Description of Clinical Presentation: A 29 year old female with history of post-partum cardiomyopathy was transferred to our institution after an episode of ventricular fibrillation leading to an aborted cardiac arrest requiring defibrillation on the field. She underwent immediate resuscitation and was initiated on hypothermia protocol with complete neurologic recovery. A 2D echocardiogram revealed severely depressed left ventricular systolic function (EF 15%) with trivial mitral regurgitation in the setting of decreased function.
Diagnostic Techniques and Their Most Important Findings: Due to the history of cardiac arrest, a cardiac MRI was performed which demonstrated anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) (Figure 1), with mildly dilated left ventricle (LV EDVi 119 cc/m2) with severe global systolic dysfunction (LV EF 27%). There was delayed enhancement in the mid to apical anterior and anterolateral segments of the left ventricle with thinning and akinesis (Figure 2), identifying the substrate for arrhythmia. Cardiac catheterization revealed normal origin of the right coronary artery from the right sinus and delayed filling of the left coronary artery on aortic root angiography, which was noted to be arising anomalously from the pulmonary artery on selective angiography (Figure 3). Patient underwent a successful coronary artery translocation with an improved left ventricular function at discharge (LV EF 44%) on 2D echocardiography. Intra operatively measured pulmonary artery pressures were normal (mean PA pressure ~ 25 mmHg), no major macro collaterals were identified.
Learning Points from this
Case: This case highlights the presentation of ALCAPA in adult population. Cases in adults have been reported with concomitant pulmonary artery hypertension and presence of macro vascular collaterals enabling antegrade coronary flow and survival into adulthood. Our case was an enigma in the setting of normal pulmonary artery pressures and no major collaterals identified on catheterization and intraoperatively with likelihood of microvascular collaterals making survival into adulthood possible. CMR was crucial in delineating the anatomy and identifying the substrate for her ventricular arrhythmia and she is due for ICD placement upon discharge.