Myocardial bridging does not predict sudden death in children with hypertrophic cardiomyopathy but is associated with more severe cardiac disease.
OBJECTIVE
We sought to examine the association between systolic compression of sections of epicardial coronary vessels (myocardial bridging) with myocardial perfusion abnormalities and clinical outcome in children with hypertrophic cardiomyopathy (HCM).


BACKGROUND
It has recently been suggested that myocardial bridging is an important cause of myocardial ischemia and sudden death in children with HCM.


METHODS
Angiograms from 57 children with HCM were reviewed for the presence of bridging (50% or more maximum systolic arterial compression). QT interval indices, echocardiographic and cardiac catheterization findings, treadmill exercise tests, exercise thallium scintigraphy, Holter monitoring and electrophysiologic study findings were compared in children with and without bridging. The findings were also related to the presence or absence of compression of septal branches of the left anterior descending artery (LAD).


RESULTS
Bridging was present in 23 (40%) of the children. Multiple coronary arteries were involved in four children. Bridging involved the LAD in 16 of 28 (57%) affected vessels. Myocardial perfusion abnormalities were present in 14 of 30 (47%) children without bridging and in 17 of 22 (94%) children with bridging, p = 0.002. However, bridging was associated with more severe septal hypertrophy (19+/-8 mm vs. 28+/-8 mm, p < 0.001), a higher septum:posterior wall thickness ratio (2.7+/-1.2 vs. 1.8+/-0.9, p < 0.001), and higher left ventricle (LV) outflow gradient (45+/-37 mm Hg vs. 16+/-28 mm Hg, p = 0.002). Compression of septal LAD branches was present in 37 (65%) of the children and was significantly associated with bridging, severity of LV hypertrophy and outflow obstruction. Multivariate analysis demonstrated that LV septal thickness and septal branch compression, and not bridging, were independent predictors of thallium perfusion abnormalities. There was a 90% power at 5% significance to detect an effect of bridging on thallium abnormalities at an odds ratio of 3. Bridging was also not associated with significantly greater symptoms, increased QT and QTc intervals and QTc dispersion, ventricular tachycardia on Holter or induced at EP study, or a worse prognosis.


CONCLUSIONS
Bridging and compression of septal branches of the LAD are common in HCM children and are related to magnitude of LV hypertrophy. Left ventricular hypertrophy and compression of intramyocardial branches of the epicardial coronary arteries may contribute to myocardial perfusion abnormalities. Our findings suggest that bridging does not result in myocardial ischemia and may not cause arrhythmias or sudden death in HCM children.
Mohiddin
S A
SA
Section of Electrophysiology and Inherited Heart Diseases, Cardiology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland, USA.
Begley
D
D
Shih
J
J
Fananapazir
L
L
D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000401:mortality* / Q000473:pathology* / Q000503:physiopathology; D002648:Child; D003251:Constriction, Pathologic; D003331:Coronary Vessels / Q000473:pathology*; D016757:Death, Sudden, Cardiac; D004562:Electrocardiography; D005080:Exercise Test; D005260:Female; D006439:Hemodynamics; D006801:Humans; D008297:Male; D011379:Prognosis; D013794:Thallium Radioisotopes
D013794:Thallium Radioisotopes