Cardiology in the Young (2014), 24, 397–402
© Cambridge University Press, 2013
doi:10.1017/S1047951113002217
Review Article Anomalous left coronary artery from the right coronary cusp with gene positive apical hypertrophic cardiomyopathy: a case report and literature review Justin Georgekutty,1 Russell R. Cross,1 Joanna B. Rosenthal,1 Deneen M. Heath,1 Pranava Sinha,2 Anitha S. John1 Department of Pediatrics, Division of Cardiology; 2Department of Pediatrics, Division of Cardiac Surgery, Children’s National Medical Center, George Washington University, Washington, District of Columbia, United States of America
1
Abstract In the United States, hypertrophic cardiomyopathy and coronary artery anomalies account for the leading two causes of sudden death in athletes. We present a case of a patient with an anomalous origin of the left main from the right coronary sinus with associated gene-confirmed hypertrophic cardiomyopathy. The patient underwent surgical repair with unroofing of the intramural portion of the left main coronary artery with a good result. We also review the reported cases in the medical literature describing this uncommon association between anomalous coronary artery origin and hypertrophic cardiomyopathy. Keywords: Anomalous coronary artery; hypertrophic cardiomyopathy; sudden cardiac death Received: 17 October 2013; Accepted: 29 October 2013; First published online: 17 December 2013
S
UDDEN CARDIAC DEATH CONTINUES TO COME INTO
the spotlight and prevention is the focus of screening competitive athletes. In the United States, hypertrophic cardiomyopathy and coronary artery anomalies account for the leading two causes of sudden death in athletes.1 In a registry of athletes who had sudden cardiac death, 36% of patients were identified as having hypertrophic cardiomyopathy.1 Of the coronary anomalies resulting in sudden cardiac death, anomalous origin of the left main coronary artery from the right sinus of Valsalva represents the most common lesion.2,3 Whereas left ventricular hypertrophy has been observed with anomalous origin of the coronary artery, the combination of hypertrophic cardiomyopathy and anomalous coronary origin is not commonly seen. We present a case of an anomalous origin of the left main from the right coronary sinus with associated gene-confirmed hypertrophic cardiomyopathy and review the literature Correspondence to: Dr A. S. John, MD, PhD, Assistant Professor of Pediatrics and Internal Medicine, Division of Cardiology, Children’s National Medical Center, 111 Michigan Ave NW, West Wing, 3.5, Washington, DC 20010, United States of America. Tel: 202 476 3543; Fax: 202 476 5700; E-mail: [email protected]
regarding anomalous coronary origin and hypertrophic cardiomyopathy.
Case presentation An 18-year-old boy presented to the emergency department for evaluation following a syncopal episode occurring the day before presentation. While exercising at the gym, he felt palpitations with associated lightheadedness and blurry vision. He then reportedly became unresponsive for ~10 minutes after which he regained consciousness without intervention. He was initially taken to an urgent care facility where computed tomography of the head and electroencephalogram were performed and were within normal limits. He was subsequently transferred to our institution for further work-up. On further questioning, he reported a similar episode 9 months prior. In addition, he also had symptoms of lightheadedness and palpitations occurring consistently with exercise. His past medical history was notable for attention deficit hyperactivity disorder and oppositional defiant disorder. He was not on any medications. On physical examination, he
398
June 2014
Cardiology in the Young
Figure 1. Echocardiogram on presentation revealed sinus bradycardia with sinus arrhythmia, ST elevation in anterior leads and left ventricular hypertrophy with strain pattern.
Ao
PA
Figure 2. Echocardiogram, pre-operative. (a) Anomalous origin of the left main coronary (see arrow) from the right aortic cusp in the parasternal shortaxis view. (b) Intramural course of anomalous left coronary artery between the aorta and pulmonary artery. (c) Apical left ventricular hypertrophy in the parasternal short-axis view. (d) Apical left ventricular hypertrophy in the apical four-chamber view. Ao = aorta; PA = pulmonary artery.
was noted to have a systolic ejection murmur along the upper sternal border with normal vital signs. An electrocardiogram (Fig 1) revealed sinus bradycardia with marked sinus arrhythmia, left ventricular hypertrophy with strain pattern and ST-segment elevation in the anterior leads. Initial laboratory data revealed a
normal basic metabolic profile, total creatine kinase of 748 U/L (34–147 U/L), CK-MB of 2.4 ng/ml (
© Cambridge University Press, 2013
doi:10.1017/S1047951113002217
Review Article Anomalous left coronary artery from the right coronary cusp with gene positive apical hypertrophic cardiomyopathy: a case report and literature review Justin Georgekutty,1 Russell R. Cross,1 Joanna B. Rosenthal,1 Deneen M. Heath,1 Pranava Sinha,2 Anitha S. John1 Department of Pediatrics, Division of Cardiology; 2Department of Pediatrics, Division of Cardiac Surgery, Children’s National Medical Center, George Washington University, Washington, District of Columbia, United States of America
1
Abstract In the United States, hypertrophic cardiomyopathy and coronary artery anomalies account for the leading two causes of sudden death in athletes. We present a case of a patient with an anomalous origin of the left main from the right coronary sinus with associated gene-confirmed hypertrophic cardiomyopathy. The patient underwent surgical repair with unroofing of the intramural portion of the left main coronary artery with a good result. We also review the reported cases in the medical literature describing this uncommon association between anomalous coronary artery origin and hypertrophic cardiomyopathy. Keywords: Anomalous coronary artery; hypertrophic cardiomyopathy; sudden cardiac death Received: 17 October 2013; Accepted: 29 October 2013; First published online: 17 December 2013
S
UDDEN CARDIAC DEATH CONTINUES TO COME INTO
the spotlight and prevention is the focus of screening competitive athletes. In the United States, hypertrophic cardiomyopathy and coronary artery anomalies account for the leading two causes of sudden death in athletes.1 In a registry of athletes who had sudden cardiac death, 36% of patients were identified as having hypertrophic cardiomyopathy.1 Of the coronary anomalies resulting in sudden cardiac death, anomalous origin of the left main coronary artery from the right sinus of Valsalva represents the most common lesion.2,3 Whereas left ventricular hypertrophy has been observed with anomalous origin of the coronary artery, the combination of hypertrophic cardiomyopathy and anomalous coronary origin is not commonly seen. We present a case of an anomalous origin of the left main from the right coronary sinus with associated gene-confirmed hypertrophic cardiomyopathy and review the literature Correspondence to: Dr A. S. John, MD, PhD, Assistant Professor of Pediatrics and Internal Medicine, Division of Cardiology, Children’s National Medical Center, 111 Michigan Ave NW, West Wing, 3.5, Washington, DC 20010, United States of America. Tel: 202 476 3543; Fax: 202 476 5700; E-mail: [email protected]
regarding anomalous coronary origin and hypertrophic cardiomyopathy.
Case presentation An 18-year-old boy presented to the emergency department for evaluation following a syncopal episode occurring the day before presentation. While exercising at the gym, he felt palpitations with associated lightheadedness and blurry vision. He then reportedly became unresponsive for ~10 minutes after which he regained consciousness without intervention. He was initially taken to an urgent care facility where computed tomography of the head and electroencephalogram were performed and were within normal limits. He was subsequently transferred to our institution for further work-up. On further questioning, he reported a similar episode 9 months prior. In addition, he also had symptoms of lightheadedness and palpitations occurring consistently with exercise. His past medical history was notable for attention deficit hyperactivity disorder and oppositional defiant disorder. He was not on any medications. On physical examination, he
398
June 2014
Cardiology in the Young
Figure 1. Echocardiogram on presentation revealed sinus bradycardia with sinus arrhythmia, ST elevation in anterior leads and left ventricular hypertrophy with strain pattern.
Ao
PA
Figure 2. Echocardiogram, pre-operative. (a) Anomalous origin of the left main coronary (see arrow) from the right aortic cusp in the parasternal shortaxis view. (b) Intramural course of anomalous left coronary artery between the aorta and pulmonary artery. (c) Apical left ventricular hypertrophy in the parasternal short-axis view. (d) Apical left ventricular hypertrophy in the apical four-chamber view. Ao = aorta; PA = pulmonary artery.
was noted to have a systolic ejection murmur along the upper sternal border with normal vital signs. An electrocardiogram (Fig 1) revealed sinus bradycardia with marked sinus arrhythmia, left ventricular hypertrophy with strain pattern and ST-segment elevation in the anterior leads. Initial laboratory data revealed a
normal basic metabolic profile, total creatine kinase of 748 U/L (34–147 U/L), CK-MB of 2.4 ng/ml (
