Partial Anomalous Pulmonary Venous Connection Treatment & Management

Updated: Jan 27, 2015
  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: Stuart Berger, MD  more...
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Treatment

Medical Care

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.

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Surgical Care

Definitive treatment for 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). [4] 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. [5]

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Consultations

Consultations include the following:

  • Cardiologist
  • Cardiothoracic surgeon
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Diet

No specific diet is recommended or prohibited.

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Activity

No limitation on activity is necessary in the pediatric patient.

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