Issn Print: 0265-0215

Publication Date: 2007/06/01


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Transoesophageal Echo (TOE) for adult type anomalous origin of the left coronary artery from the pulmonary artery; Bland-White-Garland syndrome: P-119

A. E. Van Rensburg; M. Meineri; K. Jacek; A. Vegas; J. Janson

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Author Information: Toronto General Hospital, University Health Network, Toronto, Ontario, Canada, 2Department of Cardiothoracic Surgery, Stellenbosch University, Cape Town, South Africa

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Excerpt

Introduction: Adult type anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly occurring in approximately 1 in 300,000 living and stillbirths (0.25-0.5% of all congenital heart disease).
It is characterized by a left main coronary artery arising from the main pulmonary artery and dilatation of the right coronary artery. It leads to myocardial ischaemia, ventricular failure, mitral insufficiency, congestive heart failure and death.
Only a minority of the patients survive early childhood without surgical intervention (90% mortality in first year of life).
Case Report: A 29-yr-old male patient presented with a history of increasing shortness of breath, congestive heart failure and severe mitral insufficiency. TOE performed after induction of anaesthesia showed:
The procedure consisted in a repair of the mitral valve and corrective coronary surgery (pericardial patch closure of ostium in the pulmonary artery, left internal mammary artery bypass to the left coronary system). Post cardiopulmonary bypass TOE showed:
Weaning from cardiopulmonary bypass was achieved on minimum inotropic support.
The post-operative course was uneventful.
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