Abstract
An anomalous origin of the left coronary artery from the pulmonary artery, or Blund – White – Garland syndrome, is a rare congenital heart disease in which an anatomically correctly formed left coronary artery extends away from the pulmonary artery. Most patients with this defect die from progressive left ventricular failure during the first year of life, and the rest may suddenly die in adolescence or adulthood from acute coronary insufficiency. Several surgical methods of correction of the defect have been proposed, but a small number of observations do not allow one to express unequivocally in favor of one of the methods of performing the operation. We present our own observation of a surgical correction of a defect in a teenager. We performed off-pump aortocoronary bypass surgery of the anterior interventricular artery, and separation of the left coronary artery from the pulmonary artery by ligation, with the stitching of the left coronary artery. The issue of the expediency of tying hypertrophied collaterals between right coronary artery and left coronary artery systems was discussed with the purpose of reducing competitive blood flow after performing left coronary artery shunting. Experience in performing coronary artery bypass surgery without artificial circulation made it possible to perform an off-pump operation.
Highlights
Аномалии коронарных артерий относятся к редким врождённым порокам сердца
An anomalous origin of the left coronary artery from the pulmonary artery, or Blund – White – Garland syndrome, is a rare congenital heart disease in which an anatomically correctly formed left coronary artery extends away from the pulmonary artery. Most patients with this defect die from progressive left ventricular failure during the first year of life, and the rest may suddenly die in adolescence or adulthood from acute coronary insufficiency
We present our own observation of a surgical correction of a defect in a teenager
Summary
Одним из некоторых вариантов врождённой аномалии коронарных артерий является аномальное отхождение левой коронарной артерии от лёгочного ствола (ALCAPA – anomalous left coronary artery from the pulmonary artery), или синдром Бланда – Уайта – Гарланда. Приводим собственное наблюдение операции коронарного шунтирования (КШ) на работающем сердце у подростка с ALCAPA. ПКА ретроградно через коллатерали заполняет бассейн ЛКА, которая отходит от ствола ЛА. При контрольной ЭхоКГ давление в правом желудочке составило 26 мм рт. Cooley впервые у больного с ALCAPA выполнил аортокоронарное шунтирование аутовеной ствола ЛКА с перевязкой артерии в месте впадения в ЛА [5]. Порок протекал бессимптомно и был заподозрен при диспансерном наблюдении по изменениям на электрокардиограмме и выявленной перегрузке правых отделов сердца по данным ЭхоКГ. При ККГ выявлены увеличенная в размерах ПКА, сформированные коллатерали из ПКА в ЛКА со сбросом крови из ЛКА в ЛА.
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