Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-27T04:19:56.360Z Has data issue: false hasContentIssue false
  • English
  • Français

Anomalous aortic origin of a coronary artery in siblings with Marfan syndrome*

Published online by Cambridge University Press:  08 December 2010

Julie A. Brothers ,
Matthew A. Harris  and
Stephen M. Paridon
Julie A. Brothers*
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Matthew A. Harris
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Stephen M. Paridon
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: J. A. Brothers, M.D., Division of Cardiology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Cardiology, Main Building, 8NW75, Philadelphia, Pennsylvania 19104, United States of America. Tel: 267-426-5700; Fax: 215 590 4978; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

We report a case of two brothers with Marfan syndrome and coexistent anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. To our knowledge, this is the first ever report of siblings with Marfan syndrome who also have anomalous aortic origin of a coronary artery. The treatment and management options in the context of sudden death risk are discussed.

Keywords

Coronary artery anomalyconnective tissue disordercongenital cardiac disease
Type
Brief Reports
Information
Cardiology in the Young , Volume 21 , Issue 2 , April 2011 , pp. 238 - 240
Copyright
Copyright © Cambridge University Press 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

*

The submission is with the full knowledge and approval of the above listed co-authors.

References

1. Basso, C, Maron, BJ, Corrado, D, Thiene, G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol 2000; 35: 1493–1501.Google Scholar
2. Taylor, AJ, Byers, JP, Cheitlin, MD, Virmani, R. Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J 1997; 133: 428–435.CrossRefGoogle ScholarPubMed
3. Pyeritz, RE. Marfan syndrome: current and future clinical and genetic management of cardiovascular manifestations. Semin Thorac Cardiovasc Surg 1993; 5: 11–16.Google ScholarPubMed
4. Matsuda, K, Ichikawa, H, Iwai, S, Takahashi, T. Modified aortic root remodeling for annuloaortic ectasia with abnormal coronary take-off. Ann Thorac Surg 2002; 74: 1687–1689.CrossRefGoogle ScholarPubMed
5. Papadopoulos, DP, Moyssakis, I, Votteas, VE. Coexistence of anomalous origin of the coronary arteries and severe aortic regurgitation in Marfan syndrome. Clin Rheumatol 2006; 25: 737–738.Google Scholar
6. Lacro, RV, Dietz, HC, Wruck, LM, et al. Rationale and design of a randomized clinical trial of β-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome. Am Heart J 2007; 154: 624–631.CrossRefGoogle ScholarPubMed
7. Judge, DP, Dietz, HC. Marfan's syndrome. Lancet 2005; 366: 1965–1976.Google Scholar
8. Brothers, JA, Stephens, P, Gaynor, JW, Lorber, R, Vricella, LA, Paridon, SM. Anomalous aortic origin of a coronary artery: should family screening be routine? J Am Coll Cardiol 2008; 51: 2062–2064.CrossRefGoogle ScholarPubMed
9. Davis, JA, Cecchin, F, Jones, TK, Portman, MA. Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 2001; 37: 593–597.Google Scholar
10. Yamanaka, O, Hobbs, RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21: 28–40.CrossRefGoogle ScholarPubMed