Anomalous origin of left main coronary artery from the right sinus of Valsalva leading to sudden death

Abstract

A 14-year-old female was brought to the emergency room with chest pain, shortness of breath and cyanosis. She was previously well with the exception of one previous post-exertion seizure-like event. On this day, she had been jogging when she complained of chest pain and collapsed. Her initial vital signs were heart rate 58/min, blood pressure 70/40 mmHg, respiratory rate 50/min, temperature 37 ?C, and SaO2 68%. Electrocardiogram showed significant ST changes. She received multiple fluid boluses and dopamine was initiated (5?20 ?g/kg/min). She was intubated and started on norepinephrine (0.05?0.5 ?g/kg/min) for refractory hypotension. During the resuscitation, echocardiography showed poor left ventricular function with an ejection fraction of 38%. The coronary arteries could not be visualized clearly. To maintain cardiac output, epinephrine by infusion (0.1?3.0 ?g/kg/min) was added, and she received multiple epinephrine boluses. Despite maximum ventilatory support and escalating inotropes, cardiac output rapidly deteriorated, and she developed an agonal rhythm with non-reactive pupils. Resuscitation was discontinued. Autopsy demonstrated an anomalous origin of left coronary artery from the right aortic sinus of Valsalva with acute myocardial ischemia. We describe the sudden coronary death of a young patient, and we review congenital coronary artery pathophysiology, screening difficulties and potential interventions.

The Alcapa Syndrome in Adults

“…Продолжительное существование порока приводит к развитию аритмий, недостаточности кровообращения, которые в некоторых случаях сохраняются и после оперативного лечения [25]. У ряда пациентов первым проявлением синдрома ALCAPA становится внезапная коронарная смерть [26]. В отсутствии лечения прогноз при этом врожденном пороке связан Рис .…”

Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA) in an Infant - A Case Report

“…This latter type is the only one predisposing to sudden death. 10 ALCAPA usually manifests as an isolated defect, but in 5% of cases it may be associated with other cardiac anomalies such as Atrial septal defect, Ventricular septal defect, and Coarctation, 11 it also may be associated with Tertralogy of Fallot (TOF), Transposition of great arteries (TGA) and Patent ductus arteriosus (PDA). 5 In the neonatal period, the baby is asymptomatic as there is anterograde flow of desaturated blood from the pulmonary artery to the left coronary artery.…”

Why can a case report be so important? The case of sudden cardiac death, caused by a coronary anomaly

“…Fig. 3, in paper of Kutreti et al [4], illustrates quite vividly the extreme severity of the left main trunk stenosis. It is quite likely that aggressive exercise precipitated worsening of stenosis in this unfortunate girl, reaching the level of critical severity.…”

“…The accompanying article by Kutreti et al [4] presents in remarkable, unusually available details the case of a 14-year-old girl, who succumbed to one of such cases of SCD.…”