Document Detail

Geometric Assessment of Asymmetric Septal Hypertrophic Cardiomyopathy by CMR.
MedLine Citation:
PMID:  22789938     Owner:  NLM     Status:  In-Data-Review    
OBJECTIVES: The aim of this study was to analyze the geometric pattern of hypertrophy (HT) in patients with asymmetrical septal hypertrophic cardiomyopathy (HCM) using cardiac magnetic resonance (CMR) and to test the hypothesis that at least in some patients, the HT follows a longitudinal spiral pattern.
BACKGROUND: The highly heterogeneous phenotypic expression of HCM is a well-known phenomenon. CMR has emerged as a robust 3-dimensional (3D) tomographic imaging technique that is increasingly used to explore phenotypic expression.
METHODS: Short-axis cine CMR was used to study the 3D extent of HT (i.e., radial, circumferential, and longitudinal extent, as well as the relation between circumferential and longitudinal extent). Inclusion criteria were septal wall thickness (WT) ≥15 mm and septal to free wall WT ratio >1.3.
RESULTS: CMR was performed in 132 patients. Maximal WT was 22 ± 5 mm, with a circumferential extent of 131 ± 51°, and a longitudinal extent of 64 ± 19%, resulting in a hypertrophied left ventricular (LV) surface of 26 ± 15%. Linear regression analysis showed in 86% of patients a consistent course of HT along the longitudinal direction. The HT invariably started at the basal anteroseptum and rotated, except in 2 patients, in a counterclockwise direction (CC-spiral patients) with a mean global rotation of 116 ± 68° (range 5° to 350°). After the CC-spiral patients were divided according to magnitude of rotation quartiles (Q1: 5° to 70°, Q2: 75° to 105°, Q3: 110° to 150°, and Q4: 155° to 350°), Q4 patients were significantly older and had more LV outflow tract obstruction and hypertension than patients without the spiraling pattern. In 11 patients, continuation of HT into an apical form of HCM was found.
CONCLUSIONS: Using 3D analysis, we found that the majority of patients with asymmetrical septal HCM in fact showed a spiral pattern of HT following a counterclockwise (or "left-handed") spiral trajectory. The variation in magnitude of rotation among patients, however, was highly variable. Further research is warranted to better understand the significance of the current findings, in particular to relate them to the genetic and morphological substrate, hemodynamic consequences, and patient outcome.
Anca Florian; Pier Giorgio Masci; Stijn De Buck; Giovanni Donato Aquaro; Piet Claus; Giancarlo Todiere; Johan Van Cleemput; Massimo Lombardi; Jan Bogaert
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  JACC. Cardiovascular imaging     Volume:  5     ISSN:  1876-7591     ISO Abbreviation:  JACC Cardiovasc Imaging     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-07-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101467978     Medline TA:  JACC Cardiovasc Imaging     Country:  United States    
Other Details:
Languages:  eng     Pagination:  702-11     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
Department of Radiology, UZ Leuven, Leuven, Belgium.
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