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EDITORIAL
Year : 2001  |  Volume : 47  |  Issue : 3  |  Page : 163-4

Hypertrophic cardiomyopathy: the elusive terrorist?



Correspondence Address:
Y Lokhandwala


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Source of Support: None, Conflict of Interest: None


PMID: 11832615

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Keywords: Autopsy, Biopsy, Cardiomyopathy, Hypertrophic, pathology,Human,


How to cite this article:
Lokhandwala Y. Hypertrophic cardiomyopathy: the elusive terrorist?. J Postgrad Med 2001;47:163

How to cite this URL:
Lokhandwala Y. Hypertrophic cardiomyopathy: the elusive terrorist?. J Postgrad Med [serial online] 2001 [cited 2019 Nov 11];47:163. Available from: http://www.jpgmonline.com/text.asp?2001/47/3/163/203


Described a century ago, hypertrophic cardiomyopathy (HCM) is a unique disease with characteristic inappropriate myocardial hypertrophy that occurs in the absence of obvious cause for hypertrophy.[1],[2],[3],[4],[5] The macroscopic and microscopic features of this disease have been under detailed study since 1950. This is particularly significant in the background of it being one of the most common genetically transmitted cardiac disorders. There is scant data regarding this in Indian literature. In this context, the present study by Phadke et al of 14 cases of HCM is commendable. Phadke et al have critically analysed the pathological data of 14 retrospective cases of HCM. Their study spans 15 years during which time they confirmed HCM in about 14% of all cases of cardiomyopathy as identified in post-mortem. The macroscopic features include marked myocardial hypertrophy with relatively small left ventricular cavity. The finding that predominant left ventricular involvement is commoner than right ventricular is commensurate with our clinical experience.

The pattern and extent of hypertrophy in HCM varies greatly from patient to patient and the characteristic feature is heterogeneity in the amount of hypertrophy evident in the different regions of left ventricle.[6],[7] A feature found in most patients with HCM is disproportionate involvement of interventricular septum and anterolateral wall compared with the posterior segment of the free wall of left ventricle. Echocardiographically, mild RV hypertrophy is very difficult to diagnose with certainty.

The cardinal microscopic features described in the literature include hypertrophy and disarray of muscle bundle.[8],[9] Fibrosis is usually prominent & may be extensive enough to produce grossly visible scars. What is unique about the disarray in HCM is its ubiquity and frequency. In the present study, they have attempted to grade the myocardial disarray & have taken it together with other features like hypertrophy, degeneration, fibrosis, inflammation and obliterative small vessel disease. Many investigators feel that myocardial disarray is not a very specific feature of HCM.[10],[11] But other investigators have suggested that myocardial disarray is a specific marker for HCM only when considered in a quantitative rather than qualitative fashion.[12],[13] They have showed that a cut off of 5% best differentiated patients with HCM from those with other cardiac diseases. Phadke et al were able to demonstrate in the present study that the disarray was quite variably distributed and often patchy and the diagnostic yield was 40-50% of an average of 20 sections. Also bizarre myocyte hypertrophy, large nuclei, myocyte degeneration, scarring and inflammatory infiltrates were usual accompaniments. Obliterative small vessel disease was present in 50% of this series as compared to >80% in some other series.[6],[14]

The clinical correlates of the hypertrophy and disarray are impaired relaxation (diastolic dysfunction), outflow obstruction and reentrant circuits. In later stages there is ventricular dilation and impaired contractility leading to heart failure. The diastolic dysfunction leads to pulmonary congestion, manifesting as dyspnea. The outflow obstruction produces giddiness on exertion and chest pain due to demand-supply mismatch. Reentrant circuits lead to ventricular tachycardia, which could sometimes be fatal. Sudden death is known and the high risk markers for this include a young age (less than 30 years) at diagnosis, family history of sudden death, history of syncope, non-sustained ventricular tachycardia on Holter, severe hypertrophy and identifiable genetic abnormalitities.

This brings us to the practical aspects of histological diagnosis. A histological diagnosis would require extensive sampling, so as to rule out false negative and false positive diagnoses. But with presently available techniques of transcatheter endomyocardial biopsy, only small tissue samples (up to four or five) are available for study. Hence everything boils down to the fact that biopsy diagnosis in life cannot really refute or confirm the diagnosis of HCM. The main role of biopsy is to provide aetiological diagnoses like amyloidosis, sarcoidosis, etc.

To conclude, the histological diagnosis of HCM can be confidently made by extensive studies at autopsy as demonstrated in this series, which is not possible with the current endomyocardial biopsy techniques. Hence the role of biopsy in the diagnosis and management of HCM at present is dubious at best. But detailed autopsy studies do help to confirm the diagnosis especially in sudden cardiac deaths so that the family members can be spared the trauma of further genetic screening.


  ::   Acknowledgments: Top


The author acknowledges the help of Dr. P. J. Nathani, Department of Cardiology, Seth G. S. Medical College and K. E. M. Hospital for the help in writing this manuscript.

 
 :: References Top

1. Braunwald E, Morrow AG, Cornell WP. Idiopathic hypertrophic subaortic stenosis; clinical, hemodynamic and angiographic manifestations. Am J Med 1960; 29:924.  Back to cited text no. 1    
2.Braundwald E, Lambrew CT, Rockoff SD. Idiopathic hypertrophic subaortic stenosis. A discription of the disease based on an analysis of 64 patients. Circulation 1964; 30(Suppl 4):3-119.  Back to cited text no. 2    
3.Spirito P, Seidman CE, McKenna WJ, Maron BJ. The management of hypertrophic cardiomyopathy. N Eng J Med 1997;336:775-785.  Back to cited text no. 3    
4.Maron BJ. Hypertrophic cardiomyopathy. Lancet 1997; 350:127-133.  Back to cited text no. 4    
5.Tearce D. Asymmetric hypertrophy of the heart in young adults. Br Heart J 1958;20:1-8.  Back to cited text no. 5    
6.Maron BJ. Hypertrophic cardiomyopathy Curr Probl Cardiol 1993; 18:639-704.  Back to cited text no. 6    
7.Maron BJ, Moller JH, Seidman CE, Vincent GM, Dietz HC, Moss AJ, et al. Impact of laboratory molecular diagnosis on contemporary criteria for genetically transmitted cardiovascular disease. Circulation 1998; 98:1460-1471.  Back to cited text no. 7    
8.Van Noorden S, Olsen EG, Pearse AG. Hypertrophic Obstructive Cardiomyopathy: A histological, histochemical and ultra structural study of biopsy material. Cardiovasc Res 1971; 5:118-131.  Back to cited text no. 8    
9.Davies MJ. The current status of myocardial disarray in hypertrophic cardiomyopathy. Br Heart J 1984; 51:361-363.  Back to cited text no. 9    
10.Becker AE, Caruso G. Myocardial disarray. A critical review. Br Heart J 1982; 47:527-538.  Back to cited text no. 10    
11.Davies MJ, Pomerance A, Teare RD. Pathological feature of hypertrophic obstructive cardiomyopathy. J Clin Pathol 1974; 27:529-535.  Back to cited text no. 11    
12.Maron BJ, Roberts WC. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy. Circulation 1979; 59:689-706.  Back to cited text no. 12    
13.Maron BJ, Sato N, Roberts WC, Edwards JE, Chandra RS. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum. Comparison of fetuses and infants with and without congenital heart disease and patients with hypertrophic cardiomyopathy. Circulation 1979; 60:685-696.  Back to cited text no. 13    
14.Louie EK, Edwards LC 3rd. Hypertrophic Cardiomyopathy. Prog Cardiovasc Dis 1994; 36:275-308.   Back to cited text no. 14    




 

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Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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