SCMR 22nd Annual Scientific Sessions
Description of Clinical Presentation:
A 24 year old male with pulmonary atresia/ intact ventricular septum (PA/IVS) and a large coronary-cameral fistula, status post extracardiac Fontan palliation, presented with left sided chest pain, non-sustained ventricular tachycardia (NSVT), and recurrent, unexplained syncope. He recently had coiling of aorto-pulmonary collaterals (APC) for episodic hemoptysis. During admission for APC coiling, his anti-coagulation was discontinued. For his NSVT and CP, he underwent cardiac magnetic resonance imaging (CMR) for anatomic definition, functional assessment, myocardium viability, and thrombus evaluation.
Diagnostic Techniques and Their Most Important Findings:
CMR demonstrated a severely dilated and tortuous left main coronary artery fistula emptying into the hypoplastic right ventricle (RV) (Figure 1). Both the left anterior descending (LAD) and left circumflex (Circ) arose from the fistula. Although flow assessment at the level of the aortic valve showed no regurgitation, the aortic flow assessed above the coronary origins showed 23% flow reversal, suggesting significant coronary flow. The left ventricle (LV) had mildly depressed systolic function (LV ejection fraction = 45%). There was significant LV late gadolinium enhancement (LGE) (15%) in the distribution of the LAD and Circ (Figure 2) extending from the mid to apical inferior LV. Incidentally, a large calcified thrombus was detected in the RV (Figure 3), with a small sub-segmental pulmonary embolus on chest computed tomography (CT) scan not seen on CMR. He was placed back on anticoagulation. Due to his history of unexplained syncope and risk of continued intermittent ischemia, secondary to steal from his distal left coronary system, an implantable cardioverter defibrillator (ICD) was recommended. The patient refused the ICD.
Learning Points from this Case: