Mixed constrictive pericarditis and restrictive cardiomyopathy in a 36-year-old female

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Date: May 31, 2012
From: Journal of Pakistan Medical Association(Vol. 62, Issue 5)
Publisher: Knowledge Bylanes
Document Type: Report
Length: 1,516 words
Lexile Measure: 1450L

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Byline: Naveed Akhtar, Ayesha Khalid, Sabeen Razaque, Waqas Ahmed, Habib-ur-Rahman and Munir Ahmed

Abstract

Mixed lesion of Restrictive Cardiomyopathy and Constrictive Pericarditis is a rarely reported clinical entity which poses a diagnostic and therapeutic enigma to physicians. The management of both conditions differs markedly. Restrictive Cardiomyopathy is managed either conservatively or cardiac transplant may be offered. On the other hand, Constrictive Pericarditis can be surgically treated by pericardiectomy. We report a rare case of decompensated heart failure presenting with mixed features of both constrictive and restrictive cardiomyopathy.

Introduction

Constrictive Pericarditis (CP) is characterised by scarring and loss of elasticity of the pericardium, resulting in external impedance of normal diastolic cardiac filling.1 Restrictive Cardiomyopathy (RC) is defined as a heart muscle disease that results in impaired ventricular filling, with normal or decreased diastolic volume of either or both ventricles.2 Differentiating between Constrictive Pericarditis and Restrictive Cardiomyopathy is a difficult clinical challenge that requires multiple diagnostic modalities, using haemodynamics, imaging and biopsy studies. Appropriate selection of treatment is of critical value, as both conditions are managed differently. We report a rare case of a patient from adult age group who had mixed features of both Constrictive Pericarditis and Restrictive Cardiomyopathy.

Case Report

A 35-year-old female with no past significant medical history presented with signs of predominant right-sided heart failure. She had a two-month history of gradually increasing shortness of breath and orthopnoea. She had irregular pulse, pedal oedema and raised JVP. EKG revealed atrial fibrillation, while chest X-ray showed an increased cardiac shadow with increased bronchovascular markings in both lung fields with minimal right basal pleural effusion. Echocardiography revealed dilated Left Ventricle (LV) with mild systolic dysfunction, EF-45% and preserved myocardial thickness and severe bi-atrial dilatation of left atrium (LA) 52mm and right atrium (RA) 54mm (Figure-1a),mild mitral regurgitation (MR), severe tricuspid regurgitation (TR) with estimated pulmonary artery pressure of around 60mmHg. There were respiratory variations noted at mitral and tricuspid inflow with pulse wave Doppler suggestive of interventricular interdependence. Tissue Doppler Imaging (TDI) revealed a peak Ea velocity of 16cm/s.

These features were suggestive of Constrictive Pericarditis, but, interestingly, pericardium was not thickened on 2D-echo. CT scan of the chest was performed to evaluate pericardial thickening, which was less than 2mm and did not meet the CP criteria. In view...

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Gale Document Number: GALE|A289765894