Skip to main content Accessibility help
×
Home

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

  • Hitesh Agrawal (a1) (a2), Carlos M. Mery (a1) (a3), S. Kristen Sexson Tejtel (a1) (a2), Charles D. Fraser (a1) (a3), E. Dean McKenzie (a1) (a3) (a4), Athar M. Qureshi (a1) (a2) and Silvana Molossi (a1) (a2)...

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.

Copyright

Corresponding author

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: smolossi@bcm.edu

Footnotes

Hide All

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 Young 28: 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.

Footnotes

References

Hide All
1. Mery, CM, Lawrence, SM, Krishnamurthy, R, et al. Anomalous aortic origin of a coronary artery: toward a standardized approach. Semin Thorac Cardiovasc Surg 2014; 26: 110122.
2. Maron, BJ, Doerer, JJ, Haas, TS, Tierney, DM, Mueller, FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation 2009; 119: 10851092.
3. Prakken, NH, Cramer, MJ, Olimulder, MA, Agostoni, P, Mali, WP, Velthuis, BK. Screening for proximal coronary artery anomalies with 3-dimensional MR coronary angiography. Int J Cardiovasc Imaging 2010; 26: 701710.
4. Pelliccia, A, Spataro, A, Maron, BJ. Prospective echocardiographic screening for coronary artery anomalies in 1,360 elite competitive athletes. Am J Cardiol 1993; 72: 978979.
5. Cheezum, MK, Liberthson, RR, Shah, NR, et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of valsalva. J Am Coll Cardiol 2017; 69: 15921608.
6. Yetman, AT, McCrindle, BW, MacDonald, C, Freedom, RM, Gow, R. Myocardial bridging in children with hypertrophic cardiomyopathy – a risk factor for sudden death. N Engl J Med 1998; 339: 12011209.
7. Bachmann, JM, Willis, BL, Ayers, CR, Khera, A, Berry, JD. Association between family history and coronary heart disease death across long-term follow-up in men: the Cooper Center Longitudinal Study. Circulation 2012; 125: 30923098.
8. Brothers, JA, Stephens, P, Gaynor, JW, Lorber, R, Vricella, LA, Paridon, SM. Anomalous aortic origin of a coronary artery with an interarterial course: should family screening be routine? J Am Coll Cardiol 2008; 51: 20622064.
9. Laureti, JM, Singh, K, Blankenship, J. Anomalous coronary arteries: a familial clustering. Clin Cardiol. 2005; 28: 488490.
10. Bunce, NH, Rahman, SL, Keegan, J, Gatehouse, PD, Lorenz, CH, Pennell, DJ. Anomalous coronary arteries: anatomic and functional assessment by coronary and perfusion cardiovascular magnetic resonance in three sisters. J Cardiovasc Magn Reson 2001; 3: 361369.
11. Horan, PG, Murtagh, G, McKeown, PP. Single coronary artery: a familial clustering. Heart. 2003; 89: e27.
12. Rowe, L, Carmody, TJ, Askenazi, J. Anomalous origin of the left circumflex coronary artery from the right aortic sinus: a familial clustering. Cathet Cardiovasc Diagn 1993; 29: 277278.
13. Devanagondi, R, Brenner, J, Vricella, L, Ravekes, W. A tale of two brothers: anomalous coronary arteries in two siblings. Pediatr Cardiol. 2008; 29: 816819.
14. Van Hare, GF, Ackerman, MJ, Evangelista, J-AK, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology. Circulation 2015; 132: e281e291.
15. Brothers, JA, Frommelt, MA, Jaquiss, RDB, Myerburg, RJ, Fraser, CD, Tweddell, JS. Expert consensus guideline: Anomalous aortic origin of a coronary artery. J Thorac Cardiovasc Surg 2017; 153: 14401457.
16. Agrawal, H, Mery, CM, Krishnamurthy, R, Molossi, S. Anatomic types of anomalous aortic origin of a coronary artery: a pictorial summary. Congenit Heart Dis 2017; 12: 603606.
17. Hainline, B, Drezner, JA, Baggish, A, et al. Interassociation consensus statement on cardiovascular care of college student-athletes. J Am Coll Cardiol 2016; 67: 29812995.
18. Brothers, JA, Harris, MA, Paridon, SM. Anomalous aortic origin of a coronary artery in siblings with Marfan syndrome. Cardiol Young. 2011; 21: 238240.
19. Unzué-Vallejo, L, Andreu-Dussac, J, Sánchez-Sánchez, V, Gragera-Torres, F. Congenital hereditary anomalous coronary artery origin. Rev Esp Cardiol (Engl Ed) 2012; 65: 859861.
20. Agrawal, H, Molossi, S, Alam, M, et al. Anomalous coronary arteries and myocardial bridges: risk stratification in children using novel cardiac catheterization techniques. Pediatr Cardiol 2017; 38: 624630.
21. Pepler, WJ, Meyer, BJ. Interarterial coronary anastomoses and coronary arterial pattern. A comparative study of South African Bantu and European hearts. Circulation 1960; 22: 1424.
22. Topaz, O, DeMarchena, EJ, Perin, E, Sommer, LS, Mallon, SM, Chahine, RA. Anomalous coronary arteries: angiographic findings in 80 patients. Int J Cardiol 1992; 34: 129138.
23. Garg, N, Tewari, S, Kapoor, A, Gupta, DK, Sinha, N. Primary congenital anomalies of the coronary arteries: a coronary: arteriographic study. Int J Cardiol 2000; 74: 3946.
24. Leon, AS, Bloor, CM. Quantitative analysis of coronary artery inheritance. J Hered 1968; 59: 4852.
25. Maron, BJ, Haas, TS, Ahluwalia, A, Murphy, CJ, Garberich, RF. Demographics and epidemiology of sudden deaths in young competitive athletes: from the United States National Registry. Am J Med 2016; 129: 11701177.
26. Clauss, SB, Walker, DL, Kirby, ML, Schimel, D, Lo, CW. Patterning of coronary arteries in wildtype and connexin43 knockout mice. Dev Dyn 2006; 235: 27862794.
27. Li, WEI, Waldo, K, Linask, KL, et al. An essential role for connexin43 gap junctions in mouse coronary artery development. Development 2002; 129: 20312042.

Keywords

Type Description Title
WORD
Supplementary materials

Agrawal et al. supplementary material
Table S1

 Word (77 KB)
77 KB

Metrics

Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed