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

Hitesh Agrawal, Carlos M. Mery, S. Kristen Sexson Tejtel, Charles D. Fraser, E. Dean McKenzie, Athar M. Qureshi, Silvana Molossi

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)1099-1105
Number of pages7
JournalCardiology in the young
Volume28
Issue number9
DOIs
StatePublished - Sep 1 2018
Externally publishedYes

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Sudden Cardiac Death
Cluster Analysis
Coronary Vessels
Cardiomyopathies
Coronary Artery Disease
Romano-Ward Syndrome
Cardiology
Athletes
Cardiac Arrhythmias
Heart Diseases
Referral and Consultation
Population

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges. / Agrawal, Hitesh; Mery, Carlos M.; Sexson Tejtel, S. Kristen; Fraser, Charles D.; McKenzie, E. Dean; Qureshi, Athar M.; Molossi, Silvana.

In: Cardiology in the young, Vol. 28, No. 9, 01.09.2018, p. 1099-1105.

Research output: Contribution to journalArticle

Agrawal, Hitesh ; Mery, Carlos M. ; Sexson Tejtel, S. Kristen ; Fraser, Charles D. ; McKenzie, E. Dean ; Qureshi, Athar M. ; Molossi, Silvana. / Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges. In: Cardiology in the young. 2018 ; Vol. 28, No. 9. pp. 1099-1105.
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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.",
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T1 - Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges

AU - Agrawal, Hitesh

AU - Mery, Carlos M.

AU - Sexson Tejtel, S. Kristen

AU - Fraser, Charles D.

AU - McKenzie, E. Dean

AU - Qureshi, Athar M.

AU - Molossi, Silvana

PY - 2018/9/1

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N2 - 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.

AB - 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.

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SN - 1047-9511

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