ABSTRACT
To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease. A case series. The Aga Khan University Hospital from January 1998 to December 2000. Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality. Forty four neonates met the inclusion criteria. Eleven babies [25%] had Tetralogy of Fallot or its variants. Other malformations were d-transposition of great arteries, tricuspid valve anomalies [tricuspid atresia and Ebstein`s anomaly], hypoplastic left heart syndrome, truncus arteriosus, total anomalous pulmonary venous return and complex congenital heart disease like single ventricle. Twenty eight [63.6%] neonates survived and 16 [36.4%] expired during hospital stay. Cause of death was surgical in 2 cases and medical problems in 14 babies. Tetralogy of Fallot or variants was the commonest cyanotic heart disease in neonates with frequency of 27.27%. Majority of neonates with congenital cyanotic heart disease showed survival with appropriate management