A Coronary–Pulmonary Artery Fistula with Giant Aneurysm in a Child with Ventricular Septal Defect with Pulmonary Atresia

Abstract

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).

Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect

“…Although most CPF cases were diagnosed preoperatively, others were not, even after 2000, and they were incidentally confirmed during surgery [ 8 , 26 , 37 ]. In several CPF cases, echocardiography typically revealed significant dilation of the coronary artery ostium, thus posing as a clue to the existence of CPF [ 2 , 3 , 31 , 35 , 36 , 41 , 42 ]. Therefore, careful screening of the structure of the proximal coronary artery should be performed in PAVSD to enable CPF identification.…”

“…Of these patients, eight were excluded due to inadequate data [ 4 , 5 ]. Finally, the remaining 69 patients, who were reported in 42 articles, were included in the present study [ 2 , 3 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 ]. Of these articles, 11 involving 21 patients (30%) were published before 1990 [ 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ].…”