Total anomalous pulmonary venous connections

Abstract

Aim: To asses the clinical presentation, follow-up of the rare type of total anomalous pulmonary venous connection (TAPVC) according to their anatomic subtype: supracardiac, cardiac, infracardiac or mixed drainage. Matherial and methods: In the last 3 years we diagnosed 5 cases of TAPVC, aged 1 week –5 years. All the cases were diagnosed through echocardiography, 1 case was confirmed also by MRI, other 2 during surgery. Results: Two cases had isolated TAPVC with atrial septal defect of secundum type, and 3 had complex cardiac diseases -complete cushion defect with imbalanced ventricles (1), double outlet right ventricle (2) with severe pulmonary stenosis/atresia, associating heterotaxia – asplenia (Ivemark syndrome). Concerning the anatomic type of the venous connections 3 had supracardiac drainage by a common venous collector, 1 mixed (cardiac through coronary sinus and supracardiac), 1 infracardiac. The isolated TAPVC were both operated in the moment of the diagnosis, with good results. One of them was diagnosed only at 8 month and still has a residual pulmonary hypertension. The other 3 cases were not possible to be operated because of complex malformations of the heart, 2 died shortly after diagnosis. Conclusion: Although a rare heart defect TAPVC is important to diagnose as early as possible. Echocardiography is a sure method for diagnosis. Although in all cases the cardiac malformation was obvious shortly after birth, a correct diagnosis was made late in 3 cases (8 month –5 years). Late diagnosis and surgery is associated with residual pulmonary hypertension. Isolated TAPVC has an excellent prognosis when operated in time. In Ivemark syndrome only paleative surgery techniques can be applied, and the mortality remains high even in experienced centers.