Total anomalous pulmonary venous connection. Repair using deep hypothermia and circulatory arrest in 44 consecutive infants.

Abstract

Forty-four consecutive infants aged from 3 days to 10 months underwent repair of total anomalous pulmonary venous connection using deep hypothermia with circulatory arrest. There were eight (18%) early hospital deaths. Using multivariate analysis no significant association could be shown between early mortality and age or weight at operation, preoperative pulmonary or systemic pressure, and preoperative condition for patients undergoing operation during the most recent five year period. Late pulmonary venous obstruction developed in four (11%) of the survivors and all of these patients died.

A suggested new surgical classification for mixed totally anomalous pulmonary venous connection

“…The indications of pre-operative catheterization in most studies were the anatomy being unresolved by echocardiography, the need to characterize pulmonary venous obstruction, or the exclusion of major associated cardiac anomalies. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][19][20][21][23][24][25][26][27]29,30,[32][33][34][36][37][38][39][40][41][42][43][44][46][47][48][49][50][51][52][53] Cross-sectional echocardiography and cardiac catheterization provided the necessary diagnostic information, and defined the anatomy before surgery, in 139 patients. Echocardiography was the only diagnostic study in 43 patients.…”

“…Specific reference has been made previously, nonetheless, to the presence of heterotaxy syndrome, or isomerism of the atrial appendages, which includes absence of the coronary sinus in the setting of right isomerism, bilateral superior caval veins, double inferior caval veins, obstruction of the right and left ventricular outflow tracts, coarctation of the aorta, interrupted aortic arch, a functionally univentricular heart, perimembranous ventricular septal defect, pulmonary stenosis, atrioventricular septal defect with common atrioventricular junc-tion, tetralogy of Fallot, double outlet right ventricle, mitral atresia, tricuspid atresia, discordant ventriculo-arterial connections, congenitally corrected transposition, double outlet left ventricle, pulmonary atresia, and hypoplastic left heart syndrome. 1,[3][4][5][6][7][8][9][10][11][13][14][15][16][21][22][23][24]28,31,33,35,[46][47][48] Surgical approach and management Surgical management has evolved with time. Depending on the anatomical locations, and the preference of the surgeon, the lesions have been approached through a left thoracotomy with incision across the sternum, right thoracotomy, or bilateral thoracotomies, mostly in older series.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][19][20][21][23][24][25]27,29,30,[32][33][34][36][37][38][39][40][41][42][43][44]46,[48][49][50][51][52][53] Repair under deep hypothermia and circulatory arrest was used at the beginning of the experience in some centres. 3,[4][5][6][7][8]13,20,32,35,50 At present, routine hypothermic cardiopulmonary bypa...…”

“…The anatomical variants were bizarre, and required pulmonary venous rechannelling using individualized surgical techniques. 5,13,45,[51][52][53][54]…”

“…For those patients with drainage of the three pulmonary veins into the coronary sinus, rechannelling can be accomplished by one of the three techniques, namely, unroofing of the coronary sinus and patch closure of the atrial septal defect, the technique suggested by Van Praagh and colleagues, or that proposed by Mee and his associates. [1][2][3][4][5][6][7][8][9][10][11][12][13]24 > For patients with three pulmonary veins draining at or above the junction of the superior caval vein and the right atrium, or if the right superior pulmonary vein drains cephalad into superior caval vein and the other 3 pulmonary veins drain directly into right atrium, rechannelling can be accomplished by modifying the technique proposed by Van Son and his associates. 5,6,9,21,25,51 > For patients with a mixture of supracardiac and cardiac drainage, with right upper and right middle pulmonary veins draining directly into superior caval vein, rechannelling was accomplished by the technique described by Berdat and colleagues.…”