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The Journal of Thoracic and Cardiovascular Surgery, Vol 106, 880-885, Copyright © 1993 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
FM Lupinetti, TJ Kulik, RH Beekman 3d, DC Crowley and EL Bove
From January 1985 through January 1993, 41 patients less than 1 year of age
underwent operative correction of isolated total anomalous pulmonary venous
connection. There were 24 boys and 17 girls. The median age at operation
was 13 days (range 1 to 282 days) and weight was 3.6 kg (2.5 to 5.2 kg).
Locations of the connections were supracardiac in 19, cardiac in 9,
infracardiac in 11, and mixed supracardiac and cardiac in 2. Obstruction of
the pulmonary veins was severe in 24, mild in 3, and absent in 14.
Preoperative stabilization included mechanical ventilation for 15 patients
for a mean duration of 2 1/2 days and extracorporeal membrane oxygenation
for 1 patient for 1 day. All operations were performed with deep
hypothermia and circulatory arrest (mean arrest time 34 minutes).
Supracardiac connections were repaired by performing a side-to-side
anastomosis between the pulmonary venous confluence and the dome of the
left atrium through a superior approach between the superior vena cava and
the aorta. Coronary sinus connections were repaired by enlarging the atrial
septal defect and the coronary sinus communication with the left atrium and
closing the atrial defect with a large patch. Infracardiac repairs included
elevation and rotation of the heart to the right and an elongated
side-to-side anastomosis between the common venous confluence and the left
atrium. One patient died 1 week postoperatively of persistent pulmonary
hypertension. Another patient, who was supported by extracorporeal membrane
oxygenation before the operation, died 3 months after the operation as a
consequence of pulmonary lymphangiectasia. All other patients are alive and
well with a mean follow-up of 26 months (range 3 to 77 months). One patient
required two subsequent reoperations for persistent pulmonary venous
obstruction, and another patient had superior vena cava obstruction
necessitating reoperation. Operative treatment of total anomalous pulmonary
venous connection in infants can be performed with low mortality and an
infrequent need for reoperations.
ARTICLES
Correction of total anomalous pulmonary venous connection in infancy
Department of Surgery, University of Michigan School of Medicine, Ann Arbor.
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