Myocardial Histopathology in Patients With Obstructive Hypertrophic Cardiomyopathy |
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Authors: | Hao Cui Hartzell V. Schaff Juliano Lentz Carvalho Rick A. Nishimura Jeffrey B. Geske Joseph A. Dearani Brian D. Lahr Alexander T. Lee J. Martijn Bos Michael J. Ackerman Steve R. Ommen Joseph J. Maleszewski |
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Affiliation: | 1. Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA;2. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA;3. Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota, USA;4. Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota, USA;5. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA |
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Abstract: | BackgroundHypertrophic cardiomyopathy (HCM) is characterized by multiple pathological features including myocyte hypertrophy, myocyte disarray, and interstitial fibrosis.ObjectivesThis study sought to correlate myocardial histopathology with clinical characteristics of patients with obstructive HCM and post-operative outcomes following septal myectomy.MethodsThe 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.ResultsThe 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 < 0.001). Myocyte hypertrophy (p < 0.001), myocyte disarray (p < 0.001), and interstitial fibrosis (p < 0.001) were positively associated with implantable cardioverter-defibrillator implantation. Interstitial fibrosis (p < 0.001) and endocardial thickening (p < 0.001) were associated with atrial fibrillation pre-operatively. In the Cox survival model, older age (p < 0.001), lower degree of myocyte hypertrophy (severe vs. mild hazard ratio: 0.41; 95% confidence interval: 0.19 to 0.86; p = 0.040), and lower degree of endocardial thickening (moderate vs. mild hazard ratio: 0.75; 95% confidence interval: 0.58 to 0.97; p = 0.019) were independently associated with worse post-myectomy survival. Among 256 patients who had genotype analysis, patients with pathogenic or likely pathogenic variants (n = 62) had a greater degree of myocyte disarray (42% vs. 15% vs. 20%; p = 0.022). Notably, 13 patients with pathogenic or likely pathogenic genetic variants of HCM had no myocyte disarray.ConclusionsHistopathology 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. |
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Keywords: | histology hypertrophic cardiomyopathy interstitial fibrosis myocyte disarray myocyte hypertrophy AF" },{" #name" :" keyword" ," $" :{" id" :" kwrd0040" }," $$" :[{" #name" :" text" ," _" :" atrial fibrillation HCM" },{" #name" :" keyword" ," $" :{" id" :" kwrd0050" }," $$" :[{" #name" :" text" ," _" :" hypertrophic cardiomyopathy ICD" },{" #name" :" keyword" ," $" :{" id" :" kwrd0060" }," $$" :[{" #name" :" text" ," _" :" implantable cardioverter-defibrillator LGE" },{" #name" :" keyword" ," $" :{" id" :" kwrd0070" }," $$" :[{" #name" :" text" ," _" :" late gadolinium enhancement P/LP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0080" }," $$" :[{" #name" :" text" ," _" :" pathogenic or likely pathogenic RBC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0090" }," $$" :[{" #name" :" text" ," _" :" red blood cell VUS" },{" #name" :" keyword" ," $" :{" id" :" kwrd0100" }," $$" :[{" #name" :" text" ," _" :" variant of uncertain significance |
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