This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Benotti, J. R. Articles by Cohn, P. F. Search for Related Content PubMed PubMed Citation Articles by Benotti, J. R. Articles by Cohn, P. F. Pubmed/NCBI databases Medline Plus Health Information Cardiomyopathy

Circulation, Vol 61, 1206-1212, Copyright © 1980 by American Heart Association

ARTICLES

Clinical profile of restrictive cardiomyopathy

JR Benotti, W Grossman and PF Cohn

The characteristic hemodynamic features of restrictive cardiomyopathy (normal or reduced cardiac index, normal ventricular systolic function, and "dip and plateau" early in diastole) are traditionally associated with pathologic evidence of inflammation, infiltration and fibrosis. Prognosis is usually poor. Nine patients with restrictive hemodynamic features were recently identified in our laboratory; six were males, three were females, and ages ranged from 23-57 years (mean 47 years). Only one was asymptomatic. Chest pain, dyspnea on exertion and fatigue were the most common symptoms. Echocardiography revealed various degrees of left ventricular wall thickening, but no significant pericardial effusion, pericardial thickening or calcification. Mean left ventricular end-diastolic pressure was 25 mm Hg, cardiac index 2.8 l/min/m2 and ejection fraction 0.63. Endomyocardial and pericardial biopsies, obtained in two patients, were normal. Follow-up (mean 22 months, range 16-42 months) revealed no cardiac deaths. These findings support the hypothesis that the restrictive hemodynamic profile does not necessarily indicate the presence of a specific pathologic process in the subendocardium or myocardium and that the prognosis is not necessarily ominous. The common pathophysiologic feature for this syndrome appears to be reduced ventricular diastolic compliance, but the etiology in many cases is unclear.

This article has been cited by other articles:

				J. Koyama, P. A. Ray-Sequin, and R. H. Falk Longitudinal Myocardial Function Assessed by Tissue Velocity, Strain, and Strain Rate Tissue Doppler Echocardiography in Patients With AL (Primary) Cardiac Amyloidosis Circulation, May 20, 2003; 107(19): 2446 - 2452. [Abstract] [Full Text] [PDF]

				E. TAKIMOTO, A. YAO, H. TOKO, H. TAKANO, M. SHIMOYAMA, M. SONODA, K. WAKIMOTO, T. TAKAHASHI, H. AKAZAWA, M. MIZUKAMI, et al. Sodium calcium exchanger plays a key role in alteration of cardiac function in response to pressure overload FASEB J, March 1, 2002; 16(3): 373 - 378. [Abstract] [Full Text] [PDF]

				P. Palka, A. Lange, J. E. Donnelly, and P. Nihoyannopoulos Differentiation Between Restrictive Cardiomyopathy and Constrictive Pericarditis by Early Diastolic Doppler Myocardial Velocity Gradient at the Posterior Wall Circulation, August 8, 2000; 102(6): 655 - 662. [Abstract] [Full Text] [PDF]

				N. M. Ammash, J. B. Seward, K. R. Bailey, W. D. Edwards, and A. J. Tajik Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy Circulation, May 30, 2000; 101(21): 2490 - 2496. [Abstract] [Full Text] [PDF]

				T. Akasaka, K. Yoshida, A. Yamamuro, T. Hozumi, T. Takagi, S. Morioka, and J. Yoshikawa Phasic Coronary Flow Characteristics in Patients With Constrictive Pericarditis : Comparison With Restrictive Cardiomyopathy Circulation, September 16, 1997; 96(6): 1874 - 1881. [Abstract] [Full Text]

				S. S. Kushwaha, J. T. Fallon, and V. Fuster Restrictive Cardiomyopathy N. Engl. J. Med., January 23, 1997; 336(4): 267 - 276. [Full Text] [PDF]

				D. G. Hurrell, R. A. Nishimura, S. T. Higano, C. P. Appleton, G. K. Danielson, D. R. Holmes Jr, and A. J. Tajik Value of Dynamic Respiratory Changes in Left and Right Ventricular Pressures for the Diagnosis of Constrictive Pericarditis Circulation, June 1, 1996; 93(11): 2007 - 2013. [Abstract] [Full Text]

				R. S. Vasan, E. J. Benjamin, and D. Levy Congestive Heart Failure With Normal Left Ventricular Systolic Function: Clinical Approaches to the Diagnosis and Treatment of Diastolic Heart Failure Arch Intern Med, January 22, 1996; 156(2): 146 - 157. [Abstract] [PDF]

				I. S. Nash and J. T. Fallon Case 2-1994- A 31-Year-Old Man With A Previous Pericardiectomy for Constrictive Pericarditis and Mitral Regurgitation N. Engl. J. Med., January 13, 1994; 330(2): 126 - 134. [Full Text]

				T. Katayama, K. Iwamoto, S. Ochi, Y. Honda, and K. Shigematsu Restrictive Cardiomyopathy Following Acute Myocarditis A Case Report Angiology, January 1, 1990; 41(1): 76 - 81. [Abstract] [PDF]

				D. H. Miller and J. S. Borer The Cardiomyopathies: A Pathophysiologic Approach to Therapeutic Management Arch Intern Med, November 1, 1983; 143(11): 2157 - 2162. [Abstract] [PDF]

				W. H. Lee and J. Fisher Right Ventricular Diastolic Disorders Arch Intern Med, February 1, 1983; 143(2): 332 - 337. [Abstract] [PDF]