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The association of pulmonary atresia–major aortopulmonary collaterals with total anomalous pulmonary venous drainage combines two severe congenital malformations. This association is usually found in conjunction with heterotaxy syndrome and single ventricle physiology. Patients with this combination are rare and the information regarding natural history as well as surgical outcome is very scarce. The prognosis in the absence of surgical treatment is probably extremely poor. The only chance to provide a satisfactory late outcome is to achieve a Fontan circulation by a staged surgical approach. Two goals must therefore be reached: (1) to reconstruct a normal low resistance pulmonary arterial bed and (2) to provide an unobstructed pulmonary venous drainage. This clearly represents a major surgical challenge. The data reported by Mainwaring and associates [1Mainwaring R.D. Mohan Reddy V. Reinhartz O. Punn R. Tacy T. Hanley F.L. Surgical results in patients with pulmonary atresia–major aortopulmonary collaterals in association with total anomalous pulmonary venous connection.Ann Thorac Surg. 2011; 92: 1756-1760Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar] show that this goal can be achieved in a reasonable number of patients by an aggressive initial approach which includes repair of anomalous pulmonary venous drainage and complete unifocalization procedure through a midline incision. In a series of 9 patients, 3 had successful Fontan completion, 1 (with cavopulmonary shunt) was a suitable candidate for Fontan and 1 (after initial palliation) might become a good candidate. These encouraging results deserve some comments. The current study is coming from a surgical center with exceptional expertise in the management of pulmonary atresia–major aortopulmonary collaterals and, particularly, with one-stage complete unifocalization procedure. Achieving such outstanding results, particularly when complete unifocalization must be performed very early in life because of associated obstructed total anomalous pulmonary venous drainage, is most probably very difficult to reproduce. Even if they are encouraging, the results are far from optimal. A significant number of patients (at least 4 of 9 in the present series) still have a poor outcome. Further information is needed to identify potential subsets with incremental risk factors. The current data should be kept in mind and taken into consideration at the time of prenatal counseling or postnatal information of the parents. Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous ConnectionThe Annals of Thoracic SurgeryVol. 92Issue 5PreviewPulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects. Full-Text PDF