAPVR)

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

  Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disorder that causes abnormal blood flow from your lungs to your heart. One or more pulmonary veins return oxygen-rich blood to the wrong side of your heart. This blood recirculates through your lungs instead of out to your body. Symptoms include shortness of breath and fatigue. […] Healthcare providers diagnose PAPVR through a physical exam and testing. During an exam, your provider uses a stethoscope to listen to your heart. Some people with PAPVR have a heart murmur. If your provider suspects a problem, they’ll order further testing. […] Tests that help providers diagnose PAPVR include: Echocardiogram, especially a transesophageal echocardiogram (TEE). Heart MRI. Cardiac computed tomography (CT) scan. […] Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. Children diagnosed with PAPVR are more likely than adults to need surgery. This is because they often have additional heart disorders that require surgical treatment.

• #7 Transthoracic Repair Of Left-Sided Partial Anomalous Pulmonary Venous Connection | CTSNet

  https://www.ctsnet.org/article/transthoracic-repair-left-sided-partial-anomalous-pulmonary-venous-connection

  The physiologic effect of a PAPVC is the creation of a left to right shunt caused by drainage of one or more pulmonary veins either directly into the right heart, or via a systemic vein. […] Those with an isolated PAPVC (without an ASD) of the left upper lobe to a vertical vein draining into the left innominate vein, can undergo safe and effective surgical correction via a closed approach utilizing a left thoracotomy.

• #8

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

  Partial anomalous venous connections or return (PAPVC/PAPVR) represents an anomaly in which at least one or more PVs, but not all, drain directly into the systemic venous circulation (i.e. right atrium, superior vena cava, or brachiocephalic vein). The prevalence of PAPVC has been reported between 0.4 and 0.7%. The incidence of occurrence is more common on the right side, and it may be detected incidentally on CT or MR imaging. 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. […] […] […] Therefore, accurate and early diagnosis of the condition and associated anomalies is crucial for decision-making and definitive management. Nowadays multidetector computed tomography angiography (MDCTA) and MR angiograms (MRA) have nearly replaced the traditionally used imaging techniques, helping considerably in optimal assessment. This article gives comprehensive details of PAPVC and its variations using MDCTA imaging. […] […] […] MDCTA and MR angiograms are excellent imaging techniques for accurate visualization of the anomalous anatomy in 3-dimensional space and provide optimum description of the disease, which helps the clinical in decision-making and planning of definitive management.

• #9 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Partial anomalous pulmonary venous return (PAPVR) involving the left upper lobe pulmonary vein is an exceptionally rare congenital anomaly that, if untreated, can lead to pulmonary hypertension (PH). Its nonspecific clinical presentation often results in delayed diagnosis. […] This report also highlights the diagnostic challenges associated with PAPVR. […] The clinical manifestations of PAPVR vary widely depending on the degree of shunting and associated cardiac anomalies. Symptoms range from asymptomatic cases to severe dyspnea, fatigue, or signs of right heart failure. The condition may remain undiagnosed for years, as its usual presenting symptoms such as dyspnea, right heart failure, and PH are not exclusive to PAPVR. Furthermore, it is not routinely investigated as a cause of PH, leading to frequent misclassification of PH as idiopathic or attributing it to coexisting lung diseases.

• #10 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Partial anomalous pulmonary venous return (PAPVR) involving the left upper lobe pulmonary vein is an exceptionally rare congenital anomaly that, if untreated, can lead to pulmonary hypertension (PH). Its nonspecific clinical presentation often results in delayed diagnosis. […] This report also highlights the diagnostic challenges associated with PAPVR. […] The clinical manifestations of PAPVR vary widely depending on the degree of shunting and associated cardiac anomalies. Symptoms range from asymptomatic cases to severe dyspnea, fatigue, or signs of right heart failure. The condition may remain undiagnosed for years, as its usual presenting symptoms such as dyspnea, right heart failure, and PH are not exclusive to PAPVR. Furthermore, it is not routinely investigated as a cause of PH, leading to frequent misclassification of PH as idiopathic or attributing it to coexisting lung diseases.

• #11 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. […] The overall prevalence of PAPVR is 0.4-0.7%. […] Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. […] Chest radiographic features are particular to each subtype of PAPVR. […] Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. It is considered the imaging modality of choice. […] Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

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

  https://emedicine.medscape.com/article/351007-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. Knowledge of the variation patterns of normal pulmonary venous drainage is necessary in order to diagnose 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. […] Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD. […] Prognosis is excellent for patients with partial anomalous pulmonary venous connection (PAPVC). The perioperative mortality rate is comparable to that for ASD repair (0.1%). […] Arrhythmias may occur in adults with unrepaired PAPVC secondary to chronic right atrial enlargement due to volume overload.

• #13 Partial anomalous pulmonary venous return // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/partial-anomalous-pulmonary-venous-return

  Partial anomalous pulmonary venous return is a rare heart problem that’s present at birth. That means it is a congenital heart defect. […] If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood. […] An echocardiogram is done to diagnose partial anomalous pulmonary venous return. This test uses sound waves to create images of the beating heart. An echocardiogram shows the pulmonary veins and the size of the heart chambers. It also measures the speed of blood flow. An echocardiogram can help diagnose a hole in the heart. […] Other tests such as an electrocardiogram (ECG or EKG), a chest X-ray or a CT scan may be done if more information is needed.

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

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

  Patients with a single anomalous vein and an intact atrial septum usually remain asymptomatic. […] Surgical corrections of PAPVC have good outcomes with a low complication rate and typically lead to an immediate resolution of symptoms. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

• #15 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Partial anomalous pulmonary venous return (PAPVR) involving the left upper lobe pulmonary vein is an exceptionally rare congenital anomaly that, if untreated, can lead to pulmonary hypertension (PH). Its nonspecific clinical presentation often results in delayed diagnosis. […] This report also highlights the diagnostic challenges associated with PAPVR. […] The clinical manifestations of PAPVR vary widely depending on the degree of shunting and associated cardiac anomalies. Symptoms range from asymptomatic cases to severe dyspnea, fatigue, or signs of right heart failure. The condition may remain undiagnosed for years, as its usual presenting symptoms such as dyspnea, right heart failure, and PH are not exclusive to PAPVR. Furthermore, it is not routinely investigated as a cause of PH, leading to frequent misclassification of PH as idiopathic or attributing it to coexisting lung diseases.

• #16 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

  To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard if there is a hole in the heart. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Tests are needed to diagnose partial anomalous pulmonary venous return (PAPVR). […] An ultrasound of the heart called an echocardiogram can sometimes confirm PAPVR. This test uses sound waves to make pictures of the beating heart. An echocardiogram often shows the pulmonary veins, the size of the heart chambers and any holes in the heart. It also measures the speed of blood flow. […] Sometimes, an echocardiogram may not detect PAPVR. Other tests such as a CT scan or MRI may be used.

• #17 Partial Anomalous Pulmonary Venous Return Irish Congenital Heart Centre

  https://congenitalheartcentre.ie/congenital-heart-conditions/partial-anomalous/

  Partial anomalous pulmonary venous return, sometimes called partial anomalous pulmonary venous connection, is a heart defect present at birth (congenital) in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the heart’s upper right chamber (right atrium), instead of correctly entering the heart’s upper left chamber (left atrium). This causes some oxygen-rich blood from the lungs to mix with oxygen-poor blood before entering the right atrium. […] To diagnose this condition, your doctor may review your signs and symptoms, conduct a physical exam, and listen to your heart with a stethoscope to check for a heart murmur. […] This condition is usually diagnosed with an transthoracic echocardiogram or a transoesophageal echocardiogram. This test uses sound waves to produce video images of your heart in motion. Doctors may use this test to determine whether the right atrium or ventricle is enlarged and see how many pulmonary veins are attaching to the hearts left atrium. […] Doctors may also use other imaging tests, such as an MRI or CT scan, to diagnose your condition or gather more information about your condition.

• #18 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  Cardiomegaly noted on chest radiography may be the initial reason for referral of a child with partial anomalous pulmonary venous connection (PAPVC). Other findings may include a dilated main pulmonary artery and increased pulmonary arterial vascular markings. However, chest radiography findings may be grossly normal. […] PAPVC has been diagnosed in utero. In children, echocardiography is the study of choice; however, the lesion can be easily missed if routine echocardiography does not incorporate definition of the entire pulmonary venous return. The size or the diameter of the pulmonary veins should be determined by 2-dimensional (2D) echocardiography. Spectral Doppler study of the individual pulmonary veins is also important to determine obstruction to the flow. […] In most patients, transthoracic echocardiography can be performed to diagnose PAPVC and obviate the need for cardiac catheterization. A high index of suspicion for the presence of the lesion is helpful to properly diagnose the condition.

• #19 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

  To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard if there is a hole in the heart. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Tests are needed to diagnose partial anomalous pulmonary venous return (PAPVR). […] An ultrasound of the heart called an echocardiogram can sometimes confirm PAPVR. This test uses sound waves to make pictures of the beating heart. An echocardiogram often shows the pulmonary veins, the size of the heart chambers and any holes in the heart. It also measures the speed of blood flow. […] Sometimes, an echocardiogram may not detect PAPVR. Other tests such as a CT scan or MRI may be used.

• #20 Total anomalous pulmonary venous return (TAPVR) – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/total-anomalous-pulmonary-venous-return/cdc-20385613

  In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. As a result, oxygen-rich blood mixes with oxygen-poor blood, as shown in purple. In a typical heart, shown on the left, oxygen-rich blood flows from the pulmonary veins to the upper left chamber, also called the left atrium. […] Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] An echocardiogram is the test used to diagnose total anomalous pulmonary venous return. It uses sound waves to create images of the heart as it beats. An echocardiogram can show the pulmonary veins, any holes in the heart and the size of the heart chambers. It also shows blood flow through the heart and heart valves. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

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

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

  A thorough 2D and doppler interrogation are important for the diagnosis of TAPVC. […] It is important to image all the pulmonary veins and their course, determine if there is any obstruction to the pulmonary venous drainage and evaluate for interatrial communication or a persistent ductus arteriosus. […] Non-invasive techniques such as echocardiography, CT, and MR angiograms have largely replaced cardiac catheterization. […] Surgical treatment is indicated with the development of symptoms, significant left to right shunt, or evidence of right ventricular dysfunction. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized. […] The clinical distinction between ASD and PAPVC can be difficult. […] Imaging techniques are helpful for an accurate diagnosis.

• #22 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  Cardiomegaly noted on chest radiography may be the initial reason for referral of a child with partial anomalous pulmonary venous connection (PAPVC). Other findings may include a dilated main pulmonary artery and increased pulmonary arterial vascular markings. However, chest radiography findings may be grossly normal. […] PAPVC has been diagnosed in utero. In children, echocardiography is the study of choice; however, the lesion can be easily missed if routine echocardiography does not incorporate definition of the entire pulmonary venous return. The size or the diameter of the pulmonary veins should be determined by 2-dimensional (2D) echocardiography. Spectral Doppler study of the individual pulmonary veins is also important to determine obstruction to the flow. […] In most patients, transthoracic echocardiography can be performed to diagnose PAPVC and obviate the need for cardiac catheterization. A high index of suspicion for the presence of the lesion is helpful to properly diagnose the condition.

• #23 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein. […] Magnetic resonance imaging (MRI) is rapidly becoming the procedure of choice for further investigation of PAPVC. […] Contrast-enhanced computed tomography (CT) scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults. […] Electrocardiography (ECG) findings may be normal in partial anomalous pulmonary venous connection (PAPVC). They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy. […] Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation in the workup of partial anomalous pulmonary venous connection (PAPVC). Right heart pressures are normal in the pediatric patient. Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out. Qp:Qs can be calculated.

• #24 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 are various subtypes of this condition, nearly four-fifths of diagnosed cases involve anomalous pulmonary veins draining into the RA via a sinus venosus connection with an ASD. In contrast, the diagnosis of pure PAPVC without a septal defect is seen in approximately one-tenth of cases and is substantially rare. […] Diagnostic procedures when encountering CHD usually start with CXR and an echocardiogram. Although increased heart size, abnormally located pulmonary vessel structure, and interstitial changes in the lungs might suggest abnormalities in the cardiovascular system, the first choice for detecting anatomical heart defects is an echocardiogram. TTE and TEE are used, with TEE offering more sensitivity and specificity, especially for visualization of the entire atrial septum and PV connections.

• #25

  https://journals.lww.com/md-journal/fulltext/2016/11010/echocardiographic_diagnosis_of_anomalous_pulmonary.88.aspx

  We sought to evaluate the value of echocardiography in the diagnosis of different types of anomalous pulmonary venous connections (APVCs) and summarize the diagnostic experience. […] Echocardiography is regarded as the preferred initial screening and diagnostic modality in patients with APVC. […] Compared with surgical or CTA results, the sensitivity and specificity of echocardiography in the diagnosis of APVCs were 97.6% and 99.9%, respectively. […] The echocardiography misdiagnoses were mainly seen in PAPVCs. […] The diagnostic accuracy of classification was 94% and 100% for TAPVCs and PAPVCs, respectively. […] Echocardiography has specific value in diagnosing and classification of APVC, especially the supracardiac and cardiac TAPVCs. […] The comparisons of the echocardiographic and surgical or CTA results in diagnosing APVCs are shown in Table 5.

• #26

  https://journals.lww.com/md-journal/fulltext/2016/11010/echocardiographic_diagnosis_of_anomalous_pulmonary.88.aspx

  The sensitivity and specificity of echocardiography in the diagnosis of APVCs were 97.6% and 99.9%. […] The echocardiography misdiagnoses were mainly observed in PAPVCs. […] The diagnostic accuracy of typing either TAPVC or PAPVC was 98.7%. […] The cardiac type has a relatively high diagnostic accuracy, but the pulmonary veins drain separately, increasing the difficulty in making an accurate diagnosis. […] The mixed type has the lowest diagnostic accuracy in our report and in previous reports. […] Echocardiography can provide a definite pathway of pulmonary veins in typical APVC types but markedly limited information in the atypical type and the mixed type.

• #27 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Transthoracic echocardiography is often the initial diagnostic tool for patients with suspected PH. However, the diagnosis of PAPVR is rarely made using TTE because standard imaging windows inadequately visualize pulmonary veins. […] Although CT angiography is highly sensitive for diagnosing PAPVR, it is often overlooked during initial radiological evaluations due to its low incidence and a lack of clinical suspicion. […] Diagnosing PAPVR presents unique challenges. The TTE is often inadequate, as the superior posterior atrial septum is poorly visualized, making the diagnosis rarely achievable through this modality. The gold standard for diagnosis is CT venography or cardiac MRI; however, these are not routinely performed in the evaluation of PH. […] In patients with unexplained PH, CT pulmonary angiography should be carefully re-reviewed with careful scrutiny of the pulmonary veins. Cardiac MRI is particularly valuable in detecting subtle anomalies and should be considered when PH remains unexplained or when a shunt is suspected but not confirmed through initial evaluations.

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

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

  A thorough 2D and doppler interrogation are important for the diagnosis of TAPVC. […] It is important to image all the pulmonary veins and their course, determine if there is any obstruction to the pulmonary venous drainage and evaluate for interatrial communication or a persistent ductus arteriosus. […] Non-invasive techniques such as echocardiography, CT, and MR angiograms have largely replaced cardiac catheterization. […] Surgical treatment is indicated with the development of symptoms, significant left to right shunt, or evidence of right ventricular dysfunction. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized. […] The clinical distinction between ASD and PAPVC can be difficult. […] Imaging techniques are helpful for an accurate diagnosis.

• #29 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

  To diagnose partial anomalous pulmonary venous return (PAPVR), a healthcare professional does a physical exam and listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard if there is a hole in the heart. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Tests are needed to diagnose partial anomalous pulmonary venous return (PAPVR). […] An ultrasound of the heart called an echocardiogram can sometimes confirm PAPVR. This test uses sound waves to make pictures of the beating heart. An echocardiogram often shows the pulmonary veins, the size of the heart chambers and any holes in the heart. It also measures the speed of blood flow. […] Sometimes, an echocardiogram may not detect PAPVR. Other tests such as a CT scan or MRI may be used.

• #30 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  The present series describes a group of adults with left-to-right shunts including partial anomalous pulmonary venous return (PAPVR) and/or an atrial septal defect (ASD) evaluated with ECG-gated 128-slice multidetector computed tomography (MDCT). […] The presence, course, number of anomalous veins and associated cardiovascular defects can be reliably observed by 128-slice MDCT angiography. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography. […] In our experience ECG-gated MDCT with fast data acquisition and multidimensional reconstructions offers excellent spatial resolution and the possibility to reliably depict intracardiac and pulmonary shunts.

• #31 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein. […] Magnetic resonance imaging (MRI) is rapidly becoming the procedure of choice for further investigation of PAPVC. […] Contrast-enhanced computed tomography (CT) scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults. […] Electrocardiography (ECG) findings may be normal in partial anomalous pulmonary venous connection (PAPVC). They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy. […] Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation in the workup of partial anomalous pulmonary venous connection (PAPVC). Right heart pressures are normal in the pediatric patient. Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out. Qp:Qs can be calculated.

• #32 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. […] The overall prevalence of PAPVR is 0.4-0.7%. […] Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. […] Chest radiographic features are particular to each subtype of PAPVR. […] Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. It is considered the imaging modality of choice. […] Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

• #33 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein. […] Magnetic resonance imaging (MRI) is rapidly becoming the procedure of choice for further investigation of PAPVC. […] Contrast-enhanced computed tomography (CT) scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults. […] Electrocardiography (ECG) findings may be normal in partial anomalous pulmonary venous connection (PAPVC). They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy. […] Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation in the workup of partial anomalous pulmonary venous connection (PAPVC). Right heart pressures are normal in the pediatric patient. Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out. Qp:Qs can be calculated.

• #34 Partial anomalous pulmonary venous return | Radiology Case | Radiopaedia.org

  https://radiopaedia.org/cases/partial-anomalous-pulmonary-venous-return-4?lang=us

  Abnormal ECG with incomplete right bundle branch block. Abnormal echocardiography with suspect of an anomalous pulmonary venous return. […] 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. […] MRI provides noninvasive volumetric anatomic data and enables evaluation of systemic veins, as well as the number, origin, course, and drainage of all pulmonary veins, including anomalous connections or obstruction. MRI also quantifies ventricular volumes and evaluates the right ventricular outflow tract and pulmonary trunk, sites of stenosis, pulmonary artery conduits or branch pulmonary arteries.

• #35 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

  Overall, TEE and TTE are suboptimal for diagnosing PAPVC, and multidetector computed tomography (MDCT) or magnetic resonance imaging (MRI) angiography provides significantly higher accuracy. Newer-generation three-dimensional MDCT has proven to be an excellent diagnostic modality. […] The management of PAPVC depends on clinical findings, hemodynamic characteristics, and anatomical features. Therapeutic options include (1) follow-up, (2) medical management, (3) surgical treatment, and (4) transcatheter interventional procedures. 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.

• #36 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein. […] Magnetic resonance imaging (MRI) is rapidly becoming the procedure of choice for further investigation of PAPVC. […] Contrast-enhanced computed tomography (CT) scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults. […] Electrocardiography (ECG) findings may be normal in partial anomalous pulmonary venous connection (PAPVC). They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy. […] Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation in the workup of partial anomalous pulmonary venous connection (PAPVC). Right heart pressures are normal in the pediatric patient. Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out. Qp:Qs can be calculated.

• #37 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. […] The overall prevalence of PAPVR is 0.4-0.7%. […] Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. […] Chest radiographic features are particular to each subtype of PAPVR. […] Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. It is considered the imaging modality of choice. […] Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

• #38 TAPVR | Types, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/tapvr

  The diagnosis of total anomalous pulmonary venous return may be suspected when a doctor hears a typical heart murmur. They may see evidence of overload on the right side of the heart. […] Oxygen saturations may be low in children without pulmonary venous obstruction. An electrocardiogram (ECG) may be helpful to show if there are problems with enlargement of the heart. […] A chest X-ray will show enlargement of the right side of the heart. In newborns with TAPVR and obstructed pulmonary venous return, chest X-ray may show signs of pulmonary edema (fluid in the lungs). […] The diagnosis of TAPVR is made by echocardiogram (echo). This study will show the abnormal connection of the pulmonary veins. […] Echocardiography (echo) will also show if the right atrium and right ventricle are larger than normal. It can show the size of and flow across the atrial septal defect (ASD).

• #39 Partial Anomalous Pulmonary Venous Connection Workup: Imaging Studies, Other Tests, Procedures

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

  In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein. […] Magnetic resonance imaging (MRI) is rapidly becoming the procedure of choice for further investigation of PAPVC. […] Contrast-enhanced computed tomography (CT) scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults. […] Electrocardiography (ECG) findings may be normal in partial anomalous pulmonary venous connection (PAPVC). They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy. […] Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation in the workup of partial anomalous pulmonary venous connection (PAPVC). Right heart pressures are normal in the pediatric patient. Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out. Qp:Qs can be calculated.

• #40 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] Therefore, an assessment of the shunt by an accurate measurement of the Qp/Qs ratio is essential. […] Currently, the gold standard method for PAH diagnosis and Qp/Qs measurement is RHC. […] It is therefore essential to complete the work-up of a PAPVR with a RHC, in particular if it is associated with an ASD, in order to get an accurate measurement of pulmonary pressures and resistances but also of the shunt ratio, and therefore to better classify patients at risk of developing elevated pulmonary pressures and to manage them in accordance with the current guidelines.

• #41 Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr

  Pulse oximetry: a noninvasive way to monitor the oxygen content of the blood. This number will be low, generally in the mid to high 80s (normal is 95 to 100 percent). […] Cardiac catheterization: a thin tube is inserted into the heart through a vein and/or artery in the leg. This is occasionally needed to aid in diagnosis. […] Cardiac MRI: a three-dimensional image that shows the hearts abnormalities.

• #42 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] During pregnancy, an ultrasound, a tool that creates pictures of the baby, may detect TAPVR. If the health care provider suspects TAPVR from the ultrasound findings, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. […] However, TAPVR is not commonly detected during pregnancy. It is hard for doctors to see the pulmonary veins on the prenatal screening tests. This is because not much blood goes to the lungs before the baby is born. It is easier to detect TAPVR after birth when blood is flowing to the lungs and returning to the heart. […] During a physical exam, a doctor may observe symptoms or hear a heart murmur. If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. The most common test is an echocardiogram, which is an ultrasound of the heart. Cardiac catheterization also can confirm the diagnosis by showing which blood vessels are abnormally attached. Other tests to make the diagnosis include chest x-rays and other medical tests. […] TAPVR can also be detected with newborn pulse oximetry screening. Low levels of oxygen in the blood can be a sign of a critical CHD like TAPVR. Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms.

• #43 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

  Partial anomalous pulmonary vein connections (PAPVC) are rare congenital abnormalities in which one or more pulmonary veins drain into the right atrium. This pathological condition may present in various ways, such as chest pain and dyspnea, or it may be diagnosed incidentally. Consequently, missed or late diagnoses are common, highlighting the importance of optimal diagnostic modalities. […] Detection of PAPVC is not straightforward in many cases and often requires evaluation with various imaging techniques for accurate diagnosis. Therefore, physicians encountering undefined causes of chest pain or dyspnea should consider multiple imaging modalities. Follow-up is also important, as certain groups of patients may require surgical treatment to prevent complications. […] 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.

• #44 Unusual Doppler in Pulmonary Veins Leads to the Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return with Dual Connections

  https://www.gavinpublishers.com/article/view/unusual-doppler-in-pulmonary-veins-leads-to-the-diagnosis-of-unusual-partial-anomalous-pulmonary-venous-return-with-dual-connections

  In this paper, we report an unusual case of Partial Anomalous Pulmonary Venous Return (PAPVR) in a patient with new onset, decompensated heart failure and right sided chamber enlargement. […] Imaging is essential to diagnosis and required to obtain accurate anatomy. This includes echocardiography, cardiac CT, cardiac MRI and often, cardiac catheterization, to verify the degree of shunting. Color flow mapping and Doppler methods are all helpful tools when evaluating potential anomalous connections. PAPVR should be considered in situations where SVC, innominate vein or IVC appear dilated, as well as isolated right sided enlargement. […] This case illustrates an unusual example of PAPVR with bidirectional flow, as well as the importance of thorough evaluation even on routine transesophageal echocardiograms especially for patients with right heart enlargement. […] Patients with significant right heart enlargement warrant careful evaluation of left to right shunt with multimodality imaging approach.

• #45 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Transthoracic echocardiography is often the initial diagnostic tool for patients with suspected PH. However, the diagnosis of PAPVR is rarely made using TTE because standard imaging windows inadequately visualize pulmonary veins. […] Although CT angiography is highly sensitive for diagnosing PAPVR, it is often overlooked during initial radiological evaluations due to its low incidence and a lack of clinical suspicion. […] Diagnosing PAPVR presents unique challenges. The TTE is often inadequate, as the superior posterior atrial septum is poorly visualized, making the diagnosis rarely achievable through this modality. The gold standard for diagnosis is CT venography or cardiac MRI; however, these are not routinely performed in the evaluation of PH. […] In patients with unexplained PH, CT pulmonary angiography should be carefully re-reviewed with careful scrutiny of the pulmonary veins. Cardiac MRI is particularly valuable in detecting subtle anomalies and should be considered when PH remains unexplained or when a shunt is suspected but not confirmed through initial evaluations.

• #46

  https://journals.lww.com/md-journal/fulltext/2016/11010/echocardiographic_diagnosis_of_anomalous_pulmonary.88.aspx

  We sought to evaluate the value of echocardiography in the diagnosis of different types of anomalous pulmonary venous connections (APVCs) and summarize the diagnostic experience. […] Echocardiography is regarded as the preferred initial screening and diagnostic modality in patients with APVC. […] Compared with surgical or CTA results, the sensitivity and specificity of echocardiography in the diagnosis of APVCs were 97.6% and 99.9%, respectively. […] The echocardiography misdiagnoses were mainly seen in PAPVCs. […] The diagnostic accuracy of classification was 94% and 100% for TAPVCs and PAPVCs, respectively. […] Echocardiography has specific value in diagnosing and classification of APVC, especially the supracardiac and cardiac TAPVCs. […] The comparisons of the echocardiographic and surgical or CTA results in diagnosing APVCs are shown in Table 5.

• #47

  https://journals.lww.com/md-journal/fulltext/2016/11010/echocardiographic_diagnosis_of_anomalous_pulmonary.88.aspx

  The sensitivity and specificity of echocardiography in the diagnosis of APVCs were 97.6% and 99.9%. […] The echocardiography misdiagnoses were mainly observed in PAPVCs. […] The diagnostic accuracy of typing either TAPVC or PAPVC was 98.7%. […] The cardiac type has a relatively high diagnostic accuracy, but the pulmonary veins drain separately, increasing the difficulty in making an accurate diagnosis. […] The mixed type has the lowest diagnostic accuracy in our report and in previous reports. […] Echocardiography can provide a definite pathway of pulmonary veins in typical APVC types but markedly limited information in the atypical type and the mixed type.

• #48 Partial anomalous pulmonary venous return | Radiology Case | Radiopaedia.org

  https://radiopaedia.org/cases/partial-anomalous-pulmonary-venous-return-4?lang=us

  In this case, MRI of partial anomalous pulmonary venous return quantifies any shunt, patency, and ventricular systolic function. […] Although pulmonary venous developmental anomalies have been evaluated traditionally with echocardiography and angiography, MRI is playing an increasing role in their characterization.

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

  https://radiopaedia.org/cases/partial-anomalous-pulmonary-venous-return-4?lang=us

  In this case, MRI of partial anomalous pulmonary venous return quantifies any shunt, patency, and ventricular systolic function. […] Although pulmonary venous developmental anomalies have been evaluated traditionally with echocardiography and angiography, MRI is playing an increasing role in their characterization.

• #50

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

  Partial anomalous venous connections or return (PAPVC/PAPVR) represents an anomaly in which at least one or more PVs, but not all, drain directly into the systemic venous circulation (i.e. right atrium, superior vena cava, or brachiocephalic vein). The prevalence of PAPVC has been reported between 0.4 and 0.7%. The incidence of occurrence is more common on the right side, and it may be detected incidentally on CT or MR imaging. 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. […] […] […] Therefore, accurate and early diagnosis of the condition and associated anomalies is crucial for decision-making and definitive management. Nowadays multidetector computed tomography angiography (MDCTA) and MR angiograms (MRA) have nearly replaced the traditionally used imaging techniques, helping considerably in optimal assessment. This article gives comprehensive details of PAPVC and its variations using MDCTA imaging. […] […] […] MDCTA and MR angiograms are excellent imaging techniques for accurate visualization of the anomalous anatomy in 3-dimensional space and provide optimum description of the disease, which helps the clinical in decision-making and planning of definitive management.

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

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

  Imaging differential considerations include total anomalous pulmonary venous return (TAPVR), pulmonary varix, persistent left superior vena cava, collateral venous pathways, scimitar syndrome, acquired systemic-to-pulmonary venous shunt, pulmonary vein stenosis or obstructed pulmonary venous return.

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

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

  A thorough 2D and doppler interrogation are important for the diagnosis of TAPVC. […] It is important to image all the pulmonary veins and their course, determine if there is any obstruction to the pulmonary venous drainage and evaluate for interatrial communication or a persistent ductus arteriosus. […] Non-invasive techniques such as echocardiography, CT, and MR angiograms have largely replaced cardiac catheterization. […] Surgical treatment is indicated with the development of symptoms, significant left to right shunt, or evidence of right ventricular dysfunction. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized. […] The clinical distinction between ASD and PAPVC can be difficult. […] Imaging techniques are helpful for an accurate diagnosis.

• #53 Partial Anomalous Pulmonary Venous Return (PAPVR)

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

  Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disorder that causes abnormal blood flow from your lungs to your heart. One or more pulmonary veins return oxygen-rich blood to the wrong side of your heart. This blood recirculates through your lungs instead of out to your body. Symptoms include shortness of breath and fatigue. […] Healthcare providers diagnose PAPVR through a physical exam and testing. During an exam, your provider uses a stethoscope to listen to your heart. Some people with PAPVR have a heart murmur. If your provider suspects a problem, they’ll order further testing. […] Tests that help providers diagnose PAPVR include: Echocardiogram, especially a transesophageal echocardiogram (TEE). Heart MRI. Cardiac computed tomography (CT) scan. […] Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. Children diagnosed with PAPVR are more likely than adults to need surgery. This is because they often have additional heart disorders that require surgical treatment.

• #54 Partial Anomalous Pulmonary Venous Return (PAPVR)

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

  Providers only recommend surgery if it’s necessary to ease symptoms or prevent complications. You or your child may need surgery if PAPVR is causing significant left-to-right shunting. This means a considerable amount of blood that should go out to your body is recirculating through your right heart and lungs. This can cause uncomfortable symptoms or lead to complications (like PAH or heart failure). Your provider will use imaging tests to determine the extent of shunting. […] 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. The techniques can vary depending on the anatomy of your heart and pulmonary veins. In general, surgeons aim to reroute blood flow so that oxygen-rich blood ends up in your left heart chambers.

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

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

  A thorough 2D and doppler interrogation are important for the diagnosis of TAPVC. […] It is important to image all the pulmonary veins and their course, determine if there is any obstruction to the pulmonary venous drainage and evaluate for interatrial communication or a persistent ductus arteriosus. […] Non-invasive techniques such as echocardiography, CT, and MR angiograms have largely replaced cardiac catheterization. […] Surgical treatment is indicated with the development of symptoms, significant left to right shunt, or evidence of right ventricular dysfunction. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized. […] The clinical distinction between ASD and PAPVC can be difficult. […] Imaging techniques are helpful for an accurate diagnosis.

• #56 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  The management of PAPVR depends on the severity of symptoms, the magnitude of the shunt, and associated complications. Surgical correction is recommended in cases of right ventricular (RV) volume overload and when the pulmonary vascular resistance (PVR) is less than 3 Wood units (WU), and when the pulmonary-to-systemic blood flow ratio (Qp/Qs) exceeds 1.5. These criteria, based on data from atrial septal defect (ASD) studies and limited natural history series of PAPVR, aim to prevent irreversible pulmonary vascular remodeling, PH, and right heart failure. […] In conclusion, PAPVR involving the left upper lobe pulmonary vein is rare and difficult to diagnose. Echocardiography alone is insufficient for diagnosis, and the condition often goes unrecognized on CT angiography. The RHC can provide valuable diagnostic clues, while early consideration of cardiac MRI and thorough review of CT angiograms are critical for timely diagnosis.

• #57 250. 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. […] Diagnosis is usually incidental on a cross sectional imaging such as CTA or CMR. […] 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.

• #58 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

  Overall, TEE and TTE are suboptimal for diagnosing PAPVC, and multidetector computed tomography (MDCT) or magnetic resonance imaging (MRI) angiography provides significantly higher accuracy. Newer-generation three-dimensional MDCT has proven to be an excellent diagnostic modality. […] The management of PAPVC depends on clinical findings, hemodynamic characteristics, and anatomical features. Therapeutic options include (1) follow-up, (2) medical management, (3) surgical treatment, and (4) transcatheter interventional procedures. 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.

• #59 Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

  https://atm.amegroups.org/article/view/18960/html

  This case demonstrates the importance of accurately diagnosing anomalous venous connections on imaging, as they are primarily incidental findings but can have significant clinical impact. It is, therefore, crucial that once diagnosed, patients maintain follow-up with a cardiologist to monitor for signs of cardiac dysfunction.

• #60 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  PAPVR is a rare congenital heart disease that unfortunately remains neglected and poorly managed to date, both in terms of medical follow-up after diagnosis and screening for complications Pulmonary Arterial Hypertension (PAH) and risk factors for developing PAH. More extensive use of current guidelines is essential in the assessment of these patients and referral to RHC should be systematic.

• #61 Lower Extremity Edema: Long Term Complication of Partial Anomalous Pulmonary Venous Return Repair | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-18-03/

  The goal of partial anomalous venous return repair is to divert the pulmonary veins back to the left atrium and systemic veins to the right atrium. […] Long term complications after anomalous pulmonary venous return repair include SVC stenosis, pulmonary vein obstruction, sinus node dysfunction or atrial arrhythmias. […] Our patient has a SVC obstruction at the azygous arch, with upper body venous return via the hemiazygous vein to the inferior vena cava.

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

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

  Patients with a single anomalous vein and an intact atrial septum usually remain asymptomatic. […] Surgical corrections of PAPVC have good outcomes with a low complication rate and typically lead to an immediate resolution of symptoms. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

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

  https://emedicine.medscape.com/article/351007-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. Knowledge of the variation patterns of normal pulmonary venous drainage is necessary in order to diagnose 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. […] Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD. […] Prognosis is excellent for patients with partial anomalous pulmonary venous connection (PAPVC). The perioperative mortality rate is comparable to that for ASD repair (0.1%). […] Arrhythmias may occur in adults with unrepaired PAPVC secondary to chronic right atrial enlargement due to volume overload.

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

  https://emedicine.medscape.com/article/351007-overview

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac defect. Knowledge of the variation patterns of normal pulmonary venous drainage is necessary in order to diagnose 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. […] Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD. […] Prognosis is excellent for patients with partial anomalous pulmonary venous connection (PAPVC). The perioperative mortality rate is comparable to that for ASD repair (0.1%). […] Arrhythmias may occur in adults with unrepaired PAPVC secondary to chronic right atrial enlargement due to volume overload.

• #65 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

  Partial anomalous pulmonary vein connections (PAPVC) are rare congenital abnormalities in which one or more pulmonary veins drain into the right atrium. This pathological condition may present in various ways, such as chest pain and dyspnea, or it may be diagnosed incidentally. Consequently, missed or late diagnoses are common, highlighting the importance of optimal diagnostic modalities. […] Detection of PAPVC is not straightforward in many cases and often requires evaluation with various imaging techniques for accurate diagnosis. Therefore, physicians encountering undefined causes of chest pain or dyspnea should consider multiple imaging modalities. Follow-up is also important, as certain groups of patients may require surgical treatment to prevent complications. […] 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.

• #66 Partial Anomalous Pulmonary Venous Return With Persistent Angina: Simulating Coronary Artery Disease | Namana | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2309/1671

  Our patient had multiple cardiac catheter based angiography and TTE but did not detect PAPVR as there was no clinical suspicion and ASD. TTE showed RA, RV enlargement and severe PAH, which were confirmed by TEE. PAPVR was detected on TEE and confirmed by CTA. […] The diagnosis of PAPVR may be particularly elusive when the patient also has a more common diagnosis, such as CAD. Unexplained pulmonary hypertension and enlarged right-sided chambers should alert physicians to consider additional differential diagnoses including congenital heart disease despite the patients adult age.

• #67 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. […] Patients with or without symptoms and PAPVR with a Qp: Qs >1.5, moderate to severe tricuspid regurgitation (TR), pulmonary insufficiency, and dysfunction of the right ventricle are all indicated for surgical treatment to mitigate progressive right ventricular failure and irreversible pulmonary vascular disease. […] 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.

• #68 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 are various subtypes of this condition, nearly four-fifths of diagnosed cases involve anomalous pulmonary veins draining into the RA via a sinus venosus connection with an ASD. In contrast, the diagnosis of pure PAPVC without a septal defect is seen in approximately one-tenth of cases and is substantially rare. […] Diagnostic procedures when encountering CHD usually start with CXR and an echocardiogram. Although increased heart size, abnormally located pulmonary vessel structure, and interstitial changes in the lungs might suggest abnormalities in the cardiovascular system, the first choice for detecting anatomical heart defects is an echocardiogram. TTE and TEE are used, with TEE offering more sensitivity and specificity, especially for visualization of the entire atrial septum and PV connections.

• #69 Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11915243/

  Partial anomalous pulmonary venous return (PAPVR) involving the left upper lobe pulmonary vein is an exceptionally rare congenital anomaly that, if untreated, can lead to pulmonary hypertension (PH). Its nonspecific clinical presentation often results in delayed diagnosis. […] This report also highlights the diagnostic challenges associated with PAPVR. […] The clinical manifestations of PAPVR vary widely depending on the degree of shunting and associated cardiac anomalies. Symptoms range from asymptomatic cases to severe dyspnea, fatigue, or signs of right heart failure. The condition may remain undiagnosed for years, as its usual presenting symptoms such as dyspnea, right heart failure, and PH are not exclusive to PAPVR. Furthermore, it is not routinely investigated as a cause of PH, leading to frequent misclassification of PH as idiopathic or attributing it to coexisting lung diseases.

• #70 Partial Anomalous Pulmonary Venous Return: To Fix or Not to Fix?

  https://scholarlycommons.hcahealthcare.com/northtexas2024/18/

  Patient underwent routine pre-operative screening with a STS mortality risk score of 0.5%. Chest CT scan was identified to have left upper lobe pulmonary vein that drains into the left lateral brachiocephalic vein which is consistent with a partial anomalous pulmonary venous return (PAPVR). […] Transesophageal echocardiogram was negative for any obvious or large interatrial septal defect. The prevalence of PAPVR is about 0.4-0.7%. […] Recent advancements in sophisticated diagnostic imaging and greater knowledge of this anatomical variation have increased the frequency with which this condition is diagnosed.

• #71 Intraoperatory diagnosis of partial anomalous pulmonary venous return during pulmonary resection surgery in a non-small cell lung cancer patient | Archivos de Bronconeumología

  https://archbronconeumol.org/en-intraoperatory-diagnosis-partial-anomalous-pulmonary-articulo-S1579212921003025

  Intraoperatively, during the dissection of the main pulmonary artery (MPA), a large-caliber vessel from the left upper lobe was observed straddling the left MPA and ascending through the mediastinal fat anterior to the aortic arch. […] A CT scan of the pulmonary arteries performed during the preoperative period was reviewed, and a partial anomalous pulmonary venous return (PAPVR) from the upper left pulmonary vein (PV) toward the left braquicefalic venous trunk was visualized; it was sectioned with an Endostapler and the pulmonary resection surgery was completed. […] The availability of preoperative volumetric vascular reconstruction aids surgical planning and diagnosis of pulmonary vascular abnormalities.

• #72 Partial Anomalous Pulmonary Venous Return With Persistent Angina: Simulating Coronary Artery Disease | Namana | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2309/1671

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon adult congenital heart disease caused by an abnormal return of one or more, but not all, of the pulmonary veins to the right atrium or indirectly through venous connections from the anomalous pulmonary vein. Presentations vary from incidental findings to severe heart failure and diagnosis could easily be missed. […] Diagnosis of PAPVR may be particularly elusive when patient also has a more common diagnosis, such as coronary disease. Unexplained pulmonary hypertension should alert physicians to consider additional differential diagnoses including congenital heart disease despite the patients adult age. […] In past, PAPVR is diagnosed with cardiac catheterization and cardioangiography and confirmed during surgery. Chest radiography and TTE lack sensitivity and the newer modalities of imaging such as CTA, MRA, and TEE are highly sensitive and specific in detection of PAPVR.

• #73 Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

  https://atm.amegroups.org/article/view/18960/html

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition that often goes undiagnosed due to lack of symptoms early in life. […] In adulthood, it can manifest clinically as right-sided heart failure or pulmonary hypertension. […] However, depending on the number of pulmonary veins involved and the degree of left-to-right shunting, patients may develop serious complications including right-sided heart failure and cardiac arrhythmias. […] Maintaining clinical suspicion based on subtle clinical and radiologic findings is important as early recognition can prevent long-term complications such as pulmonary hypertension and heart failure. […] Diagnosis is confirmed with echocardiogram and cardiac CT. Cardiac MRI can further elucidate anatomy. […] With the growing use of imaging to both aid in diagnosis and treatment in patients with multiple comorbidities, it is important for clinicians and radiologists to become familiar with finding and evaluating congenital anomalies in adults.

• #74 250. 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. […] Diagnosis is usually incidental on a cross sectional imaging such as CTA or CMR. […] 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.

• #75 250. 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 gated cardiac CTA or CMR is helpful and recommended for definitive diagnosis. A CTA offers higher spatial resolution than a CMR at the cost of radiation and iodinated contrast exposure. A CMR offers high resolution for defining vascular anatomy, quantifying chamber dimensions, estimate shunt burden and degree of stenoses using flow quantification techniques. […] Cross sectional imaging such as CTA or CMR may be helpful to identify pulmonary venous connections and other extracardiac vascular anatomy.

• #76 Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

  https://atm.amegroups.org/article/view/18960/html

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition that often goes undiagnosed due to lack of symptoms early in life. […] In adulthood, it can manifest clinically as right-sided heart failure or pulmonary hypertension. […] However, depending on the number of pulmonary veins involved and the degree of left-to-right shunting, patients may develop serious complications including right-sided heart failure and cardiac arrhythmias. […] Maintaining clinical suspicion based on subtle clinical and radiologic findings is important as early recognition can prevent long-term complications such as pulmonary hypertension and heart failure. […] Diagnosis is confirmed with echocardiogram and cardiac CT. Cardiac MRI can further elucidate anatomy. […] With the growing use of imaging to both aid in diagnosis and treatment in patients with multiple comorbidities, it is important for clinicians and radiologists to become familiar with finding and evaluating congenital anomalies in adults.

• #77 Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

  https://atm.amegroups.org/article/view/18960/html

  This case demonstrates the importance of accurately diagnosing anomalous venous connections on imaging, as they are primarily incidental findings but can have significant clinical impact. It is, therefore, crucial that once diagnosed, patients maintain follow-up with a cardiologist to monitor for signs of cardiac dysfunction.

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