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Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges

Published online by Cambridge University Press:  13 July 2018

Hitesh Agrawal ,
Carlos M. Mery ,
S. Kristen Sexson Tejtel ,
Charles D. Fraser Jr ,
E. Dean McKenzie ,
Athar M. Qureshi  and
Silvana Molossi
Hitesh Agrawal
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA The Lillie Frank Abercrombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
Carlos M. Mery
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
S. Kristen Sexson Tejtel
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA The Lillie Frank Abercrombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
Charles D. Fraser Jr
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
E. Dean McKenzie
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA Division of Cardiothoracic Surgery, Children’s Healthcare of Atlanta, GA, USA
Athar M. Qureshi
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA The Lillie Frank Abercrombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
Silvana Molossi*
Affiliation:
Coronary Anomalies Program, Texas Children’s Hospital, Houston, TX, USA The Lillie Frank Abercrombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
*
Author for correspondence: S. Molossi, MD, PhD, Texas Children’s Hospital, Baylor College of Medicine, 6621 Fannin Street, WT 19345-C, Houston, Texas 77030, USA. Tel: +832 8265663; Fax: +832 825 0165; E-mail: [email protected]
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Abstract

Background

Anomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population.

Methods

Patients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children’s Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant.

Results

Of 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff–Parkinson–White (1, 3%).

Conclusion

In patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.

Keywords

Anomalous coronary arterymyocardial bridge
Type
Original Article
Information
Cardiology in the Young , Volume 28 , Issue 9 , September 2018 , pp. 1099 - 1105
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Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Agrawal H, Mery CM, Sexson Tejtel SK, Fraser CD, McKenzie ED, Qureshi AM, Molossi S. (2018) Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges. Cardiology in the Young28: 1099–1105. doi: 10.1017/S1047951118000835

*

This paper was presented in an oral format at the American Academy of Pediatrics meeting, San Francisco, CA, USA, October 2016.

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