We report the clinical case of a previous healthy and sporty young woman admitted to the Cardiac Intensive Care Unit for aborted sudden cardiac death.
A 28-year-old sporty woman without relevant medical history had a pre-syncope at home and activated the national emergency system. Upon arrival of the medical team she was still conscious referring vague complaints. She fainted and the EKG showed ventricular fibrillation (VF) that was promptly defibrillated with a 200J shock. Upon return of spontaneous circulation, she had 15 in Glasgow Coma Scale and denied any cardiovascular symptom. The EKG after defibrillation showed sinus tachycardia with voltage criteria for left ventricular (LV) hypertrophy and poor R wave progression in the precordial leads. She remained asymptomatic and hemodynamically stable. Point-of-care transthoracic echocardiography showed moderate LV concentric hypertrophy, global hypokinesia, moderately depressed LV function and no pericardial effusion. High-sensitivity cardiac troponin was slightly elevated (0.045 ng/mL). She was admitted in the Cardiac Intensive Care Unit with the leading diagnosis of myocarditis or hypertrophic cardiomyopathy.
Serial cardiac enzymes were negative. Detailed echocardiographic evaluation revealed moderate LV enlargement, hypokinesia of the basal and mid segments of the anterior and anteroseptal walls and all distal segments, and moderately depressed LV function. Interestingly, there was evidence of possible right coronary artery dilatation or aneurysm and fistulous tracts along the interventricular septum.
Coronary CT angiography showed an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and a dilated right coronary artery with extensive collateral network (Fig. 1). To better understand the functional significance of this anomaly and evaluate for myocardial fibrosis, cardiac magnetic resonance (CMR) imaging was performed. There was evidence of subendocardial late gadolinium enhancement in the anterior wall and mid-distal anteroseptum, with transmural extent only in the medium segment of anterior wall. Furthermore, CMR showed extensive peri-infarct ischemia. An invasive coronary angiography confirmed ALCAPA with retrograde filling of the left coronary artery territory through collaterals arising from an enlarged right coronary artery.
This patient was referred for cardiac surgery and will do a coronary button transfer. An ICD will be implanted at discharge.
This clinical case highlights the importance of considering the rare causes of sudden cardiac death, especially in young patients with otherwise unremarkable medical history. Furthermore, it stresses the integrative multimodality imaging approach to thoroughly characterize coronary artery anomalies and plan the management of those patients.