LETTER TO THE EDITOR
Management of anomalous coronary artery
Colin K. L. Phoon, MD, MPhil, FACCa
a New York University School of Medicine, Pediatric Cardiology Program, 540 First Avenue, TWR Suite 9U, New York, New York 10016, USA
colin.phoon{at}med.nyu.edu
The Davis et al. (1) finding of a 0.17% prevalence of anomalous coronary artery from the wrong aortic sinus in an asymptomatic pediatric population is important data. These investigators and others (2) suggest that surgical management is mandatory, while admitting that the duration of coronary artery bypass graft surgery (CABG) patency is not known. The accompanying editorial is more cautious for asymptomatic patients <35 years of age, citing uncertainty surrounding the most appropriate management option and stating that "surgical repair should be considered" (3). Pelliccia (3) also believes that these lesions in patients >35 years of age have "likely no clinical significance" and "most probably [need] no surgical treatment."
I am concerned that the specter of sudden death seems to have compelled many to "do something," in the absence of good outcomes data. A prevalence of  0.1% to 0.2% among 4 million live births annually in the U.S. (4) yields about 4,000 to 8,000 children born each year with these coronary anomalies. Of 275 million people in the U.S. (assuming a low incidence of death), 275,000 to 550,000 would be affected; approximately half are under age 35. (As an aside, the risk in an adult over age 35 may not be insignificant, as a recent case report shows [5].) And yet, the number of cases of sudden death appears exceedingly small when compared with these population figures (<<1%), as data from U.S. and Italian registries suggest (2). Is this truly the natural history of these lesions, or are we underestimating the risk?
The CABG procedure is not risk-free (6,7). Admittedly, surgical risk is likely to be lower in patients with anomalous coronary artery from the wrong sinus than with other coronary problems, as patients are typically healthy. Nevertheless, even the simplest of pediatric heart operations carry risks of neurologic, myocardial, and other organ injury, as well as death. Moreover, surgical "repair" by no means guarantees cure. One recent study of children showed patency rates of 78% and 36% for internal mammary grafts and saphenous vein grafts, respectively, at 10 years (7). Such figures virtually guarantee reoperation in most patients, with its concomitant risks.
Some physicians and families may prefer to accept the surgical risks rather than to live with an unknown potential for sudden death, but these risks must be fully understood before an operation is undertaken. I do believe that activity can often be modified, and therefore risk can be reduced, even if not eliminated. But the unanswered questions remain: 1) What is the true natural history of these coronary anomalies? 2) What is the long-term "unnatural history" of CABG in children? Although sudden death, particularly in a young and seemingly healthy person, is especially tragic, we must remember the fundamental principle of primum non nocere—"first, do no harm." Therefore, when it comes to surgical management, one must consider the adage, however difficult: "Don’t just do something, stand there!"
 |
References
|
|---|
1. 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[Abstract/Free Full Text]
2. Basso C, Maron BJ, Corado D, Thienne G. Clinical profile of congenital coronary anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493–1501[Abstract/Free Full Text]
3. Pelliccia A. Congenital coronary artery anomalies in young patients. J Am Coll Cardiol. 2001;37:598–600[Free Full Text]
4. Guyer B, Hoyert DL, Martin JA, Ventura SJ, MacDorman MF, Strobino DM. Annual summary of vital statistics—1998. Pediatrics. 1999;104:1229–1246[Abstract/Free Full Text]
5. Cox ID, Bunce N, Fluck DS. Failed sudden cardiac death in a patient with an anomalous origin of the right coronary artery. Circulation. 2000;102:1461–1462[Free Full Text]
6. Mavroudis C, Backer CL, Duffy E, Pahl E, Wax DF. Pediatric coronary artery bypass for Kawasaki, congenital, post-arterial switch, and iatrogenic lesions. Ann Thorac Surg. 1999;68:506–512[Abstract/Free Full Text]
7. Yoshikawa Y, Yagihara T, Kameda Y, et al. Result of surgical treatments in patients with coronary-arterial occlusive disease after Kawasaki disease. Eur J Cardiothorac Surg. 2000;17:515–519[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
M. D. Cheitlin
Finding Asymptomatic People With a Coronary Artery Arising From the Wrong Sinus of Valsalva: Consequences Arising From Knowing the Anomaly to Be Familial
J. Am. Coll. Cardiol.,
May 27, 2008;
51(21):
2065 - 2067.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. R. Deibler, R. S. Kuzo, M. Vohringer, E. E. Page, R. E. Safford, J. N. Patton, G. E. Lane, R. L. Morin, and T. C. Gerber
Imaging of Congenital Coronary Anomalies With Multislice Computed Tomography
Mayo Clin. Proc.,
August 1, 2004;
79(8):
1017 - 1023.
[Abstract]
[PDF]
|
|
|
|
Top
References