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Significance of left ventricular apical–basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy


Gruner, Christiane; Chan, Raymond H; Crean, Andrew; Rakowski, Harry; Rowin, Ethan J; Care, Melanie; Deva, Djeven; Williams, Lynne; Appelbaum, Evan; Gibson, C Michael; Lesser, John R; Haas, Tammy S; Udelson, James E; Manning, Warren J; Siminovitch, Katherine; Ralph-Edwards, Anthony C; Rastegar, Hassan; Maron, Barry J; Maron, Martin S (2014). Significance of left ventricular apical–basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy. European Heart Journal, 35(39):2706-2713.

Abstract

Aims Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e. apical-basal). Methods and results CMR was performed in 230 genotyped HCM patients (48 ± 15 years, 69% male), 30 genotype-positive/phenotype-negative (G+/P−) family members (32 ± 15 years, 30% male), and 126 controls. Left ventricular apical-basal muscle bundle was identified in 145 of 230 (63%) HCM patients, 18 of 30 (60%) G+/P− family members, and 12 of 126 (10%) controls (G+/P− vs. controls; P < 0.01). In HCM patients, the prevalence of an apical-basal muscle bundle was similar among those with disease-causing sarcomere mutations compared with patients without mutation (64 vs. 62%; P = 0.88). The presence of an LV apical-basal muscle bundle was not associated with LV outflow tract obstruction (P = 0.61). In follow-up, 33 patients underwent surgical myectomy of whom 22 (67%) were identified to have an accessory LV apical-basal muscle bundle, which was resected in all patients. Conclusion Apical-basal muscle bundles are a unique myocardial structure commonly present in HCM patients as well as in G+/P− family members and may represent an additional morphologic marker for HCM diagnosis in genotype-positive status

Abstract

Aims Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e. apical-basal). Methods and results CMR was performed in 230 genotyped HCM patients (48 ± 15 years, 69% male), 30 genotype-positive/phenotype-negative (G+/P−) family members (32 ± 15 years, 30% male), and 126 controls. Left ventricular apical-basal muscle bundle was identified in 145 of 230 (63%) HCM patients, 18 of 30 (60%) G+/P− family members, and 12 of 126 (10%) controls (G+/P− vs. controls; P < 0.01). In HCM patients, the prevalence of an apical-basal muscle bundle was similar among those with disease-causing sarcomere mutations compared with patients without mutation (64 vs. 62%; P = 0.88). The presence of an LV apical-basal muscle bundle was not associated with LV outflow tract obstruction (P = 0.61). In follow-up, 33 patients underwent surgical myectomy of whom 22 (67%) were identified to have an accessory LV apical-basal muscle bundle, which was resected in all patients. Conclusion Apical-basal muscle bundles are a unique myocardial structure commonly present in HCM patients as well as in G+/P− family members and may represent an additional morphologic marker for HCM diagnosis in genotype-positive status

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Item Type:Journal Article, refereed, original work
Communities & Collections:National licences > 142-005
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Uncontrolled Keywords:Cardiovascular magnetic resonance; Genotype; Hypertrophic cardiomyopathy; Muscle bundle
Language:English
Date:14 October 2014
Deposited On:30 Oct 2018 16:11
Last Modified:29 Sep 2019 05:52
Publisher:Oxford University Press
ISSN:0195-668X
OA Status:Green
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1093/eurheartj/ehu154
Related URLs:https://www.swissbib.ch/Search/Results?lookfor=nationallicenceoxford101093eurheartjehu154 (Library Catalogue)
PubMed ID:24810389

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