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The Role of Cardiac Catheterization for Patient with Restrictive Cardiomyopathy and Severe Mitral Insufficiency

Author : I. Y. Suhartono, F. Basalamah
Upload Date : 19-04-2018

Background: Restrictive cardiomyopathy (RCM) is a myocardial disorder characterized by restrictive filling, normal or reduced left ventricular (LV) and right ventricular (RV) volume, and normal or nearly normal systolic function. Its clinical appearance resembles constrictive pericarditis (CP). However, the pathophysiology between them is quite different, as well as the work up and treatment. Echocardiographic assessment remains the “front line” for diagnosis in most cases. Nevertheless, cardiac catheterization has role to confirm diagnosis if echocardiogram is uncertain.

Case Report: This case report presents a 25-year-old female with shortness of breath and palpitation since a few months before. Electrocardiogram showed atrial fibrillation with non-specific ST-T changes. Fourth grade holosystolic murmur was heard at apex. Echocardiographic assessment showed nondilated, nonhypertrophied and normally contracting ventricles. Atria were severely dilated. Doppler echocardiography showed restrictive type diastolic dysfunction. Giant LA and severe mitral and tricuspid regurgitation made it difficult to distinguish RCM from CP. Cardiac catheterization could differentiate between RCM and CP using hemodynamic profile. Criteria favoured restrictive cardiomyopathy are 1) no end-diastolic pressure equalization, 2) higher pulmonary artery pressure, 3) right ventricular end-diastolic pressure (RVEDP) equal to or less than one third of the right ventricular systolic pressure (RVSP), 4) LV rapid filling wave equal to or less than 7 mmHg and 5) Kussmaul’s sign.

Conclusion: RCM an CP have similar clinical features. Despite echocardiographic assessment could distinguish them in most cases but there are some conditions that obscure the result. Cardiac catheterization could contribute in confirming the diagnosis.

KEYWORDS : restrictive cardiomyopathy, constrictive pericarditis, echocardiographic assessment, cardiac catheterization


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