Evaluation of pathological criteria for diagnosis of hypertrophic cardiomyopathy

Abstract

This study aims to evaluate and compare the current pathological criteria for the diagnosis of hypertrophic cardiomyopathy (HOCM). The following criteria were applied to 39 autopsy patients whose hearts showed myofibre disarray: (i) disarray involving more than 5% of ventricular septal myofibres, (ii) asymmetrical septal hypertrophy (ASH), (iii) mirror-image subaortic plaque, and (iv) a positive histologic HOCM index (HHI). Group 1 (27 patients) with greater than 5% ventricular septal myofibre disarray were diagnosed as HCM, whereas group 2 (12 patients) had less than 5% disarray. The mean disarray value in group 1 was 52% compared to 1.2% in group 2. The two groups showed significant differences regarding ASH and in the amounts of myofibre disarray in the free walls of both ventricles. A new finding was that the histologic HOCM index was significantly higher in patients who died suddenly. The HHI was the commonest positive criterion in group 1, followed by ASH and a mirror-image endocardial plaque. None of the current imperfect pathological criteria for the diagnosis of HCM can be used as the 'gold standard'.

Hypertrophic Cardiomyopathy Phenotype Revisited After 50 Years With Cardiovascular Magnetic Resonance

“…These observations support an emerging role for CMR in the contemporary evaluation of Hypertrophic cardiomyopathy (HCM) has historically been regarded as a disease characterized by hypertrophy involving particularly substantial portions of the left ventricular (LV) wall (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). These perceptions regarding the morphology of HCM were derived largely from nontomographic, 2-dimensional echocardiographic imaging, as well as postmortem studies (5)(6)(7)(8)(9)(10)(11)(12)14). Volumetric cardiovascular magnetic resonance (CMR) offers advantages of high spatial resolution and 3-dimensional tomographic imaging thereby allowing for better characterization of the pattern and distribution of LV hypertrophy in HCM (15)(16)(17)(18)(19)(20)(21).…”

“…Contiguous portions of anterior free wall and septum constituted the predominant region of wall thickening, with implications for clinical diagnosis. These observations support an emerging role for CMR in the contemporary evaluation of Hypertrophic cardiomyopathy (HCM) has historically been regarded as a disease characterized by hypertrophy involving particularly substantial portions of the left ventricular (LV) wall (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). These perceptions regarding the morphology of HCM were derived largely from nontomographic, 2-dimensional echocardiographic imaging, as well as postmortem studies (5)(6)(7)(8)(9)(10)(11)(12)14).…”

Kalpte Yaygın Fibrozis Bulunan 12 Yaşında Anı Kardiyak Ölüm Olgusu

“…McKenna ve arkadaß-larõ, kalp aÛõrlõÛõ ve duvar kalõnlõk artõßlarõnõn bazõ ailesel hipertrofili olgularda her zaman belirgin olmayabileceÛi-ni belirtmißlerdir (12). Histopatolojik ve morfometrik analizlerde sol ventrikŸl ve asimetrik septum kalõnlõk artõßlarõndan sšzedilmekte (4,6,13), duvar kalõnlõklarõnõn, olgumuzda olduÛu gibi 20 mm. Ÿzerindeki deÛerleri ise artmõß ani šlŸm riski ile ilißkilindirilmektedir (6,13,14).…”

“…Histopatolojik ve morfometrik analizlerde sol ventrikŸl ve asimetrik septum kalõnlõk artõßlarõndan sšzedilmekte (4,6,13), duvar kalõnlõklarõnõn, olgumuzda olduÛu gibi 20 mm. Ÿzerindeki deÛerleri ise artmõß ani šlŸm riski ile ilißkilindirilmektedir (6,13,14). Spirito ve arkadaßlarõ duvar kalõnlõk artõßõnõ baÛõmsõz prognostik faktšr olarak šne •õkarmaktadõr (15).…”

“…Olgumuzda da bulunan adale liflerinde hipertrofik gšrŸnŸm ve lif dŸzensizliÛi, belirleyici hipertrofik kardiyomiyo-pati kriterleri olarak sunulmakta (6,13), miyosit lif dŸ-zensizliÛi prognostik belirte• olarak šne •õkmaktadõr (16). Olgumuzda da izlendiÛi ßekilde kalp adale liflerinde fibrozisin saptanmasõ šnemli bulgu olarak šne •õk-maktadõr (6,13,17). Choudhury ve arkadaßlarõ, yaygõn miyokardiyal fibrozisin sõklõkla asemptomatik olgularda gšrŸldŸÛŸ ve hipertrofi bšlgelerinde tespit edildiÛini belirtmekte (18) iken, bazõ araßtõrmacõlar ise fibrozisin kalbin elektriksel aktivitesini etkileyerek ani šlŸme neden olabileceÛi gibi ritim dŸzensizlikleri ile semptom verebileceÛini savunmaktadõr (6,16).…”