Myocardial Histopathology in Patients With Obstructive Hypertrophic Cardiomyopathy.

Published on May 4, 2021in Journal of the American College of Cardiology20.589
· DOI :10.1016/J.JACC.2021.03.008
Hao Cui4
Estimated H-index: 4
(Mayo Clinic),
Hartzell V. Schaff131
Estimated H-index: 131
(Mayo Clinic)
+ 9 AuthorsJoseph J. Maleszewski41
Estimated H-index: 41
(Mayo Clinic)
Abstract Background Hypertrophic cardiomyopathy (HCM) is characterized by multiple pathological features including myocyte hypertrophy, myocyte disarray, and interstitial fibrosis. Objectives This study sought to correlate myocardial histopathology with clinical characteristics of patients with obstructive HCM and post-operative outcomes following septal myectomy. Methods The authors reviewed the pathological findings of the myocardial specimens from 1,836 patients with obstructive HCM who underwent septal myectomy from 2000 to 2016. Myocyte hypertrophy, myocyte disarray, interstitial fibrosis, and endocardial thickening were graded and analyzed. Results The median age at operation was 54.2 years (43.5 to 64.3 years), and 1,067 (58.1%) were men. A weak negative correlation between myocyte disarray and age at surgery was identified (ρ = −0.22; p  Conclusions Histopathology was associated with clinical manifestations including the age of disease onset and arrhythmias. Myocyte hypertrophy and endocardial thickening were negatively associated with post-myectomy mortality.
Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from P...
54 CitationsSource
#1Anita Nguyen (Mayo Clinic)H-Index: 10
#2Hartzell V. Schaff (Mayo Clinic)H-Index: 131
Last. Steve R. Ommen (Mayo Clinic)H-Index: 78
view all 8 authors...
Background Survival of patients with obstructive hypertrophic cardiomyopathy who undergo septal myectomy appears to be excellent and, in some series, is similar to that of matched populations. In this study, we investigated causes of late death in a large cohort of surgical patients. Methods Between January 1961 and October 2017, 2,956 patients underwent surgery for hypertrophic cardiomyopathy. We reviewed medical records of these patients. Vital status and causes of mortality were verified usin...
10 CitationsSource
#1Hao Cui (Mayo Clinic)H-Index: 4
#2Hartzell V. Schaff (Mayo Clinic)H-Index: 131
Last. Steve R. Ommen (Mayo Clinic)H-Index: 78
view all 7 authors...
Abstract Background Conduction abnormalities including bundle branch block are recognized sequelae following septal myectomy in patients with hypertrophic cardiomyopathy, but their incidence and effect on long-term mortality have not been well studied. Objectives This study was conducted to determine the impact of conduction abnormalities on mortality in patients with obstructive hypertrophic cardiomyopathy following myectomy. Methods The outcomes of 2,482 patients with obstructive hypertrophic ...
16 CitationsSource
#1Patricia Garcia-Canadilla (UCL: University College London)H-Index: 8
#2Andrew C. Cook (UCL: University College London)H-Index: 33
Last. Gabriella Captur (UCL: University College London)H-Index: 26
view all 9 authors...
Myoarchitectural disarray – the multiscalar disorganisation of myocytes, is a recognised histopathological hallmark of adult human hypertrophic cardiomyopathy (HCM). It occurs before the establishment of left ventricular hypertrophy (LVH) but its early origins and evolution around the time of birth are unknown. Our aim is to investigate whether myoarchitectural abnormalities in HCM are present in the fetal heart. We used wild‐type, heterozygous and homozygous hearts (n = 56) from a Mybpc3‐target...
14 CitationsSource
#1Rina Ariga (University of Oxford)H-Index: 14
#2Elizabeth M. Tunnicliffe (University of Oxford)H-Index: 12
Last. Hugh Watkins (University of Oxford)H-Index: 134
view all 10 authors...
Abstract Background Myocardial disarray is a likely focus for fatal arrhythmia in hypertrophic cardiomyopathy (HCM). This microstructural abnormality can be inferred by mapping the preferential diffusion of water along cardiac muscle fibers using diffusion tensor cardiac magnetic resonance (DT-CMR) imaging. Fractional anisotropy (FA) quantifies directionality of diffusion in 3 dimensions. The authors hypothesized that FA would be reduced in HCM due to disarray and fibrosis that may represent the...
42 CitationsSource
#1Gabrielle Norrish (UCL: University College London)H-Index: 7
#2Ella Field (UCL: University College London)H-Index: 7
Last. Juan Pablo Kaski (UCL: University College London)H-Index: 21
view all 18 authors...
AIMS: Understanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort. METHODS AND RESULTS: Six hundred and eighty-seven patients with HCM presented at a median age of 5.2 yea...
25 CitationsSource
Hypertrophic Cardiomyopathy HCM is the most common genetic disorder of the heart, with 1 case per 200 to 500 persons, and often remains clinically silent. HCM is the most common cause of sudden dea...
191 CitationsSource
#1Rick A. Nishimura (Mayo Clinic)H-Index: 128
#2Hubert Seggewiss (Mayo Clinic)H-Index: 1
Last. Hartzell V. Schaff (Mayo Clinic)H-Index: 131
view all 3 authors...
Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be ...
56 CitationsSource
#1Giovanni QuartaH-Index: 21
#2Michael Papadakis (St George's, University of London)H-Index: 42
Last. Iacopo OlivottoH-Index: 64
view all 8 authors...
Genetic cardiomyopathies are complex diseases with heterogeneous clinical presentations and phenotypes, and mild pathology can overlap with physiological variation, particularly in athletes. In this Review, three of the most common and controversial areas are discussed, including left ventricular hypertrophy; left ventricular dilatation, noncompaction, and fibrosis; and arrhythmias originating from the right ventricle.
18 CitationsSource
#1Godelieve R. F. Claes (Maastricht University Medical Centre)H-Index: 5
#2Florence H J van Tienen (Maastricht University Medical Centre)H-Index: 7
Last. Arthur van den Wijngaard (Maastricht University Medical Centre)H-Index: 21
view all 23 authors...
Aims Phenotypic heterogeneity and incomplete penetrance are common in patients with hypertrophic cardiomyopathy (HCM). We aim to improve the understanding in genotype–phenotype correlations in HCM, particularly the contribution of an MYL2 founder mutation and risk factors to left ventricular hypertrophic remodelling. Methods and results We analysed 14 HCM families of whom 38 family members share the MYL2 c.64G > A [p.(Glu22Lys)] mutation and a common founder haplotype. In this unique cohort, we ...
45 CitationsSource
Cited By3
#1Diego PenelaH-Index: 18
#2Antonio SorgenteH-Index: 18
Last. Riccardo CappatoH-Index: 37
view all 3 authors...
Atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM) are two very common clinical entities, which often occur simultaneously, giving a hard time to both patients and cardiologists. Myocyte hypertrophy, myocyte disarray and interstitial fibrosis in the left atrium (LA) predisposes to atrial arrhythmias due to modifications of the substrate that promote re-entry. AF is usually poorly tolerated due to the shortening of the diastolic time with rapid heart rates and the lack of the atrial c...
Atrial fibrillation (AF) leads to remodeling characterized by changes in both size and shape of the left atrium (LA). Here we aimed to study the effect of hypertrophic cardiomyopathy (HCM) on the pattern of LA remodeling in AF-patients. HCM-patients (n = 23) undergoing AF ablation (2009-2012) were matched and compared with 125 Non-HCM patients from our prospective registry. Pre-procedural CT data were analyzed (EnSite Verismo, SJM, MN) to determine the maximal sagittal (anterior-posterior, AP), ...
#1Ethan J. Rowin (Tufts Medical Center)H-Index: 26
#2Michael A. Fifer (Harvard University)H-Index: 43