(Circulation. 2000;101:1219.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Myocardial Infarction in Children With Hypoplastic Coronary Arteries
From the Service de Cardiologie A, Hôpital de la Timone, and the Service de Chirurgie thoracique et cardiovasculaire, Hôpital d’enfants de la Timone (F.A.), Marseille, France.
Correspondence to A. Fraisse, MD, Service de Cardiologie A, Hôpital de la Timone, Blvd Jean Moulin, 13385 Marseille Cedex 5, France. E-mail afraisse{at}ap-hm.fr
An 11-year-old boy
with a past history of asthma was admitted to the pediatric intensive
care unit (ICU) for a non–Q-wave myocardial infarction that occurred
during sustained exercise (a handball match). He presented with
chest pain, anterior ST-segment depression on the ECG (Figure 1
), and elevation of
creatinine kinase (peak, 2580 mU; MB, 240). Initial
physical examination was normal, and his ICU course was uncomplicated.
He was discharged to the ward after 2 days, and cardiac
catheterization was performed 8 days after admission.
Left ventriculography (Figure 2) revealed
mild apical hypokinesia with an ejection fraction of 55%. Selective
coronary arteriography showed no atherosclerotic lesion but
hypoplasia of the distal left anterior descending (LAD) and right
coronary (RCA) arteries (Figures 3 and 4).
There was no supply of the inferior aspect of the
interventricular septum by a posterior branch from the RCA
or left circumflex coronary artery (LCx). The proximal
LAD was normal, with well-developed septal branches but no diagonal
branches filled by contrast on the anterolateral free wall of the left
ventricle (Figure 4). Intracoronary infusion of
nitroglycerin showed no significant changes in
coronary artery diameter (Figure 5). Total cholesterol, HDL,
sedimentation rate, serological studies for connective-tissue diseases,
antithrombin III, protein C, protein S, endogenous tissue
plasminogen activator, and
plasminogen activator inhibitor
were normal. The patient was discharged home on diltiazem after a
normal maximal exercise test 16 days after admission.
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In 85% of patients, the coronary circulation is
right-dominant, and the RCA supplies the inferior aspect of
the interventricular septum by giving rise to the posterior
descending artery. The LCx, which is often small, does not reach the
crux of the heart. Conversely, when the LCx is the dominant
coronary artery, it courses to the crux of the heart and the
RCA is often small.1 In 
7% of patients, there is a
codominant or balanced system in which both RCA and LCx give rise to a
posterior descending branch. Hypoplastic coronary artery
disease (HCAD) occurs rarely and refers to the underdevelopment of 
1
coronary arteries or their major branches.2 Most
of the patients reported were young adults and experienced sudden
cardiac death without antecedent symptoms. Diagnosis is often made at
autopsy.1 2 Although reversible myocardial
ischemia has previously been angiographically documented in an
infant, it is unusual to see a patient with myocardial infarction and
isolated HCAD diagnosed at coronary angiography, as in our
patient.3 Hypoplasia of the RCA and LCx with no posterior
descending artery supplying the inferior aspect of the
interventricular septum is more commonly
found.1 Hypoplasia of the LAD has also been
reported.2 3 In addition, HCAD was found in several cases
of myocardial infarction distal to atherosclerotic or thrombotic
occlusions.2
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
References
1. Roberts WC, Glick BN. Congenital hypoplasia of both right and left circumflex coronary arteries. Am J Cardiol. 1992;70:121–123.[Medline] [Order article via Infotrieve]
2. Zugibe FT, Zugibe FT Jr, Costello JT, Breithaupt MK. Hypoplastic coronary artery disease within the spectrum of sudden unexpected death in young and middle age adults. Am J Forensic Med Pathol. 1993;14:276–283.[Medline] [Order article via Infotrieve]
3. Casta A. Hypoplasia of the left coronary artery complicated by reversible myocardial ischemia in a newborn. Am Heart J. 1987;114:1238–1241.[Medline] [Order article via Infotrieve]
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