[Cyanotic congenital heart disease. Part 1]

This section deals with subgroups of cyanotic complex congenital heart diseases, most present early in life during neonatal period or early infancy. And some present with ductal dependency for survival. Here included are the complete transposition of the great arteries (TGA) [or Taussig-Bing anomaly] with/without aortic arch obstruction, total anomalous pulmonary venous connection (TAPVC), pure pulmonary atresia/stenosis (PPA/PPS) [pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis], hypoplastic left heart syndrome (HLHS) and its variants. In d-TGA cases except those with left ventricular outflow obstruction, primary Jatene procedure with/without concomitant repair of the aortic arch obstruction is the procedure of choice with good surgical and long-term results although acceptable rate of re-intervention is mandatory. In TAPVC, primary repair usually goes with good long-term result, although minority of the patients eventually develops for midable complication, postoperative pulmonary venous obstruction. Sutureless pericardial repair has been developed for coping with and later for prevention of this complication with promising mid-term results. Its introduction in primary repair especially in complex cases appears around the corner. HLHS and its variants has been the most challenging group of patients requiring staged approach starting with Norwood procedure or bilateral pulmonary artery banding. Right ventricle-to-pulmonary artery conduit, introduced as a Japanese innovation, has contributed marked improvement of surgical results although further refinements surely are necessary. Patients with PPA/PPS have a wide range of clinical pictures with intrinsic problems of right ventricle, tricuspid valve and associated coronary artery abnormalities. Adequate use of single ventricle physiology in many cases offers fair surgical results.