[Total anomalous pulmonary venous connection].
The severity of symptoms in total anomalous pulmonary venous connection (TAPVC) depends primarily on the degree of pulmonary venous obstruction (PVO). With severe PVO, patients can present in extremis with severe cyanosis, respiratory distress, and acidosis within the first few hours of life. The diagnosis is usually established by two-dimensional echocardiography. Surgical repair of TAPVC has involved a number of techniques using various strategies of cardiopulmonary bypass and circulatory arrest, methods of cardioplegia, sites of venous cannulation, and cavitary exposures. Recent clinical investigations have uncovered some long-term negative effects of deep hypothermia and circulatory arrest strategies. Our current strategies for surgical management of TAPVC are 1) using moderate hypothermic cardiopulmonary bypass with bicaval venous cannulation combined with antegrade cardioplegia strategy and 2) performing the side-to-side anastomosis between the confluence of pulmonary veins and the left atrium by right side approach. The critical parts of this anastomosis are 1) making incisions of the confluence of the pulmonary veins and the left atrium to avoid the distortion of the anastomotic site and 2) performing the anastomosis without "purse string" the suture line. The prognosis of the patients with recurrent PVO is still developing; therefore great care should be taken at the initial procedure to avoid obstruction.
Kagisaki
Koji
K
Department of Cardiovascular Surgery, National Cardiovascular Center, Suita, Japan.
Yagihara
Toshikatsu
T
D000714:Anastomosis, Surgical; D006348:Cardiac Surgical Procedures / Q000379:methods; D006330:Heart Defects, Congenital / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery*; D011668:Pulmonary Veno-Occlusive Disease / Q000209:etiology / Q000601:surgery