Definitive anatomic diagnosis is obtained by selective right and left pulmonary artery angiography observing pulmonary venous return for each lobe from each lung. Complications of catheterization include bleeding at the vascular entry site, infection, decreased pulses distal to an arterial entry site, and arrhythmia induction. Medical therapy is not indicated for asymptomatic patients. Heart failure that occurs in the adult can be managed with diuretics, cardiac glycosides, afterload reduction, and beta blockade. Arrhythmias should be treated appropriately.
Controversy remains regarding indications for surgical repair.
One school of thought claims that in view of the exceptionally low morbidity and mortality following this surgical procedure, all children should undergo repair. The usual approach is via a midline sternotomy and cardiopulmonary bypass. Surgical treatment of associated lesions may be necessary. No limitation on activity is necessary in the pediatric patient. Routine post operative care of the cardiac surgery patient.