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), pulmonary artery (PA) systolic pressure less than 50% systemic pressure, and pulmonary vascular resistance less than one third of systemic resistance 1.
Diagnosis and Evaluation
Diagnosis of PAPVR involves:
- Echocardiography to identify abnormal connections
- Cardiac MRI or CT angiography to evaluate pulmonary venous connections and quantify shunt magnitude
- Invasive hemodynamic assessment can be useful in higher-risk patients to measure pressures, quantify shunt magnitude, and assess pulmonary arterial resistance and responsiveness to pulmonary vasodilator therapy 1
Treatment
Treatment depends on severity, with surgical correction recommended for significant left-to-right shunting causing right heart enlargement or symptoms.
- Surgical repair typically involves redirecting the anomalous veins to the left atrium using patches or baffles
- The timing of surgery is based on symptoms and hemodynamic significance rather than age alone
- Regular cardiology follow-up is essential after diagnosis, even for asymptomatic patients, to monitor for development of pulmonary hypertension or right heart dysfunction
Key Considerations
- Pulmonary hypertension is a risk factor for adverse outcomes with surgery
- Repair of a scimitar vein can be technically challenging with a greater risk of postoperative vein thrombosis than simpler anomalous pulmonary vein abnormalities 1
- Asymptomatic adults with RV volume overload and a net left-to-right shunt sufficiently large to cause physiological sequelae may benefit from surgery 1
From the Research
Definition and Prevalence
- Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly where one or more pulmonary veins connect to the right atrium instead of the left atrium 2, 3.
- The prevalence of PAPVR is estimated to be around 0.4-0.7% of the general population 3.
Clinical Presentation
- Patients with PAPVR may be asymptomatic or present with symptoms such as recurrent pulmonary infections, congestive heart failure, or exertional dyspnea 2, 3, 4.
- The severity of symptoms depends on the extent of the anomalous connection and the presence of associated cardiovascular anomalies 2, 3.
Diagnosis and Treatment
- Diagnosis of PAPVR is typically made using imaging studies such as echocardiography, computed tomography, or magnetic resonance imaging 3, 5, 6.
- Surgical correction is the treatment of choice for PAPVR, with the goal of redirecting the anomalous pulmonary vein to the left atrium 2, 3, 4, 6.
- Surgical techniques may vary depending on the location and extent of the anomalous connection, as well as the presence of associated anomalies 2, 3, 4, 6.
Surgical Outcomes
- Surgical correction of PAPVR has been shown to be effective in reducing symptoms and improving cardiac function 2, 3, 4, 6.
- Complication rates are generally low, with some studies reporting no early or late deaths 3, 4, 6.
- Long-term follow-up is necessary to monitor for potential complications such as pulmonary vascular disease or right ventricular failure 3, 4.
Special Considerations
- PAPVR may be associated with other cardiovascular anomalies, such as atrial septal defects or sinus node dysfunction 2, 3.
- In patients with lung cancer, PAPVR may complicate surgical management and require special consideration during lobectomy 5, 6.
- Preoperative evaluation is crucial to determine the extent of the shunting and the presence of symptoms or underlying right heart strain or pulmonary hypertension 5, 6.