Materiały źródłowe

• #1 Partial Anomalous Pulmonary Venous Return (PAPVR)

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

  Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. […] Providers only recommend surgery if its necessary to ease symptoms or prevent complications. […] Surgery can typically treat PAPVR successfully. […] Many people dont 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.

• #1 Partial anomalous pulmonary venous return | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/partial-anomalous-pulmonary-venous-return

  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. If surgery for another heart condition is needed, surgeons may repair PAPVR at the same time. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options. During repair surgery, the heart surgeon will: […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #1 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease in which one or more pulmonary veins drain into the systemic venous circulation. […] We discuss the challenges in the diagnosis and medical management of isolated PAPVC and highlight the clinical and hemodynamic indications for pulmonary vasodilators and diuretics. […] Surgical correction is recommended for patients with impaired functional capacity, RV enlargement, large net left-to-right shunt with Qp:Qs 1.5:1, pulmonary artery (PA) systolic pressure less than 50% of the systemic pressure, and pulmonary vascular resistance (PVR) less than one-third of the systemic vascular resistance (SVR). […] Medically, clinical observation and/or pulmonary artery vasodilator therapy with guanylate cyclase inhibitors, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors have been reported.

• #1 Partial Anomalous Pulmonary Venous Connection Treatment & Management: Approach Considerations, Surgical Care, Consultations

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

  Medical therapy of partial anomalous pulmonary venous connection (PAPVC) is not indicated for asymptomatic patients. Heart failure in adults can be managed with diuretics, cardiac glycosides, afterload reduction, and beta blockade. Arrhythmias should be appropriately treated. […] If necessary, patients should be transferred to an institution skilled in pediatric cardiology and pediatric cardiac surgery for assessment and treatment. […] Definitive treatment for partial anomalous pulmonary venous connection (PAPVC) is surgical repair. Indications for surgical repair are controversial. […] One school of thought claims that all children should undergo repair because of the exceptionally low morbidity and mortality following this surgical procedure. Others suggest that appropriate criteria include a significant left-to-right shunt (Qp:Qs of about 2:1 or more) or such as an entire lung that anomalously drains, before recommending surgery.

• #1 Partial anomalous pulmonary venous return | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20359714/

  Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if: […] If you dont have symptoms, surgery may not be needed. If surgery for another heart condition is needed, surgeons may repair PAPVR at the same time. […] There are several types of surgery for PAPVR. Together, you and your surgeon will talk about the best options. During repair surgery, the heart surgeon will: […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. Its best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #1 Surgical Outcomes of Three Repair Techniques for Partial Anomalous Pulmonary Venous Connection in Adult Patients

  https://www.mdpi.com/2673-3846/3/4/16

  Surgical repair of partial anomalous venous connection (PAPVC) remains one of the most performed congenital cardiac surgery procedures. The ‘Achilles heel’ for the correction of PAPVC is the high rate of pacemaker (PM) implantation due to sinus node dysfunction and superior caval or pulmonary vein obstruction during surgery. The important point is the selection of an appropriate surgical procedure based on the anatomy of the defect. Surgical techniques to mitigate the risk of SVC obstruction or sinus node dysfunction include transcaval (TCR), transatrial, and modified Warden approaches. The goal of this study is to investigate and compare primary and secondary outcomes following surgical repair of PAPVC through TCR, transatrial, or modified Warden techniques. […] All surgical operations were performed by the same surgeon (AD). TCR consisted of a lateral aspect incision of the SVC extending up to the superior board of the pulmonary vein. The modified Warden technique consists of a longitudinal incision made along the anterior wall of the cephalic end of the divided SVC up to the innominate vein–SVC junction, which is then anastomosed to the posterior edge of the right atrial appendage. Preoperative planning was based on the anomaly location.

• #1 Partial Anomalous Pulmonary Venous Connection Treatment & Management: Approach Considerations, Surgical Care, Consultations

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

  Operative technique depends on the site of the anomalous vein or veins. The usual approach is a midline sternotomy and cardiopulmonary bypass. Surgical treatment of associated lesions may be necessary. […] For the PAPVC to the superior vena cava (SVC), the repair techniques may include internal patch technique, with or without SVC enlargement, or the caval division technique with atriocaval anastomosis (Warden technique). […] Routine postoperative care of the patient who has undergone cardiac surgery for PAPVC should be performed. Pain control should be optimal to reduce the risk of atelectasis. […] Intermittent follow-up to assess right heart size and pressures and cardiac function and rhythm is necessary in patients with partial anomalous pulmonary venous connection (PAPVC) who do not undergo surgical treatment.

• #1 Partners in Care | Atrial Septal Defect With Partial Anomalous…

  https://partnersincare.health/conditions/atrial-septal-defect-with-partial-anomalous-pulmonary-venous-return

  Treatment of ASD with PAPVR requires open-heart surgery to close the hole inside the heart and to reroute the anomalous pulmonary vein connection over to the left atrium. The timing of surgery is usually elective and based upon symptoms, the degree of right heart enlargement, and where the abnormal pulmonary vein connection is located.

• #1 Anomalous pulmonary venous connection – Wikipedia

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

  Partial anomalous pulmonary venous connection (or Partial anomalous pulmonary venous drainage or Partial anomalous pulmonary venous return) 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 sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure. However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases.

• #1 Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. Case resolution

  https://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S2604-73062023000100017&lng=en&nrm=iso&tlng=en

  The heart team decision was to percutaneously treat both the coronary artery disease and the partial anomalous pulmonary venous connection (PAPVC). […] Treatment of the PAPVC was performed 3 months later. […] The treatment of PAPVC is indicated with symptoms attributed to significant left-to-right shunt. […] Although surgery is the treatment of choice, percutaneous treatment may be a feasible alternative in patients with a pulmonary vein dually draining into a left innominate vein and the LA since the sealing of the VV with a vascular device redirects all pulmonary vein blood flow into the LA. […] Before sealing, transient balloon occlusion should be considered to assess LA and pulmonary pressure changes. If pressure increases, sealing should be reconsidered and if performed, LA decompression with an atrial flow regulator should follow.

• #1 Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. How would I approach it?

  https://recintervcardiol.org/en/clinical-cases/percutaneous-treatment-of-partial-anomalous-pulmonary-venous-connection-with-dual-drainage-how-would-i-approach-it

  PAPVC with dual LUPV drainage is unusual. Its peculiarity is that it allows us to perform a percutaneous procedure to redirect the flow of the LUPV towards the left atrium by occluding the conduit that communicates with systemic venous circulation (usually the VV). This allows us to eliminate the left-right shunt and normalize the LUPV return flow thus preventing the setbacks and potential complications associated with heart surgery. […] Therefore, the percutaneous option seems highly attractive. […] The procedure can be performed without general anesthesia and with sedation only since transesophageal ultrasound is not required. […] Selective angiographies of the VV even with the possibility of temporarily occluding the LUPV with an Amplatzer type of balloon to prevent rapid contrast washout allow us to choose the right device for proper anchoring purposes (with certain compression) avoiding embolization.

• #1 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Medical management of heart failure and PH in patients with isolated PAPVC remains a significant challenge. Surgical repair is the mainstay of corrective treatment for PAPVC when there is functional impairment, RV enlargement, large net left-to-right shunt (e.g., Qp:Qs 1.5), PA systolic pressure less than 50% of systemic pressure, and PVR that is less than one-third of SVR. […] However, most patients will benefit from medical therapy to maintain euvolemia and rate and/or rhythm control for concurrent arrhythmia as a bridge to surgery or as a destination therapy when surgery is not an option. […] Medical therapy for PH in a patient with PAPVC should be individualized based on several factors including clinical symptoms, the number of anomalous venous connections, hemodynamics, degree of RV failure, degree of left-to-right shunt, presence of shunt reversal, and presence of an ASD.

• #1 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Pulmonary vasodilators are typically used in patients with Eisenmenger syndrome when the development of shunt reversal (Qp:Qs 1) precludes surgical repair due to high surgical risk. […] However, they may be contraindicated in patients whose vasoreactivity test shows worsening left-to-right shunt and/or reduction in CO. […] In isolated PAPVC with predominantly pre-capillary PH and RV failure, oral diuretics may be a more appropriate option to decrease RV filling volume and PCWP, thereby preventing further pulmonary venous congestion and RV failure.

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

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

  Babies with TAPVR will need surgery to repair the defect. The age at which the surgery is done depends on: […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually: […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9627227/

  Treatment of SVC syndrome requires addressing the underlying condition, such as radiation therapy for tumor compression or removal of an indwelling catheter. Noninvasive methods are preferred, such as angioplasty and stent placement, but surgical intervention is sometimes required. […] Complications of the Warden procedure are primarily related to vascular obstruction at the sites of anastomosis (SVC and right atrial appendage), breakdown of the atrial patch used to redirect the anomalous pulmonary venous drainage into the left atrium, and sinus node dysfunction. […] In patients with SVC syndrome after the Warden procedure, recurrent right-to-left shunting can occur through hemiazygos-azygos collateral flow. This complication can occur remotely after surgical intervention, though most commonly it is described as an early complication requiring reintervention.

• #1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9627227/

  The Warden procedure is one of the most common techniques for surgical correction of partial anomalous pulmonary venous return (PAPVR), involving transection and reanastomosis of the superior vena cava (SVC) to the right atrial appendage and baffling of the anomalous pulmonary veins through the atrial septal defect. […] Although this procedure is typically well-tolerated, SVC syndrome is a rare but well-described complication. […] Patients requiring surgical correction undergo surgical transection and reanastomosis of the superior vena cava (SVC) to the right atrial appendage and baffling of the anomalous pulmonary veins through the sinus venosus defect, also known as the Warden procedure. […] In both adult and pediatric patients, endovascular intervention is preferred over repeat thoracotomy.

• #1 Infradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience | Cardiology in the Young | Cambridge Core

  https://www.cambridge.org/core/journals/cardiology-in-the-young/article/infradiaphragmatic-partial-anomalous-pulmonary-venous-connection-in-adulthood-considerations-for-management-a-single-centre-experience/2D1FEDE8CCB1DFC9FE08B61DA306D928

  Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation. […] Patients in Group I have been managed conservatively, as they were asymptomatic, without a significant shunt. Patients in Group II were surgically corrected using long right intra-atrial baffles. […] Conservative management should be considered for asymptomatic patients, without a significant shunt. Surgical treatment of infradiaphragmatic partial anomalous pulmonary venous connection of the entire right lung in inferior vena cava is challenging. Slow blood flow inside the long intra-atrial baffles inclines to thrombosis and occlusion, as we observed in two cases. Therefore, oral anticoagulation should be considered for long baffles with slow blood flow.

• #1 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

  In cases of PAPVC, there are a number of correction procedures, depending on the number and the site of the anomalous vein or veins. […] Their analysis demonstrated that corrective surgery can be done safely with low mortality and morbidity, independently of the type of surgical technique used, especially if conducted prior to the development of pulmonary hypertension. […] 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. Finally, in patients who have already progressed to severe pulmonary hypertension, lung or heart-lung transplantation may be necessary.

• #1 PAPVR Repair Surgery | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/treatments/surgical/papvr-repair/

  Partial anomalous pulmonary venous return (PAPVR) repair surgery is often, but not always, necessary to treat PAPVR. […] Many, but not all, children with this condition will need PAPVR repair surgery. […] In a PAPVR repair, Norton Children’s Heart Institute pediatric cardiothoracic surgeons typically will perform some combination of disconnecting and reconnecting the veins. The surgeons also may construct a patch to redirect the oxygen-rich blood from the pulmonary veins to the left atrium. […] Norton Children’s Heart Institute specialists expect children who have had surgery to correct PAPVR to live long and active lives after their repair.

• #1 Surgical Outcomes of Three Repair Techniques for Partial Anomalous Pulmonary Venous Connection in Adult Patients

  https://www.mdpi.com/2673-3846/3/4/16

  Surgical outcomes in adult patients with PAPVC are important. An experienced heart-team including cardiologists, anesthesiologists, perfusionists, and nurses is essential for good results. When using other techniques, a higher incidence of rhythm disturbance should be considered. High-volume centers may prefer this approach due to the potential benefit to the patient. […] Our findings suggest that the three surgical approaches may each play a role, although a definitive answer cannot be given at present in terms of technique superiority. A multi-center study would be required for validation purposes.

• #1 Management of partial anomalous pulmonary venous connections in patients requiring pulmonary resection: a case report and systematic review – Singhal – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17661/html

  Partial anomalous pulmonary venous connections (PAPVCs) are rare congenital anomalies that are frequently asymptomatic in adults. […] When PAPVCs are encountered in the patient requiring pulmonary resection, improper management can result in fulminant right-heart failure and death. […] Management of PAPVCs in the setting of pulmonary resection requires careful consideration of several factors including PAPVC anatomy, preoperative cardiopulmonary physiology (as assessed by Qp/Qs), and the extent of planned pulmonary resection. […] For PAPVCs that arise from a pulmonary segment that will be included in the resection specimen, simple ligation is adequate. […] When a PAPVC arises from an anatomic segment which will not be included in the resection, the management approach is more challenging and requires a pre-resection assessment of Qp/Qs and consideration of resection extent.

• #1 Management of partial anomalous pulmonary venous connections in patients requiring pulmonary resection: a case report and systematic review – Singhal – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17661/html

  Although a clear Qp/Qs cut-off is unknown, patients with Qp/Qs greater than 1.5 or symptomatic PAPVCs are at high risk for post-operative complications, and thus should undergo PAPVC repair. […] Successful PAPVC repair has been described both prior to and during pulmonary resection with good success. […] For patients with asymptomatic PAPVCs and normal Qp/Qs, standard pulmonary resection is likely sufficient. […] Finally, in patients with lesser resections and borderline Qp/Qs (ranging from 1.0 to 1.5), it remains unclear if PAPVC correction is necessary with reports demonstrating success with both approaches. […] In summary, management of PAPVCs during resection is an uncommon clinical scenario that requires careful consideration of venous anatomy, preoperative cardiopulmonary physiology, and resection extent.

• #1 Anomalous pulmonary venous return in a pregnant woman identified by cardiac magnetic resonance | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-anomalous-pulmonary-venous-return-in-articulo-S2174204914001469

  Anomalous pulmonary venous return (APVR) is a rare cardiac anomaly defined as one or more pulmonary veins draining into a structure other than the left atrium, with venous return directly or indirectly to the right atrium. The most common form is partial APVR, in which one to three pulmonary veins drain into systemic veins or into the right atrium. […] Partial APVR is usually diagnosed by echocardiography or conventional angiography, but since complete and selective characterization of the pulmonary veins may not be possible with these methods, other noninvasive exams are increasingly used, including magnetic resonance imaging (MRI) and computed tomography angiography. […] In view of these findings, the patient was referred for total surgical repair of APVR, which was performed with extracorporeal circulation, hypothermia (24C) and administration of cardioplegic solution. […] The indications for surgical repair of partial APVR depend on detailed assessment of the anatomy of the pulmonary and systemic veins, presence of ASD and quantification of left-to-right shunting.

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

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

  A 49-year-old female following transesophageal echocardiogram (TEE) to investigate an undiagnosed cardiac murmur and progressive dyspnea on exertion was found to have isolated left-sided anomalous pulmonary venous connection of all veins draining into the coronary sinus. This unique lesion required novel repair by coronary sinus unroofing and atrial septal closure. […] 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.

• #2 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. […] MANAGEMENT […] Surgery.

• #2 Partial Anomalous Pulmonary Venous Connection Treatment & Management: Approach Considerations, Surgical Care, Consultations

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

  Medical therapy of partial anomalous pulmonary venous connection (PAPVC) is not indicated for asymptomatic patients. Heart failure in adults can be managed with diuretics, cardiac glycosides, afterload reduction, and beta blockade. Arrhythmias should be appropriately treated. […] If necessary, patients should be transferred to an institution skilled in pediatric cardiology and pediatric cardiac surgery for assessment and treatment. […] Definitive treatment for partial anomalous pulmonary venous connection (PAPVC) is surgical repair. Indications for surgical repair are controversial. […] One school of thought claims that all children should undergo repair because of the exceptionally low morbidity and mortality following this surgical procedure. Others suggest that appropriate criteria include a significant left-to-right shunt (Qp:Qs of about 2:1 or more) or such as an entire lung that anomalously drains, before recommending surgery.

• #2 250. 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. […] 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. […] Repair of PAPVR may be considered at the time of closure of sinus venosus or other ASD. […] Transcatheter therapies are an area of ongoing innovation.

• #2 Partial and Total Anomalous Pulmonary Venous Connection – MD Searchlight

  https://mdsearchlight.com/heart-health/partial-and-total-anomalous-pulmonary-venous-connection/

  With Partial Anomalous Pulmonary Venous Connection (PAPVC), which is a condition where one or more, but not all, pulmonary veins drain into the right atrium instead of the left, the need for surgery depends on symptoms, the presence of a significant left to right shunt (a condition that allows blood to flow from the left side of heart to the right, potentially causing too much blood to reach the lungs), or evidence of right ventricular dysfunction (a condition that affects the lower right chamber of the heart). If these factors are not present, patients can usually be monitored without surgery. However, most patients with PAPVC of the sinus venosus type will likely need surgery between the ages of 2 and 4. If only one vein drains abnormally, they might not need any intervention at all in their lifetime.

• #2 Partial Anomalous Pulmonary Venous Connection Treatment & Management: Approach Considerations, Surgical Care, Consultations

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

  Operative technique depends on the site of the anomalous vein or veins. The usual approach is a midline sternotomy and cardiopulmonary bypass. Surgical treatment of associated lesions may be necessary. […] For the PAPVC to the superior vena cava (SVC), the repair techniques may include internal patch technique, with or without SVC enlargement, or the caval division technique with atriocaval anastomosis (Warden technique). […] Routine postoperative care of the patient who has undergone cardiac surgery for PAPVC should be performed. Pain control should be optimal to reduce the risk of atelectasis. […] Intermittent follow-up to assess right heart size and pressures and cardiac function and rhythm is necessary in patients with partial anomalous pulmonary venous connection (PAPVC) who do not undergo surgical treatment.

• #2 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

  In cases of PAPVC, there are a number of correction procedures, depending on the number and the site of the anomalous vein or veins. […] Their analysis demonstrated that corrective surgery can be done safely with low mortality and morbidity, independently of the type of surgical technique used, especially if conducted prior to the development of pulmonary hypertension. […] 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. Finally, in patients who have already progressed to severe pulmonary hypertension, lung or heart-lung transplantation may be necessary.

• #2

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9627227/

  The Warden procedure is one of the most common techniques for surgical correction of partial anomalous pulmonary venous return (PAPVR), involving transection and reanastomosis of the superior vena cava (SVC) to the right atrial appendage and baffling of the anomalous pulmonary veins through the atrial septal defect. […] Although this procedure is typically well-tolerated, SVC syndrome is a rare but well-described complication. […] Patients requiring surgical correction undergo surgical transection and reanastomosis of the superior vena cava (SVC) to the right atrial appendage and baffling of the anomalous pulmonary veins through the sinus venosus defect, also known as the Warden procedure. […] In both adult and pediatric patients, endovascular intervention is preferred over repeat thoracotomy.

• #2 PAPVR Repair Surgery | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/treatments/surgical/papvr-repair/

  Partial anomalous pulmonary venous return (PAPVR) repair surgery is often, but not always, necessary to treat PAPVR. […] Many, but not all, children with this condition will need PAPVR repair surgery. […] In a PAPVR repair, Norton Children’s Heart Institute pediatric cardiothoracic surgeons typically will perform some combination of disconnecting and reconnecting the veins. The surgeons also may construct a patch to redirect the oxygen-rich blood from the pulmonary veins to the left atrium. […] Norton Children’s Heart Institute specialists expect children who have had surgery to correct PAPVR to live long and active lives after their repair.

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

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

  If you arent experiencing symptoms or if there isnt much mixing of oxygen-rich and oxygen-poor blood, you may not need surgery to treat this condition. […] Surgery may be needed if there is a large amount of oxygen-rich and oxygen-poor blood mixing in the heart, or if you have had many pulmonary infections. If youre having surgery for another heart condition, surgeons may repair this heart defect at the same time. […] To repair this defect, surgeons generally reconnect the pulmonary veins to the left atrium. Surgeons will also close the hole between the atria, if there is one. […] Children will need regular follow-up appointments with paediatric cardiologists. Adults with this heart defect will need lifelong care and regular follow-up appointments with doctors trained in congenital heart conditions (adult congenital cardiologists) to monitor for any changes in their condition.

• #2 Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. How would I approach it?

  https://recintervcardiol.org/en/clinical-cases/percutaneous-treatment-of-partial-anomalous-pulmonary-venous-connection-with-dual-drainage-how-would-i-approach-it

  Tratamiento percutaneo de un drenaje venoso pulmonar anomalo parcial con drenaje dual. Cmo lo hara? […] PAPVC is a congenital malformation that can be associated with interatrial septum defects (it has been reported in 10% to 15% of the cases) or happen in isolation. […] Traditionally, the management of this entity requires surgery to redirect pulmonary flow towards the left cavities through the creation of an intracardiac baffle or the reimplantation of pulmonary vein into the left atrium. Results are favorable in experienced centers since many of these procedures are performed during childhood. […] In patients with an indication for surgical repair due to PAPVC, the European Society of Cardiology clinical practice guidelines of 2020 recommend estimating pulmonary vascular resistances. If under 5 Wood units, the procedure is safe and improves functional class while lowering pulmonary pressures.

• #2 Transcatheter treatment of partial anomalous pulmonary venous connection to left subclavian vein [Turk Kardiyol Dern Ars]

  https://archivestsc.com/jvi.aspx?un=TKDA-44376

  Reports of transcatheter treatment for dual drainage of an abnormal pulmonary venous connection are rare. Presently described is the case of a 27-year-old female with exertional dyspnea and a partial anomalous pulmonary venous connection of the left upper pulmonary vein with dual drainage to a vertical vein (VV) and the left atrium. The patient was evaluated with a balloon occlusion test to determine whether closing the anomalous VV connection would increase pulmonary pressure. The results of this test are an important guide to treatment decisions. A 12×9 mm Amplatzer Vascular Plug II device was successfully used to occlude the anomalous pulmonary venous connection using a transcatheter technique. This is a less invasive option than surgical repair and can be an appropriate choice in suitable cases.

• #2

  https://journals.lww.com/aopc/fulltext/2023/16030/partial_anomalous_venous_connection_with.15.aspx

  Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. […] Here, we report a case of intraparenchymal duplicate drainage in a patient with a complex cardiac history, who underwent a successful transcatheter occlusion and improvement in systemic hypoxemia. […] Recognition of this entity in angiography can allow prompt interventional treatment which has the potential to shorten the length of stay and avoid repeat surgical intervention.

• #2 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Medical management of heart failure and PH in patients with isolated PAPVC remains a significant challenge. Surgical repair is the mainstay of corrective treatment for PAPVC when there is functional impairment, RV enlargement, large net left-to-right shunt (e.g., Qp:Qs 1.5), PA systolic pressure less than 50% of systemic pressure, and PVR that is less than one-third of SVR. […] However, most patients will benefit from medical therapy to maintain euvolemia and rate and/or rhythm control for concurrent arrhythmia as a bridge to surgery or as a destination therapy when surgery is not an option. […] Medical therapy for PH in a patient with PAPVC should be individualized based on several factors including clinical symptoms, the number of anomalous venous connections, hemodynamics, degree of RV failure, degree of left-to-right shunt, presence of shunt reversal, and presence of an ASD.

• #2 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Pulmonary vasodilators are typically used in patients with Eisenmenger syndrome when the development of shunt reversal (Qp:Qs 1) precludes surgical repair due to high surgical risk. […] However, they may be contraindicated in patients whose vasoreactivity test shows worsening left-to-right shunt and/or reduction in CO. […] In isolated PAPVC with predominantly pre-capillary PH and RV failure, oral diuretics may be a more appropriate option to decrease RV filling volume and PCWP, thereby preventing further pulmonary venous congestion and RV failure.

• #2 An Integrated Treatment for Isolated Partial Anomalous Pulmonary Venous Connection with Severe Pulmonary Hypertension

  https://www.jstage.jst.go.jp/article/jpccs/advpub/0/advpub_23-007/_article

  The optimal treatment for unrepaired congenital heart disease in adults with pulmonary arterial hypertension (PAH) has not yet been clarified. […] He underwent successful surgical repair of isolated partial anomalous pulmonary venous connection (PAPVC) following target therapy for PAH. […] Specific target therapy for PAH could improve severe PAH related to isolated PAPVC in adults patients, and could make management of their circumstances easy and safe during the perioperative period.

• #2 Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. How would I approach it?

  https://recintervcardiol.org/en/clinical-cases/percutaneous-treatment-of-partial-anomalous-pulmonary-venous-connection-with-dual-drainage-how-would-i-approach-it

  Follow-up after the procedure should include an echocardiogram at 3-6 months to assess the reverse remodeling of right cavities and pulmonary systolic pressure. Performing a thoracic CCTA at 6 months is advised to confirm the complete occlusion of the shunt, and the correct position of the device into the VV.

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

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

  The long-term outcome after surgical repair of TAPV is also excellent. Because the surgical repair results in a normal circulation, these children are expected to grow and develop normally. […] Rare complications of TAPVR can occur late after surgery. Regular follow up by a qualified cardiologist is needed to detect these problems early if they occur. […] Rarely, obstruction to one or more pulmonary veins can develop. This can occur at the site of surgical repair, or due to abnormalities of the pulmonary veins themselves. […] Abnormal cardiac rhythm is another late complication of TAPVR. It is also rare. Because of the extensive atrial surgery involved in the repair, some patients can have abnormal electrical impulses in the atrium. […] When these impulses occur in single beats, they are typically benign. They do not need any treatment. On rare occasion, patients can have either continuous episodes of rapid heartbeat, or slow heart rates. Either sustained fast heart rates or slow heart rates may need treatment. The results of treatment (either medical or with a pacemaker) are excellent.

• #2 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/

  A 57 year old woman with a history of sinus venosus atrial septal defect and partial anomalous pulmonary venous return (PAPVR) repaired in childhood presented to the adult congenital heart disease clinic with junctional rhythm and significant lower extremity swelling. […] She was referred for cardiac MRI to better evaluate her PAPVR repair and sinus venous repair. […] 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. […] Most patients have anomalous pulmonary venous return to the superior vena cava. […] There are three described techniques for right sided pulmonary veins including a single patch technique, double patch technique and the Warden procedure. […] 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.

• #2 Surgical Outcomes of Three Repair Techniques for Partial Anomalous Pulmonary Venous Connection in Adult Patients

  https://www.mdpi.com/2673-3846/3/4/16

  The primary outcomes were cardiac death, death from any other cause, PM implantation, SVC or pulmonary vein obstruction, and MI. Secondary outcomes were infection, stroke, and NYHA class at discharge. The findings from this study elicit that all three techniques have low postoperative morbidity and are feasible and reliable procedures. […] The number of adults diagnosed with congenital cardiac abnormalities is higher than the number of children due to an improved assessment of cardiac anatomy and medical imaging diagnosis in adults. The main findings from this study can be summarized as follows: Firstly, TCR was utilized in 70% of patients with ACHD, including patients with unilateral total anomalous venous connection and ASDs. This technique was shown to have a high survival rate (100%) and few postoperative complications.

• #2 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. […] 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. […] Surgical management is reserved for persons with a hemodynamically significant left-to-right shunt (a ratio of pulmonary to systemic flow greater than 2:1), those with recurrent pulmonary infections, or during surgical repair of other major cardiac lesions. […] It is, therefore, crucial that once diagnosed, patients maintain follow-up with a cardiologist to monitor for signs of cardiac dysfunction.

• #2 Management of partial anomalous pulmonary venous connections in patients requiring pulmonary resection: a case report and systematic review – Singhal – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17661/html

  Although a clear Qp/Qs cut-off is unknown, patients with Qp/Qs greater than 1.5 or symptomatic PAPVCs are at high risk for post-operative complications, and thus should undergo PAPVC repair. […] Successful PAPVC repair has been described both prior to and during pulmonary resection with good success. […] For patients with asymptomatic PAPVCs and normal Qp/Qs, standard pulmonary resection is likely sufficient. […] Finally, in patients with lesser resections and borderline Qp/Qs (ranging from 1.0 to 1.5), it remains unclear if PAPVC correction is necessary with reports demonstrating success with both approaches. […] In summary, management of PAPVCs during resection is an uncommon clinical scenario that requires careful consideration of venous anatomy, preoperative cardiopulmonary physiology, and resection extent.

• #2 Management of partial anomalous pulmonary venous connections in patients requiring pulmonary resection: a case report and systematic review – Singhal – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17661/html

  For patients with PAPVCs arising from the pulmonary segment to be included in the resection specimen, simple vein ligation is appropriate. […] For those patients with PAPVCs arising from pulmonary segments which will not be included in the resection, consideration of preoperative Qp/Qs and resection extent is essential when determining the need for PAPVC correction.

• #2 Total Anomalous Pulmonary Venous Connection (TAPVC) | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/total-anomalous-pulmonary-venous-connection-tapvc

  This defect must be surgically repaired in early infancy. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] This defect is almost always surgically repaired in early infancy or childhood. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] It’s rare when the TAPVC hasn’t been repaired in childhood, but most adults in this category are able to have surgery. […] Once the TAPVC has been repaired, it’s unlikely that more surgery will be needed. Rarely, an adult patient may have obstruction that may need to be corrected depending on the severity.

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

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.icoradiopaedia-icon-144.png&lang=gb

  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. […] Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

• #3 Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease in which one or more pulmonary veins drain into the systemic venous circulation. […] We discuss the challenges in the diagnosis and medical management of isolated PAPVC and highlight the clinical and hemodynamic indications for pulmonary vasodilators and diuretics. […] Surgical correction is recommended for patients with impaired functional capacity, RV enlargement, large net left-to-right shunt with Qp:Qs 1.5:1, pulmonary artery (PA) systolic pressure less than 50% of the systemic pressure, and pulmonary vascular resistance (PVR) less than one-third of the systemic vascular resistance (SVR). […] Medically, clinical observation and/or pulmonary artery vasodilator therapy with guanylate cyclase inhibitors, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors have been reported.

• #3 Management of partial anomalous pulmonary venous connections in patients requiring pulmonary resection: a case report and systematic review – Singhal – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17661/html

  Partial anomalous pulmonary venous connections (PAPVCs) are rare congenital anomalies that are frequently asymptomatic in adults. […] When PAPVCs are encountered in the patient requiring pulmonary resection, improper management can result in fulminant right-heart failure and death. […] Management of PAPVCs in the setting of pulmonary resection requires careful consideration of several factors including PAPVC anatomy, preoperative cardiopulmonary physiology (as assessed by Qp/Qs), and the extent of planned pulmonary resection. […] For PAPVCs that arise from a pulmonary segment that will be included in the resection specimen, simple ligation is adequate. […] When a PAPVC arises from an anatomic segment which will not be included in the resection, the management approach is more challenging and requires a pre-resection assessment of Qp/Qs and consideration of resection extent.

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

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

  If you arent experiencing symptoms or if there isnt much mixing of oxygen-rich and oxygen-poor blood, you may not need surgery to treat this condition. […] Surgery may be needed if there is a large amount of oxygen-rich and oxygen-poor blood mixing in the heart, or if you have had many pulmonary infections. If youre having surgery for another heart condition, surgeons may repair this heart defect at the same time. […] To repair this defect, surgeons generally reconnect the pulmonary veins to the left atrium. Surgeons will also close the hole between the atria, if there is one. […] Children will need regular follow-up appointments with paediatric cardiologists. Adults with this heart defect will need lifelong care and regular follow-up appointments with doctors trained in congenital heart conditions (adult congenital cardiologists) to monitor for any changes in their condition.

• #3

  https://link.springer.com/article/10.1007/s00246-021-02583-4

  Alternative options for the correction of partial anomalous pulmonary venous connection (PAPVC) have been proposed. […] A selective customized strategy to address PAPVC taking into account atrial shunt (AS) and growth potential was pursued. […] A customized approach reserves the advantages of each technique tailored to patients needs. […] Expanding surgical capacity with favorable outlook for all PAPVC variations, irrespective of association with AS, can maximize efficiency and reproducibility paired with the lowest morbidity. […] Key elements of any type of PAPVC repair, with or without ASD, should target closure of the inter-atrial communication to create unobstructed drainage of the anomalous PV channel to the left atrium (LA), through a native or surgically created ASD, and of the SVC to the RA.

• #3

  https://journals.lww.com/aopc/fulltext/2017/10010/interventional_therapy_for_partial_anomalous.16.aspx

  A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein in addition to its normal connection to the left atrium. […] The presence of normal connection to the LA, in addition to LACV, makes it an ideal candidate for interventional therapy. An interventional closure of patent LACV was, therefore, contemplated. […] This case illustrates the feasibility of complete interventional cure of select cases of partial anomalous pulmonary venous drainage with dual drainage.

• #3 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. […] 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. […] Surgical management is reserved for persons with a hemodynamically significant left-to-right shunt (a ratio of pulmonary to systemic flow greater than 2:1), those with recurrent pulmonary infections, or during surgical repair of other major cardiac lesions. […] It is, therefore, crucial that once diagnosed, patients maintain follow-up with a cardiologist to monitor for signs of cardiac dysfunction.

• #3 Anomalous Pulmonary Veins (APVR) | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/heart-institute/services-conditions-treated/anomalous-pulmonary-veins/

  Patients with PAPVR are usually diagnosed as toddlers or older children and have minimal symptoms. […] Thankfully, all forms of PAPVR can now be safely addressed with surgery in patients of all sizes and ages, and they can go on to live normal, active healthy lives. […] There are a variety of different techniques to re-route the venous flow back to the left atrium and since most patients also have an ASD, this is closed as well. […] From 2009-2013 alone, we repaired 27 patients with PAPVR with a 100 percent success and survival rate. […] Sometimes, patients with repaired PAPVR or TAPVR return to their doctors months or years later having developed some additional blockages in the repaired veins. Although further surgery is sometimes necessary, many of these patients can be managed with catheterization, an outpatient procedure that does not require an incision.

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

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

  A 49-year-old female following transesophageal echocardiogram (TEE) to investigate an undiagnosed cardiac murmur and progressive dyspnea on exertion was found to have isolated left-sided anomalous pulmonary venous connection of all veins draining into the coronary sinus. This unique lesion required novel repair by coronary sinus unroofing and atrial septal closure. […] 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.

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