Drenaje venosa pulmonar anómalo total. Técnicas y resultados

Abstract

The total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the absence of direct connection between the pulmonary veins and the left atrium.It may occur as an isolated lesion main subject of this review, or less frequently in the context of heterotaxy.Natural History in biventricular hearts without surgical repair is very poor: 50% mortality until the third month of life and survival without therapeutic intervention in one years old children about 20%.TAPVC most is characterized by the existence of a retropericardic pulmonary venous collector, where the pulmonary veins of both lungs converge, and that in turn drained via the other vein called “vertical vein” in a systemic vein tributary right atrium. According to the place to get the draining vein, they are called supracardiac, the most common, cardiac, infracardiac and mixed forms, the rare ones.Clinical presentation is variable in time and symptoms, depending whether there is obstruction to pulmonary venous return in the vertical vein, atrial septal defect restriction flow, or both. Tachypnea is the symptom more times present.The diagnosis, confirmed by echocardiography is itself indication for surgery. The presence or absence of obstructive symptoms determines the time of surgical repair and the preoperative management to improve the clinical situation.Technical options to repair the different forms of TAPVC and hospital and late follow-up results are reviewed.Advice on postoperative management are cited the most recent literature that provides information about the treatment and results of the most lethal late complication: anastomotic obstruction and/or pulmonary veins obstruction.