Prenatally diagnosed hypoplastic left heart syndrome with intact atrial septum and ventriculocoronary arterial fistula.

Intact atrial septum (IAS) and left ventriculocoronary arterial fistula (LVCAF) are the significant risk factors of hypoplastic left heart syndrome (HLHS), and when they are present, prognosis is grim. We report a case of prenatally diagnosed HLHS with IAS and LVCAF that was treated with surgical atrial-septostomy and bilateral pulmonary arterial banding (BPAB) immediately after birth. The Doppler flow pattern of left anterior descending coronary artery (LAD) with dominant retrograde flow was normalized after atrial-septostomy with decompression of left atrial (LA) hypertension. This is the first report showing the influence of atrial-septostomy on coronary flow in HLHS patient with LVCAF.