Restrictive Cardiomyopathy or Hypertensive Heart Disease?: A Case Report

Abstract

Cardiomyopathies include a spectrum of intrinsic myocardial diseases without significant inflammation. Out of these, restrictive cardiomyopathy is considered a rare form compared to Hypertrophic and Dilated types. A 67-year-old male with a history of hypertension and diabetes for more than 10 years and a previous echo-cardiographic diagnosis of restrictive cardiomyopathy was admitted with bilateral lower limb swelling, severe dyspnoea and other features of cardiovascular compromise. Despite resuscitative attempts, he died shortly after admission. During the post mortem examination, pink frothy discharge from the mouth and icteric sclerae were noted. Gross pulmonary oedema, gross ascites with 3 liters of straw-colored fluid, nutmeg liver and oedematous kidneys were noted. The spleen was shrunken. Heart was grossly enlarged with a weight of 700 g and increased epicardial fat. Right and Left ventricular thicknesses were 0.8 cm and 2.2 cm respectively showing marked bi-ventricular hypertrophy. All major branches of coronary circulation showed over 60% atherosclerotic narrowing. An organized clot at right auricle was noted with dilated atria. Histological examination revealed pulmonary oedema and congestion, centri-lobular hepatic necrosis, patchy renal tubular necrosis and relatively hypertrophic cardiac myocytes with diffuse interstitial hyaline material. Gross myocyte disarray was not noted specially in sections from the inter-ventricular septum and apex. Special stains did not show evidence of amyloid deposition.  Although the clinical picture might have been compatible with diastolic dysfunction due to a restrictive element restricting the diastolic filling, the primary disease/pathology was confirmed at autopsy as hypertrophy with scarring due to hypertensive heart disease. An infiltrative condition leading to restrictive cardiomyopathy had been excluded. Functional restriction to diastolic filling could be attributed to gross ventricular hypertrophy.