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The Journal of Thoracic and Cardiovascular Surgery, Vol 98, 1018-1028, Copyright © 1989 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
FJ Puga, FE Leoni, PR Julsrud and DD Mair
From 1982 to 1987, 38 consecutive patients with pulmonary atresia and
ventricular septal defect underwent staged unifocalization procedures in
preparation for final intracardiac repair of the anomaly. Thirty-six
patients had concordant visceroatrial, atrioventricular, and
ventriculoarterial connections. A central pulmonary artery confluence was
present in 19 patients and absent in the remaining group. All patients with
central pulmonary arteries had inadequate peripheral pulmonary arterial
arborization. Systemic collateral arteries were present in all 38 patients.
The objectives of the unifocalization procedures were the interruption of
extracardiac sources of pulmonary arterial blood flow, the restoration of
segmental, lobar, and pulmonary arterial confluence, the replacement of
missing central pulmonary arterial branches, and the creation of a central,
accessible source of pulmonary arterial blood flow. A total of 54
unifocalization procedures were performed in the 38 patients. These
procedures included 85 permanent connecting anastomoses, 15 temporary
anastomoses to the ascending aorta, 13 angioplasty procedures, and 15
modified Blalock- Taussig shunts. Three patients died after unifocalization
(two early and one late). By the end of the study, eight patients were
still waiting for further unifocalization procedures or angiographic
assessment. Four patients were rejected for further surgical treatment
because of persistent, uncorrectable defects of the pulmonary arterial
arborization pattern. Twenty-three patients underwent complete intracardiac
repair with two deaths (one early and one late). Postrepair, intraoperative
right ventricular/left ventricular systolic pressure ratio after complete
surgical repair ranged from 0.4 to 1.0 (mean = 0.63, standard deviation =
0.14). At the end of follow-up, 21 survivors of complete repair were free
of significant symptoms. Unifocalization procedures designed to improve the
pulmonary arterial arborization pattern of patients with pulmonary atresia
and ventricular septal defect with or without central pulmonary arteries
can prepare a significant number of these patients for successful
intracardiac repair of the anomaly.
ARTICLES
Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries. Experience with preliminary unifocalization procedures in 38 patients
Mayo Clinic, Rochester, MN 55905.
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