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Interruption of the aortic arch with patency of the arterial duct, discrete subaortic stenosis, restrictive ventricular septal defect and severe pulmonary hypertension—survival to adulthood and successful correction by balloon dilation and surgery

Published online by Cambridge University Press:  19 August 2008

Robin Joseph Pinto ,
Satyavan Sharma  and
Nemish Shah
Robin Joseph Pinto
Affiliation:
From the Department of Cardiology, Bombay Hospital and Medical Research Centre, Bombay
Satyavan Sharma*
Affiliation:
From the Department of Cardiology, Bombay Hospital and Medical Research Centre, Bombay
Nemish Shah
Affiliation:
Department of Cardiac Surgery, Bombay Hospital and Medical Research Centre, Bombay
*
Dr. Satyavan Sharma, 104, First floor, New Wing, Bombay Hospital and Medical Research Centre, 12 Marine Lines, Bombay 400 020, India. Tel. 91-22-2054532, 91-22-4932827; Fax. 91-22-2067676, 91-22-2080871.
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Abstract

We report a case of interruption of the aortic arch associated with patency of the arterial duct, restrictive ventricular septal defect, obstruction of the left ventricular outflow due to a discrete subaortic shelf and severe pulmonary arterial hypertension. The patient survived to adulthood, when a complete angiographic diagnosis was made. The subaortic obstruction was treated by percutaneous balloon dilation performed through a right brachial arteriotomy. Correction of the interrupted arch was carried out surgically using a woven Dacron graft from the aortic arch to the descending thoracic aorta associated with ligation of the duct. Hemodynamic studies after one year revealed a marked decrease in pulmonary vascular resistance and a sustained reduction in the gradient across the left ventricular outflow tract. The case is of interest in the light of the unusual survival and the success of staged management of this complex situation.

Keywords

Aortic arch interruptionsubaortic stenosisballoon dilatation
Type
Brief Report
Information
Cardiology in the Young , Volume 5 , Issue 4 , October 1995 , pp. 367 - 369
Copyright
Copyright © Cambridge University Press 1995

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