Syncope-Running

HISTORY: A 14-year-old male was running at school when he felt unwell, had nausea & vomiting, followed by syncope. When he regained consciousness, he endorsed chest pain & vision changes. En route to the hospital, he developed ventricular tachycardia to 180 bpm. In the emergency room, he deteriorated into cardiogenic shock & was transferred to our institution. Upon arrival, refractory to resuscitation maneuvers, cannulation to extracorporeal membrane oxygenation (ECMO) ensued. Diagnostic evaluation revealed elevated troponin, lactic acidosis, pulmonary edema, ST elevation on electrocardiogram, mildly depressed right ventricular (RV) & severely depressed left ventricular (LV) function on echocardiogram (ECHO). PHYSICAL EXAMINATION: Exam revealed an ill appearing male, cold extremities, decreased but equal pulses, no murmurs. DIFFERENTIAL DIAGNOSIS: 1. Myocarditis2. Myocardial Infarction/Ischemia3. Coronary anomalies4. Pulmonary embolism. TEST AND RESULTS: Patient required significant inotropic support & persistent troponin leak following ECMO decannulation.With a working diagnosis of myocarditis, repeat ECHO was obtained after ECMO decannulation, 1 week from presentation & showed anomalous aortic origin of the left coronary artery from the right sinus of Valsalva with concern for proximal coronary stenosis in its intramural course.Cardiac computed tomography angiography confirmed this finding. FINAL WORKING DIAGNOSIS: Anomalous aortic origin of the left coronary artery from right sinus of Valsalva TREATMENT AND OUTCOMES: - Underwent surgical reimplantation of LCA with 3.5 mm Gortex® interposition graft (following unsuccessful direct reimplantation), repair of left aortic sinus and main pulmonary artery.- Postoperative course was complicated by low cardiac output state, requiring vasoactive medications and volume resuscitation, with improvement in markers of cardiac output.- Once off vasoactives, β-blockade, ACE inhibition, and spironolactone were initiated to decrease myocardial oxygen demand.- Prior to discharge, ECHO showed normal RV and mild-moderately depressed LV systolic function.- It is anticipated that the patient’s function will continue to recover though the extent of myocardial insult is unknown. Post-op studies will be completed at 3 months following repair.