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Microvascular coronary disease and myocardial fibrosis within the spectrum of hypertrophic cardiomyopathy: a histopathologic study.

Session Atrial fibrillation in hypertrophic cardiomyopathy

Speaker Alberto Foa

Congress : ESC Congress 2018

  • Topic : arrhythmias and device therapy
  • Sub-topic : Hypertrophic Cardiomyopathy
  • Session type : Abstract Session
  • FP Number : 1473

Authors : A Foa (Bologna,IT), C Rapezzi (Bologna,IT), I Olivotto (Florence,IT), F Cecchi (Florence,IT), R Coppini (Florence,IT), C Ferrantini (Florence,IT), P Stefano (Florence,IT), V Agostini (Bologna,IT), G Vitale (Bologna,IT), R Ditaranto (Bologna,IT), E Biagini (Bologna,IT), O Leone (Bologna,IT)

A. Foa1 , C. Rapezzi1 , I. Olivotto2 , F. Cecchi2 , R. Coppini2 , C. Ferrantini2 , P. Stefano3 , V. Agostini4 , G. Vitale1 , R. Ditaranto1 , E. Biagini1 , O. Leone4 , 1University Hospital Policlinic S. Orsola-Malpighi, Cardiology - Bologna - Italy , 2Careggi University Hospital (AOUC), Cardiology - Florence - Italy , 3Careggi University Hospital (AOUC) - Florence - Italy , 4University Hospital Policlinic S. Orsola-Malpighi, Pathology - Bologna - Italy ,

European Heart Journal ( 2018 ) 39 ( Supplement ), 276

Background: Although several radiological imaging techniques have demonstrated the existence of microvascular coronary abnormalities in patients with hypertrophic cardiomyopathy (HCM), a detailed histopathologic assessment is lacking. We had the opportunity to analyse explanted hearts of end-stage (ES) HCM and surgical samples of patients undergoing septal myectomy for obstructive HCM.

Purpose: To assess and compare histopathologic characteristic of patients with obstructive HCM versus ES-HCM.

Methods: Surgical samples of 27 patients who underwent septal myectomy for obstructive HCM and 30 explanted hearts for ES-HCM were retrospectively analysed. Myocardial fibrosis extent was determined by a histomorphometric quantitative analysis using a dedicated software; types of fibrosis were qualitatively assessed and classified as “replacement” (scar-like) and “interstitial” myocardial fibrosis. Coronary arterioles were evaluated separately according to lumen diameter: 100–500 micron and <100 micron. For each specimen the presence of microvasculopathy, including tunica media hypertrophy and/or fibrosis as well as intimal abnormalities, was evaluated in a semi-quantitative manner. Microvasculopathy was classified as mild if generating a lumen stenosis <30%, moderate >30% and <60%, severe >60%. Myocyte abnormalities were evaluated considering hypertrophy, myofibrillar disarray and vacuolization. For the purpose of this comparative study only anterobasal septum of ES-HCM explanted hearts was considered.

Results: Mean age of our population was 46.8±12 in transplanted patients and 45.4±13.5 in patients who underwent septal myectomy. The average amount of fibrosis in the anterobasal septum of explanted hearts was 36.8% ± 20.4 as opposed to 12.8% ± 8.46 of post-myectomy surgical samples (p<0.001). Type of fibrosis was also different between subgroups: replacement in 53.3% of ES-HCM vs 14.8% in myectomies specimens (p=0.005), interstitial in 26.7% of anterobasal septum of explanted hearts vs 81.5% in myectomies samples (p<0.001). Almost all specimens showed alterations of 100–500 micron coronary arterioles (93.3% ES-HCM and 100% in myectomies); the majority of specimens showed involvement of both tunica media and the intimal layer (80% ES-HCM, 77.8% myectomies). Approximately one quarter of cases showed severe 100–500 micron arterioles stenosis (30% explanted hearts, 25% myectomies). Microvasculopathy of smaller coronary arterioles (<100 micron) was less frequent however without significant differences between subgroups (73.3% ES-HCM, 77.8% myectomies). Diffuse myocyte vacuolization was detected only in ES-HCM specimens (23.3% vs 0, p=0.011).

Conclusions: Microvascular coronary disease is frequent in all the spectrum of HCM and involves both medial and intimal layers. Although interstitial fibrosis is present in both ES-HCM and myectomies samples, scar-like fibrosis along with advanced myocyte abnormalities are distinctive patterns of ES patients.

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