Differentiation of Constriction and Restriction

Abstract

Differentiation of constrictive pericarditis (CP) from restrictive cardiomyopathy (RCM) is a complex and often challenging process. Because CP is a potentially curable cause of heart failure and therapeutic options for RCM are limited, distinction of these 2 conditions is critical. Although different in regard to etiology, prognosis, and treatment, CP and RCM share a common clinical presentation of predominantly right-sided heart failure, in the absence of significant left ventricular systolic dysfunction or valve disease, due to impaired ventricular diastolic filling. Fundamental to the diagnosis of either condition is a clear understanding of the underlying hemodynamic principles and pathophysiology. We present a contemporary review of the pathophysiology, hemodynamics, diagnostic assessment, and therapeutic approach to patients presenting with CP and RCM.

Hemodynamics of constrictive pericarditis and restrictive cardiomyopathy

“…Thus, the inspiratory decrease in LV filling allows a small relative increase in tricuspid inflow and RV filling as phasic septal shifts right‐to‐left. These findings are evident in Doppler flow velocities, as indicated by >25% expiratory increase in mitral E velocity and expiratory decrease in hepatic vein diastolic flow velocity and >25% increase in diastolic flow reversals compared with inspiratory velocity (Figure ) These disparate effects on ventricular filling lead to discordant respiratory changes in ventricular systolic pressures (Figure ), with inspiration inducing an increase in RV but decrease in LV systolic pressure termed “ventricular discordance,” a reliable indicator of CP which helps discriminate from RCM . As expected, the opposite changes occur during expiration.…”

“…The hemodynamic pathophysiology underlying ventricular discordance is complex but can be simplified as follows: pericardial constraint alters chamber compliance and limits total cardiac volume; increased pericardial resistance couples the two ventricles, increasing their interdependence and magnifying septal shifting wherein a change in filling on one side of the heart exerts the opposite effect on the contralateral ventricle; the constricted pericardium isolates the heart from the lungs and therefore intrathoracic pressure (ITP) oscillations are not fully transmitted to the cardiac chambers, resulting in dissociation of respiratory effects on intrathoracic and intracardiac flows and pressures …”

“…M‐mode of the ventricular septum demonstrates respirophasic septal shift (downward translation of the septum with inspiration, upward translation with expiration) and septal shudder (circle, with enlarged view in upper right corner) in a patient with constrictive pericarditis (CP) [Color figure can be viewed at wileyonlinelibrary.com]…”

CMR in Pericardial Diseases - an Update

“…Diagnosis of CP is challenging. Historically, cardiac catheterization has been the gold standard approach demonstrating equalization of diastolic pressures of the right and left ventricles, square root sign, rapid x, and y descents of the atrial pressure curves and ventricular interdependence using the left ventricular and right ventricular pressures [37]. CMR is the only non-invasive image technique with a similar diagnostic accuracy as cardiac catheterization [38].…”

Precise Medicine of Restrictive Cardiomyopathy

“…Among 1,226 patients with familial HCM (688 families), 1.5% had phenotypes diagnostic of RCM [1] . Consequently, the variety of etiopathogenesis and the difficulty of diagnosis lead to poor prognosis of patients with RCM [2][3][4] .…”