Total anomalous pulmonary venous connection in a 9-year-old girl at Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease in which all the four pulmonary veins drain into the right atrium instead of the left. Without surgical intervention, 80% of the patients die before the age of 1 year. We report a 9-year-old girl with unrepaired supracardiac TAPVC complicated by severe pulmonary artery hypertension. The patient was managed conservatively including the use of pulmonary antihypertensive (sildenafil), with significant improvement. She is currently on follow-up at our pediatric cardiology clinic. TAPVC requires surgical intervention in early infancy to prevent the onset of pulmonary hypertension, which may contraindicate surgery. High index of suspicion and improved diagnostic skill will enhance early diagnosis and enable timely intervention.

Keywords

• Nigeria
• Sokoto
• total anomalous pulmonary venous connection