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The Journal of Thoracic and Cardiovascular Surgery, Vol 85, 686-690, Copyright © 1983 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
A Mazzucco, G Rizzoli, A Fracasso, G Stellin, C Valfre, P Pellegrino, U Bortolotti and V Gallucci
Twenty patients presenting with total anomalous pulmonary venous connection
(TAPVC) in infancy underwent cardiac repair. Four had associated major
intracardiac anomalies (complex TAPVC) and 16 had isolated TAPVC. All
patients with complex lesions died during or shortly after the operation;
they all had pulmonary venous obstruction (PVO). The associated
malformations were critical pulmonary stenosis (one case), multiple
ventricular septal defects (VSDs) (one case), mitral atresia (one case),
and asplenia syndrome with common atrioventricular valve, double-outlet
right ventricle, and pulmonary stenosis (one case). Among the patients with
isolated lesions there were two surgical deaths, both in infants less than
1 month of age, with severe PVO and subdiaphragmatic drainage. Statistical
analysis of these data shows a strongly incremental risk of surgical death
due to the presence of associated malformations. Analysis of just isolated
TAPVC shows a possible incremental effect due to neonatal age (less than 1
month) and PVO, these two factors being not clearly separated. There have
been no late deaths and no late complications among the 14 survivors
followed from 7 months to 10 years (mean 44 months). An aggressive surgical
approach regardless of the age, degree of pulmonary hypertension, and type
of anatomic connection is advised for isolated TAPVC. Some caution is
recommended for complex TAPVC, in which a much higher risk is anticipated,
particularly when a palliative pulmonary vein-to-left atrium anastomosis is
performed.
ARTICLES
Experience with operation for total anomalous pulmonary venous connection in infancy
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