Pediatric Cardiology and Cardiac Surgery
Vol.26 No.2 2010 (144-152)

Satoshi Yasukochi

Department of Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan


Total anomalous pulmonary venous connection (TAPVC) is a cardiac malformation in which there is no direct connection between the left atrium and any pulmonary veins: all pulmonary veins connect to the systemic venous system or right atrium The incidence of TAPVC is 1.5–3% of all congenital heart disease. The many cases with TAPVC need emergent surgery in neonatal and early infantile period because of hypoxemia and low cardiac output syndrome. Diagnosis is often made by echocardiography to determine the drainage site of an anomalous common pulmonary vein (CPV), which can be classified as a supracardiac, paracardiac, or infracardiac type of TPAVC (Darling classification). The urgency of surgical correction for TAPVC is based on the degree of pulmonary venous obstruction and restriction of inter-atrial communication in addition to the clinical symptoms of heart failure and hypoxia. The surgical outcome of TAPVC is generally stable if the case is not associated with any other cardiac malformation such as asplenia. However, the surgical results of TAPVC with asplenia or TAPVC with postoperative pulmonary venous obstruction are poor. The role of the pediatric cardiologist in the perioperative management is to diagnose the precise anatomy and hemodynamics by using multiple imaging modalities. The pediatric cardiologist must send patients with TAPVC who are in good condition to surgery at the right time.