Interrupted aortic arch: Is it always a neonatal emergency?

Abstract

Interrupted aortic arch (IAA) is a rare congenital ductal-dependent heart disease. It commonly presents in infancy following spontaneous closure of the patent ductus arteriosus (PDA) [1] , [2] . Few cases have been reported in pediatric age [3] . We present the case of 13 years old boy with IAA and a large ventricular septal defect (VSD), a restrictive PDA and an Eisenmenger syndrome. A thirteen years old Tunisian boy presented to our consultation with malaise, headache and weakness of his legs. He reported reduced exercise tolerance since two years. There was no family history of congenital heart disease or sudden unexpected death ( Fig. 1 , Fig. 2 ). Physical examinations noted a generalized cyanosis. Radial pulses were normal but his femoral pulses were weak. The systolic pressure difference between the upper and lower limbs was 40 mmHg. Transthoracic echocardiography noted a conal VSD with a posterior malalignment of the conal septum with right to left shunt. The short axis examination showed a large pulmonary tract and a restrictive PDA with an increased velocity of 3.3 m/s and right to left shunt. The high left parasternal examination with a slightly rightward angulation of the transducer demonstrated a complete interruption of aortic arch. Computed tomography revealed an IAA between the left common carotid artery and the left subclavian artery (type B) and a PDA (3.3 mm) between the left pulmonary artery (LPA) and the descending aorta. Pulmonary arterial hypertension was confirmed in cathlab with mean pulmonary arterial pressure (mPAP) at 100 mmHg. Following multidisciplinary team discussion, on regard of patient health state, Eisenmenger syndrome, the restrictive PDA and the contraindication to performing surgery, we discuss the percutaneous stenting of the PDA. Interrupted aortic arch is a rare congenital abnormality that presents in infancy. We presented the case of 13 years old boy with IAA, Eisenmenger syndrome and a restrictive PDA. Following multidisciplinary team discussion, giving the restrictive pattern of the PDA and the Eisenmenger syndrome, we discuss a percutaneous of the PDA.