Partial Anomalous Pulmonary Venous Connection Treatment & Management

Updated: Dec 16, 2020
  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: Stuart Berger, MD  more...
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Treatment

Approach Considerations

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.

No limitation on activity is necessary in the pediatric patient.

No specific diet is recommended or prohibited.

Advise patient and parents regarding long-term risks if a large shunt remains unrepaired. If pulmonary hypertension has developed, outline the risks of pregnancy, including death.

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Surgical Care

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.

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). [8] Children with internal patch technique must be observed for obstruction of the SVC with SVC syndrome, sick sinus syndrome, obstruction of the pulmonary veins, and supraventricular tachyarrhythmias.

A study by Pace Napoleone et al of 59 patients who underwent intracardiac patch rerouting for PAPVC, including 14 who also had SVC patch enlargement, found the procedure to yield good results at medium-term follow-up. The investigators reported that at mean follow-up of 46 months, the rate of arrhythmias (including sinus node dysfunction) was comparable to that found in association with other techniques. In addition, all patients were asymptomatic, and 55 of them (93%) presented with sinus rhythm and were antiarrhythmic drug – free. [9]

Zubritskiy et al reported on the successful repair of supracardiac partial anomalous right upper and middle pulmonary venous connection in 21 pediatric patients. The Warden technique was performed through right-sided midaxillary thoracotomy with direct cardiopulmonary bypass cannulation and induction of ventricular fibrillation. No operative or early postoperative deaths or complications occurred, and at discharge, all patients were in sinus rhythm. [10]

Ait-Ali et al recommend techniques that avoid any manipulation on the superior cavoatrial junction in the surgical repair of PAPVC in pediatric patients. In a retrospective review of 70 patients who underwent surgical repair of PAPVC, investigators found ectopic atrial rhythm, as an expression of sinoatrial node disturbance, in 28.8% of the 49 pediatric patients. [11]

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.

Anticipate early extubation unless contraindications are recognized; these include excessive chest tube drainage, hemodynamic instability, and oversedation. Encourage early mobilization. Monitor for atrial flutter, atrial fibrillation, and sinus node dysfunction.

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Consultations

Consultations include the following:

  • Cardiologist

  • Cardiothoracic surgeon

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Long-Term Monitoring

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. With a significant shunt, the pulmonary artery pressures can be elevated, and pulmonary vascular resistance can increase with age.

Postoperatively, possible obstruction of the pulmonary veins and superior vena cava (SVC) should be evaluated with echocardiography.

Regular electrocardiography (ECG) and 24-hour ambulatory ECG are also indicated to monitor for atrial arrhythmias.

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