Scandinavian Journal of Thoracic and Cardiovascular Surgery

ISSN: 0036-5580 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/icdv19

Total Anomalous Pulmonary Venous Drainage Hans Rostad, Svein Sørland, Steinar Tjønneland & Tor Froipaker To cite this article: Hans Rostad, Svein Sørland, Steinar Tjønneland & Tor Froipaker (1979) Total Anomalous Pulmonary Venous Drainage, Scandinavian Journal of Thoracic and Cardiovascular Surgery, 13:1, 13-15, DOI: 10.3109/14017437909101779 To link to this article: http://dx.doi.org/10.3109/14017437909101779

Published online: 12 Jul 2009.

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Date: 24 April 2016, At: 12:28

Scand J Thor Cardiovasc Surg 13: 13-16, 1979

TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE

Downloaded by [RMIT University Library] at 12:28 24 April 2016

Hans Rostad, Svein S d a n d , Steinar Tjnnneland and Tor Froipaker

Ahstrtrcf. Twelve patients with total anomalous pulmonary venous drainage (TAPVD) underwent complete surgical correction. Six were of the supracardiac type, 2 were cardiac and 4 of the infracardiac type. Pulmonary hypertension due to pulmonary vein obstruction was present in 6 patients. There were 6 early deaths, which occurred on the table or soon after surgery. One patient died 8 months after the operation because of a marked obstruction of the pulmonary venous inflow. Early diagnosis and operative correction in these severely ill patients should not be delayed. Postoperative intensive care and longterm follow-up are of the greatest importance.

In patients with total anomalous pulmonary venous drainage (TAPVD), all the pulmonary venous blood drains into the right atrium, either directly or via the coronary sinus, superior vena cava or inferior vena cava. A patent foramen ovale or atrial septal defect allows mixed blood in the right atrium to enter the systemic circulation. TAPVD comprises approximately 1.5-292 of congenital anomalies (Darling, Rothny & Craig, 1957; Bharati & Lev, 1973). Although first described almost two centuries ago (Wilson, 1798, cit. Delisle. Ando, Calder, Zuberbuhler, Rochenmachen, Alday, Mangini, Van Praagh & Van Praagh, 1976), TAPVD still constitutes a difficult challenge both to the cardiologist and the cardiac surgeon. MATERIAL AND METHODS The first surgical correction of this defect in Norway was carried out in 1969. Until March 1977. 12 patients had been operated on, 4 females and 8 males. The distribution of the patients into the 3 main types described by Darling et al. (1957) as well as the number of deaths are seen from Table I . In the supracardiac type, the pulmonary veins from both lungs drain into a common transverse trunk posterior to the heart. This vessel drains into the left innominate vein via a vertical anomalous vein, as in our 5 patients, or sometimes directly into the superior vena cava. In the cardiac type, the pulmonary veins connect

with the right atrium or drain into the coronary sinus. In the infracardiac type, the pulmonary venous blood drains from the common retrocardiac trunk into a descending vein that transverses the diaphragm to enter the portal vein, as was the case in all our 4 patients, or into the inferior vena cava. Principal symptoms were those of pulmonary congestion and cardiac failure. Obvious obstruction of the anomalous pulmonary venous return was present in 6 patients, all 4 with the infracardiac type, one with the supra- and one with the cardiac type of TAPVD. After catheterization. all patients were operated on under extracorporeal circulation and varying degrees of hypothermia. In 50% of the cases circulatory arrest was also used. The principal surgical procedure was anastomosis between the common venous trunk and left atrium and a simultaneous closure of the atrial septal defect. The relevant data of the patients are summarized in Tables 11-IV, including some important facts from the pre-operative haemodynamic study as well as aspects of the surgical technique. Three of the 6 patients with the suprrrctirtlicrc, type of TAPVD died (Table 11). Patient 2 had a marked pulmonary hypertension due to obstruction of the pulmonary venous return. Furthermore, a large patent ductus arteriosus was revealed at angiography. -

Total anomalous pulmonary venous drainage.

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