From the Guidelines
Surgical correction is recommended for patients with partial anomalous pulmonary venous return (PAPVR) when functional capacity is impaired and right ventricular (RV) enlargement is present, with a net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥ 1.5:1), as stated in the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1.
Diagnosis and Evaluation
The diagnosis of PAPVR involves the use of imaging techniques such as cross-sectional imaging with CMR or CTA, which is ideal for delineating pulmonary venous connections, as noted in the 2018 AHA/ACC guideline 1. Echocardiography is also an important part of the evaluation and may identify the anomalous veins, particularly in patients with excellent acoustic windows.
Treatment
Treatment depends on the severity of the shunt and associated symptoms. Surgical repair is recommended for patients with significant shunting (pulmonary-to-systemic flow ratio >1.5:1), right ventricular enlargement, or symptoms like exercise intolerance, shortness of breath, or heart failure, as recommended in the guideline 1.
Surgical Options
The surgery involves redirecting the anomalous veins to the left atrium, often using patches or baffles. For asymptomatic patients with minimal shunting, watchful waiting with regular cardiac monitoring may be appropriate.
Follow-Up
Patients should undergo regular echocardiographic follow-up to monitor for development of pulmonary hypertension or right heart enlargement, as suggested in the guideline 1. PAPVR often coexists with other cardiac defects, particularly atrial septal defects, which may require simultaneous repair.
Key Considerations
- The condition results from abnormal embryological development of the pulmonary veins, which fail to connect properly to the left atrium during fetal development.
- Pulmonary hypertension is a risk for adverse outcomes with surgery, as noted in the guideline 1.
- Surgical repair of a scimitar vein includes direct reimplantation of the scimitar vein into the left atrium, conduit placement to the left atrium, or intracaval baffling, and can be technically challenging with a greater risk of postoperative vein thrombosis than is associated with more common and simpler anomalous pulmonary vein abnormalities, as stated in the guideline 1.
From the Research
Definition and Prevalence
- Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart disease where one or more pulmonary veins connect to the right atrium or one of its tributaries instead of the left atrium 2, 3.
- The prevalence of PAPVR is not well established, but it is considered a rare condition, with some studies reporting a small number of cases 2, 3.
Symptoms and Diagnosis
- Symptoms of PAPVR can range from asymptomatic to recurrent pulmonary infections, moderate congestive heart failure, and exertional dyspnea 2, 3, 4.
- Diagnosis of PAPVR can be made through echocardiography, catheterization, and nuclear magnetic resonance imaging 3, 5.
Surgical Treatment
- Surgical treatment of PAPVR involves correcting the anomalous venous connection and redirecting the pulmonary veins to the left atrium 2, 3, 6, 4, 5.
- Different surgical techniques can be used, including high ligation and division of the anomalous vein, anastomosis to the left atrium, and creation of an atrial septal defect 2, 3, 4.
- A customized approach to surgical repair, taking into account the patient's specific anatomy and needs, can help maximize efficiency and minimize morbidity 5.
Outcomes and Complications
- Surgical correction of PAPVR can be effective in relieving symptoms and improving outcomes, with some studies reporting excellent long-term results 2, 3, 4, 5.
- Complications of surgical correction can include obstruction of the superior vena cava, arrhythmias, and thrombogenic complications 3, 4.
- A good hemostasis at the site of the patch, leaving the sinus node free of compression, and performing the atrial incision at the posterior wall can help decrease the risk of arrhythmias 3.