Congenital coronary artery anomalies in young patients: New perspectives for

timely identification
Antonio Pelliccia
J. Am. Coll. Cardiol. 2001;37;598-600

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Journal of the American College of Cardiology
© 2001 by the American College of Cardiology
Published by Elsevier Science Inc.
EDITORIAL COMMENT
Congenital Coronary Artery
Anomalies in Young Patients
New Perspectives for
Timely Identification*
Antonio Pelliccia, MD
Rome, Italy
Sudden and unexpected cardiac deaths in young subjects are
rare, but tragic events in competitive athletes assume high
public profile, and that unavoidably raise questions regard-
ing the underlying pathological causes and clinical strategies
to prevent these catastrophes (1,2).
Various pathological investigations have reported that
several structural cardiovascular abnormalities are responsi-
ble for sudden death in young athletes, but hypertrophic
cardiomyopathy is the most common disease in U.S.
autopsy-based series, and right ventricular cardiomyopathy
predominates in young Italian athletes (1,3– 8). Neverthe-
less, congenital coronary artery anomalies (CCAA) are
frequently found (in about 20% of cases), and they represent
the second most frequent disease responsible for athletic
field deaths (9 –14). Specifically, the wrong sinus coronary
artery origin, that is, the left main artery arising from the
right anterior sinus (ALMCA), and the right coronary
artery originating from the left sinus (ARCA), with a
proximal course between aorta and pulmonary trunk, are the
most frequent occurrences of CCAA.
See page 593
The article by Davis et al. (15) in this issue of the Journal
describes the results of prospective echocardiographic iden-
tification of wrong sinus coronary anomalies in a large
population of children and adolescents. Their contribution
is remarkable, and it is likely to generate new clinical interest
regarding these malformations, with special attention to the
issues set forth below.
Prevalence in the general population. Prevalence of
CCAA and, specifically, of the wrong aortic sinus coronary
anomalies in the general population is still uncertain.
Previous studies suggested that these malformations occur
in 0.2% to 1% of a large group of patients undergoing
cardiac catheterization (16). Davis et al. (15) in the present
article report a prevalence of 0.17%, derived from a popu-
lation of 2,388 children and adolescents prospectively ex-
*Editorials published in the Journal of the American College of Cardiology reflect the
views of the authors and do not necessarily represent the views of JACC or the
American College of Cardiology.
From the Institute of Sports Science, Department of Medicine, Italian National
Olympic Committee, Rome, Italy.
Downloaded from content.onlinejacc.org by on May 31, 2010
Vol. 37, No. 2, 2001
ISSN 0735-1097/01/$20.00
PII S0735-1097(00)01122-0
amined by echocardiography. However, their study popula-
tion comprised asymptomatic children and adolescents
referred for cardiovascular investigation, and thus cannot be
considered a true expression of the “normal” population.
The prevalence of wrong sinus coronary malformations in a
large and unselected young population should likely be less
than 0.2%. This hypothesis is also supported by results of
our previous investigation: in a population of 1,360 asymp-
tomatic competitive athletes, routinely examined by echo-
cardiography, we found no anomalies of origin and course of
major coronary arteries, which is consistent with a preva-
lence of less than 0.1% (17).
Clinical identification. Wrong sinus coronary anomalies
are rarely diagnosed or even suspected during life; this is
because of the scarcity of symptoms and inadequacy of
routine diagnostic testing (8, 18).
Premonitory symptoms, such as chest discomfort, atypi-
cal chest pain, and/or syncope, are reported in less than 30%
of patients (9 –14,19). Symptoms are more likely to be
reported by subjects with ALMCA, and they usually occur
in association with exercise, but rarely raise clinical suspicion
for CCAA (12,13). Indeed, when young individuals pre-
senting with symptoms are evaluated with 12-lead resting
and exercise electrocardiography, ischemic changes sugges-
tive for CCAA are rarely detected. In a recent investigation
describing the anatomical and clinical profiles of young
athletes with wrong sinus origin of coronary arteries, Basso
et al. (19) reported that all the resting 12-lead and exercise
electrocardiograms (ECGs) available in the 12 subjects
examined during life were within normal limits. Indeed, a
review of the literature encompassing the results of exercise
electrocardiograms in 18 young patients (⬍35 years) with
CCAA reveals that ischemic changes were present in only
four subjects (i.e., 22%), including two who were already
symptomatic (19).
Echocardiography, instead, has the potential to address
correct diagnosis, because it provides good anatomic defi-
nition of the ostium and proximal epicardial course of
coronary arteries. In 1,360 young athletes prospectively
evaluated by echocardiography we were able to visualize the
ostium and proximal epicardial course of LMCA in 97%
and RCA in 80% of subjects (17). Indeed, in young
individuals presenting with symptoms or abnormal 12-lead
ECG, echocardiography provided correct identification of
wrong sinus origin of the coronary arteries, which was
subsequently confirmed by coronary angiography (20 –22).
Therefore, the present article by David et al. (15) is
outstanding because it demonstrates that prospective echo-
cardiographic assessment is an efficient means to detect
during life wrong sinus coronary malformations in a large
population of children and adolescents. The present study
emphasizes the diagnostic role of echocardiography, and it
suggests that investigation of ostium and proximal course of
coronary arteries should be a routine part of any echocar-
JACC Vol. 37, No. 2, 2001
February 2001:598–600
diographic study. When the origin of both coronary arteries
cannot be identified by transthoracic echocardiography,
either transesophageal echocardiography or magnetic reso-
nance imaging (MRI) is recommended (23). Finally, if none
of the noninvasive techniques are successful and suspicion
for CCAA is still high, coronary arteriography is definitive.
However, it is unwarranted to generate enthusiasm and
expectation for easy noninvasive identification of wrong
sinus coronary artery malformations; false negative results
are likely to occur with transthoracic echocardiography, and
the present study reports one of these cases (15). False
negative results may be caused by a poor acoustic window or
by incorrect interpretation of the echocardiographic images.
Previous studies have failed to address this question, and, at
present, both the sensitivity and the specificity of echocar-
diography for identifying CCAA in a large and unselected
young population have still to be defined.
Mechanism(s) precipitating sudden death. The mecha-
nism(s) leading to sudden death in patients with CCAA are
triggered by myocardial ischemia. Occurrence of ischemia is
suggested by pathological evidence of acute myocardial
damage (small infarcts) and/or chronic injuries (patchy areas
of myocardial fibrosis) in the area supplied by the anomalous
coronary artery (10 –12,19). Ischemia is the consequence of
anatomical malformations, including the acute angle takeoff
of the anomalous vessel, with a narrowed slitlike orifice that
collapses in a valvelike manner, thereby limiting the blood
flow. Other anatomical features responsible for ischemia are
the proximal intramural course of the anomalous vessel,
which is squeezed within the aortic wall, and the compres-
sion of the anomalous vessel along its course between the
aorta and the pulmonary artery, particularly during exercise.
However, in a pathology analysis that comparatively as-
sessed the anatomical features of ALMCA or ARCA in 12
patients who died by these anomalies and in 18 patients who
died of unrelated causes, Taylor and colleagues (13) found
that neither the size of the ostium, the degree of angle
takeoff, the length of intramural aortic course, nor the
presence of an ostial ridge was predictive for incidence of
sudden death.
Whatever the anatomic malformation, patients who died
suddenly during exercise have usually done the same amount
of exercise or even more strenuous exercise multiple times,
without symptoms or physical impairment. It has been
suggested, therefore, that ischemia may be caused by spo-
radic spasm of the anomalous coronary artery induced by
endothelial injury (14). Another mechanism may be the
occurrence of ventricular tachyarrhythmia, on the basis of an
electrically unstable myocardium. In a few patients being
monitored with ECG at the time of their sudden death,
ventricular fibrillation was the final event (10,14).
Clinical management. Timely identification of patients
with wrong sinus coronary anomalies also raises the ques-
tion of clinical management. Though it is clear that not all
individuals with such anomalies are at risk of sudden cardiac
death and that many patients have lived a full life and died
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Pelliccia 599
Editorial Comment
of unrelated causes, it is also well known that these
malformations are among the most common reasons for
sudden and unexpected death in young individuals, partic-
ularly during sporting activities (3–5,7–14). The paucity of
reports describing the clinical profile and efficacy of surgical
approach in patients with incidentally discovered CCAA
makes the present recommendations for clinical manage-
ment still controversial. For simplicity, we may consider the
most common instances:
1. A young patient (⬍35 years) with unequivocal diagno-
sis of wrong sinus coronary malformation, presenting
with symptoms and/or showing evidence of myocardial
ischemia: In this case, the surgical approach to repair
this malformation is mandatory.
2. A young subject (⬍35 years) with incidental diagnosis of
wrong sinus coronary anomaly, in the absence of symp-
toms or signs suggestive of myocardial ischemia: In this
case there is uncertainty regarding the most appropriate
therapeutic choice. In making this decision, we have to
consider that precise mechanism(s) leading to ischemia
and sudden death are still unknown and unpredictable,
and these patients cannot be reliably stratified with regard
to risk. Therefore, it seems reasonable that these young
subjects should not be exposed to the risk of sudden death,
and surgical repair of the coronary malformation should be
considered. Attention should be paid to the level of the
patient’s physical activity, since the greatest incidence of
sudden death occurs during heavy physical exertion.
Changing to a completely and chronic sedentary lifestyle
in children and adolescents is not a realistic option, and
the desire to participate in regular heavy exercise or engage
in competitive athletics represents a further indication for
surgical repair. Heavy exercise programs and competitive
sports should be clearly discouraged in young patients with
CCAA, at least until surgical correction is performed (24).
3. An adult or older patient with incidental diagnosis of
wrong sinus coronary malformations, in the absence of
symptoms and inducible ischemia: In consideration that
sudden death occurs at a young age, this occasional
finding has likely no clinical significance and most
probably needs no surgical therapy. However, because
the magnitude of the risk remains unknown, individu-
alization of the therapeutic choice is appropriate.
Finally, this author believes that it is now timely and
appropriate to establish an international registry of prospec-
tively identified young patients with CCAA in order to
achieve a better understanding of the clinical profile and the
impact of surgical correction on the natural history of these
malformations.
Reprint requests and correspondence to: Dr. Antonio Pelliccia,
MD, Institute of Sports Science, Department of Medicine, Via dei
Campi Sportivi, 46, 00197 Rome, Italy. E-mail:
ant.pelliccia@libero.it.
600 Pelliccia
Editorial Comment
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Congenital coronary artery anomalies in young patients: New perspectives for
timely identification
Antonio Pelliccia
J. Am. Coll. Cardiol. 2001;37;598-600
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