A 6-year-old girl born of a consanguinous marriage, with an uneventful birth history presented with cyanosis, clubbing, and effort intolerance. Her physical examination revealed cyanosis (oxygen saturation 78%), clubbing, single second heart sound, and a grade II/VI ejection systolic murmur over the left parasternal region. Echocardiography (Philips HD7 XE System, Eindhoven, The Netherlands) revealed the presence of a large subaortic ventricular septal defect, overriding of aorta, aneurysm of the left sinus of Valsalva at the level of the left coronary artery and pulmonary atresia (Fig. 1). Parasternal shortaxis view at the aortic valve level demonstrated confluent pulmonary arteries and left coronary to pulmonary artery fistula with a giant aneurysm at Figure 1. Parasternal long-and short-axis views show a large subaortic ventricular septal defect (VSD) with pulmonary atresia (P At) and an aneurysm (An) at the left sinus at the origin of the left coronary artery (marked with red arrow).Figure 2.Parasternal short-axis view shows the aneurysm at the origin of left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery (PA) (marked with red arrow).

No abstract

Coarctoplasty with stenting is often an effective strategy in cases of recoarctation following surgical repair. The potential benefit of coarctoplasty in a patient with Eisenmenger's syndrome is unknown. We describe the case of a 21-year-old male who presented with claudication of lower limbs. He was known to have congenital heart disease, consisting of ventricular septal defect, patent ductus arteriosus (PDA), and coarctation of the aorta. Coarctation repair and PDA ligation had been done at two months of age. At the time of presentation for evaluation of claudication, echocardiography revealed severe coarctation and evidence of Eisenmenger's syndrome. This patient subsequently underwent balloon angioplasty and stenting of coarctation without any increase in cyanosis.