TOTAL, UNCOMPLICATED, ANOMALOUS PULMONARY VENOUS CONNECTION

Abstract
Anatomic observations on 13 necropsy specimens of total, uncomplicated, anomalous pulmonary venous connection are presented. These cases accounted for 0.8% of all deaths and 3.9% of congenital cardiac deaths in a consecutive necropsy series. Neill's embryologic classification is recommended because it is suitable for clinical purposes. The pulmonary venous return in one of the cases drained directly to the right atrium (type 1); in six it drained via a left superior vena cava (type 3A); in three it drained via the coronary sinus (type 3B); and in three the pulmonary venous return drained via the portal system (type 4A). Findings common to all cases were: 1) interatrial communication; 2) enlarged right heart; 3) small left heart; 4) increased pulmonary circulation; and 5) normal aorta. Anatomic findings are related to their surgical implication. The disproportion in size and implied functional capacities of the right and left sides of the heart is stressed, and the authors agree with previously recommended surgical correction in multiple stages. Factors favoring surgical correction are: 1) The anomalous channel is so located that anastomosis with the left atrium is anatomically feasible. 2) The small left heart contains a reasonable, if not normal, number of myocardial fibers that should be capable of hypertrophy and increased function. 3) This anomaly is uncomplicated by other cardiac or extracardiac anomalies in most instances. All cases in this report were uncomplicated. 4) Pulmonary vascular changes in these infants are without scarring. 5) Myocardotomy in most instances can be limited to the atrial wall. Factors unfavorable to surgical correction are: 1) Age at death in this series varied from 15 days to 14 months. The risk of cardiac surgery in infants is well-known. 2) The anomalous veins are scarred vessels, and may predispose to thrombosis. 3) The size of the anastomosis is limited by the small size of the left atrium. Anomalous pulmonary venous connection must be excluded in all cases of left-to-right shunt at the atrial level. Anatomic variations that might lead to a proper diagnosis are summarized for the various types included in this study.

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