Diagnostic problem: Constrictive pericarditis or restrictive cardiomyopathy?

Abstract

When the patient presents with the signs and symptoms of high venous pressure, a quiet heart and no apparent structural defects, two diagnoses that come to mind are constrictive pericarditis and myocardial disease. The patients that fall into these two groups usually can be separated by a series of diagnostic steps, beginning with the history and extending through noninvasive and invasive evaluations. However, listed among the various classifications of myocardial disease is the one termed restrictive cardiomyopathy [ 11. Whatever the underlying disease process, whether involving the endomyocardium or myocardium, the major pathophysiologic effect is to shift leftward and upward the ventricular passive pressure-volume relationship. Consequently, the ventricular diastolic properties of these patients mimic those of the patient with constrictive pericarditis and so produce the same signs and symptoms [2-51.This commentary was written to accompany an article that categorizes a child as having a restrictive cardiomyopathy based on the signs and symptoms of a high venous pressure, nearly normal pump function, normal ventricular size, a 6-mm Hg end-diastolic pressure gradient between the right and left ventricle and enlarged atria. No pathologic material was obtained. These findings may not be enough to categorize a patient as having a restrictive cardiomyopathy instead of constrictive pericarditis. For example, we have seen a 3-year-old with elevated venous pressure, a 13-mm Hg end-diastolic pressure gradient (33 versus 20 mm Hg) between the left and right ventricle, tricuspid insufficiency, enlarged atria, and a pathologic diagnosis of constrictive pericarditis. The purpose of the following comments is to point out, as has been done previously, the difficulties in separating patients with constrictive pericarditis from those with restrictive cardiomyopathy.

Ventricularization of right atrial wave form in amyloid restrictive cardiomyopathy

“…Evaluation of such patients by invasive [ 1,3,4, and noninvasive techniques [5-101 may not establish the exact diagnosis. Endocardia1 biopsy and/or exploratory thoracotomy could be hazardous but might be the last resort [I].…”

“…Endocardia1 biopsy and/or exploratory thoracotomy could be hazardous but might be the last resort [I]. The significance of such differentiation cannot be overemphasized because of the vast difference in therapeutic approach in these cases [ 1,2].…”

Restrictive cardiomyopathy and constrictive pericarditis: non-invasive distinction by digitised M mode echocardiography.

“…Occasionally, even cardiac catheterisation fails to make the diagnosis. 3 We describe the features of digitised M mode Restrictive cardiomyopathy-Left ventricular angiogram showing predominantly abnormal diastolic function with comparative preservation of systolic function; raised and different left and right ventricular end diastolic pressures in the absence of primary valve or congenital heart disease; left ventricular end diastolic pressure usually more raised than right ventricular end diastolic pressure'";…”