Severe Dilated Cardiomyopathy Resulted from a Large Single Coronary Artery Fistula Drained Into the Left Ventricle

Coronary artery fistula (CAF) draining into the left ventricle (LV) is a rare condition and dilated cardiomyopathy (DCMP) that results from single coronary artery (SCA) accompanied by CAF also is extremely rare.

We report the case of a 36-year-old man, who presented with severe DCMP that resulted from SCA with CAF draining into the LV. Transthoracic echocardiogram (TTE) showed severe diffuse hypokinesia of the LV with ejection fraction (EF) of 15-20%. Coronary angiography (CAG) revealed SCA connected between left anterior descending artery (LAD) and posterior descending artery (PDA), course of the PDA was very tortuous from apex to base of the LV, and connected to posterior lateral (PL) branch, which was drained into the LV at distal part of the PL. Coronary artery computed tomography (CACT) showed LAD ran over the apex of the LV and connected to PDA, which was drained into the mid portion of lateral wall of the LV. Cardiac magnetic resonance imaging (CMRI) showed no evidence of irreversible myocardial change in global wall of the LV. The patient underwent surgical ligation of PDA near the base of the posterior wall of the LV as close to the entry of CAF to the LV as possible without any surgery-related complications. Three months after the surgical ligation, follow-up TEE  showed much improved EF of 45-50%. He has been doing well without congestive heart failure (CHF) until now.

Symptomatic CAF with hemodynamic deterioration may need mechanical correction of CAF, including surgical ligation or percutaneous interventional occlusion. How to treat this condition in terms of methodology is a very difficult issue. The detailed methods related to surgical or interventional correction of CAF have to be determined based on anatomical characteristics of CAF, underlying comorbidities, and relevant complications risk.