Giant Aorto–Right Ventricular Fistula With Single Coronary Artery

Abstract

A 15-year-old boy with symptoms of dyspnea and fatigue since early childhood was referred to our institution for evaluation. Physical examination of the patient showed long, slender extremities and a pectus carinatum deformity of the chest (Figure 1A). His arm span–to-height ratio was 1.03, and his upper segment–to–lower segment ratio was 0.82. He had blood pressure of 126/70 mm Hg, with a heart rate of 96 bpm; a grade 4/6 continuous murmur was present in the right lower parasternal area. A 12-lead ECG showed right bundle-branch block (Figure 2), and chest radiography (posteroanterior view) revealed mild cardiomegaly. Radiography of the thoracolumbar spine of the patient disclosed a mild degree of scoliosis (inset, Figure 1A). Two-dimensional echocardiography (parasternal long-axis view) showed a large fistula (2 cm in diameter) arising from the right aortic sinus (Figure 1B; online-only Data Supplement Movie I). A tilted apical 4-chamber view showed a turbulent jet (velocity 3.5 m/s) at the free wall of the right ventricle (Figure 1C; online-only Data Supplement Movie II). Three-dimensional echocardiography in a tilted apical 5-chamber view showed a broad tunnel arising from the aorta (Figure 1D; online-only Data Supplement Movie III). Computed tomographic cardiac angiography revealed the presence of a …