Myocardial fibrosis in patients with symptomatic obstructive hypertrophic cardiomyopathy: correlation with echocardiographic measurements, sarcomeric genotypes, and pro-left ventricular hypertrophy polymorphisms involving the renin-angiotensin-aldosterone system

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is a heterogeneous disorder of the cardiac sarcomere, resulting in myocyte hypertrophy and disarray, interstitial fibrosis, and cardiac dysfunction. Our aim was to determine whether the amount of fibrosis in HCM correlates with echocardiographi...

Full description

Saved in:
Bibliographic Details
Published in:Cardiovascular pathology 2009-09, Vol.18 (5), p.262-268
Main Authors: Blauwet, Lori A, Ackerman, Michael J, Edwards, William D, Riehle, Darren L, Ommen, Steve R
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Cardiac fibrosis
Cardiomyopathies - diagnostic imaging
Cardiomyopathies - genetics
Cardiomyopathies - pathology
Cardiomyopathy, Hypertrophic - diagnostic imaging
Cardiomyopathy, Hypertrophic - genetics
Cardiomyopathy, Hypertrophic - pathology
Child
Chromatography, High Pressure Liquid
Diastolic dysfunction
Echocardiography
Female
Fibrosis
Gene mutations
Genetic Predisposition to Disease
Genotype
Humans
Hypertrophic cardiomyopathy
Hypertrophy, Left Ventricular - diagnostic imaging
Hypertrophy, Left Ventricular - genetics
Hypertrophy, Left Ventricular - pathology
Male
Middle Aged
Pathology
Polymerase Chain Reaction
Polymorphism, Genetic
Renin-Angiotensin System - genetics
Sarcomeres - genetics
Young Adult
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Introduction Hypertrophic cardiomyopathy (HCM) is a heterogeneous disorder of the cardiac sarcomere, resulting in myocyte hypertrophy and disarray, interstitial fibrosis, and cardiac dysfunction. Our aim was to determine whether the amount of fibrosis in HCM correlates with echocardiographic measures of diastolic dysfunction, presence of HCM-susceptibility mutations, or polymorphisms in the renin-angiotensin-aldosterone system (RAAS). Methods Surgical specimens from patients with obstructive HCM undergoing septal myectomy at the Mayo Clinic (2001–2004) were examined and compared with autopsy-derived tissues from age- and sex-matched normal controls. Digital image analysis was used to quantitate the fibrosis in representative microscopic sections. Genotyping was performed for myofilament-HCM using polymerase chain reaction, high-performance liquid chromatography, and direct DNA sequencing. RAAS polymorphism status was similarly established. Results The study included 59 HCM cases and 44 controls. Patients with HCM exhibited more fibrosis (mean 17%, range 3–45%) than controls (mean 8%, range 3–17%) ( P
ISSN:1054-8807
1879-1336
DOI:10.1016/j.carpath.2008.08.003