Circulation, Vol 57, 1026-1030, Copyright © 1978 by American Heart Association
WW Pinsky, PC Gillette, DF Duff, N Wanderman, JH Morriss, CE Mullins and DG McNamara
Only two cases have been reported previously of the association of
ventricular septal defect (VSD) with anomalous origin of the left coronary
artery (ALCA) arising from the pulmonary artery. The purpose of this paper
is to present two additional cases, to describe the pathophysiology, and to
emphasize how the clinical course of this combination of defects differs
from that of isolated ALCA. Patients with both of these anomalies present
in infancy with manifestations only of a large left-right ventricular shunt
and pulmonary hypertension. Initially the ALCA is well perfused from the
high pressure in the pulmonary artery. In these instances in which the
pulmonary artery pressure subsequently decreased because of spontaneous
reduction in size of the VSD, the left coronary arterial system became less
well perfused. Because of this decreased perfusion in association with the
left ventricular myocardial stress initially caused by volume overload,
myocardial ischemia and ultimately infarction occurred. Early
identification and repair of the anatomic abnormality could prevent
irreversible myocardial damage.
ARTICLES
Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect
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G. Shanmugam, A. J. McLennan, J. C. Pollock, and K. J.D. MacArthur Anomalous Left Coronary Artery, Ventricular Septal Defect, and Double Aortic Arch Ann. Thorac. Surg., July 1, 2005; 80(1): 334 - 336. [Abstract] [Full Text] [PDF] |
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