Patterns of Anomalous pulmonary Venous Connection as Seen at Care Hospital, Hyderabad - India.

Background: Anomalous pulmonary venous connection (APVC) is a rare, life threatening congenital heart disease presenting early in life. It may be partial (PAPVC) or total (TAPVC). PAPVC mainly involve the right pulmonary arteries and is associated with sinus venosus atrial septal defect with a low morbidity and mortality. TAPVC is classified into supracardiac, intracardiac, infracardiac and mixed types. It carries a high morbidity and mortality. The aim of this study was to determine the morphological patterns and surgical outcome of APVC at CARE hospital, Hyderabad-India.
Methods: This was a prospective study form January 2004 to December 2006. Study included all patients who underwent surgery for anomalous pulmonary venous connection and followed up to the time of discharge.
Results: A total of 65 patients with APVC were studied where the results revealed 60% had TAPVC and 40% had PAPVC. Mean age of presentation was 21.6 months for TAPVC compared to 135.2 months for PAPVC. Among all patients with PAPVC 84.6 % had sinus venosus atrial septal defect. Supra-cardiac and intra-cardiac anomalous were the commonest type of TAPVC representing 43.6% and 35.9% respectively. Among all patients with TAPVC 51.35% were associated with ostium secundum atrial septal defect, 74.4% had moderate to severe pulmonary hypertension. Overall mortality was 9.25%. Mortality within TAPVC group was 15.4% and none from PAPVC.
Conclusion: It was concluded that TAPVC present early in life and is associated with ostium secundum atrial septal defect, pulmonary hypertension, high morbidity and mortality. Accurate diagnosis and early correction improves the surgical outcome.