(Circulation. 2000;101:2490.)
© 2000 American Heart Association, Inc.
Clinical Investigation and Reports |
Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy
From the Division of Cardiovascular Diseases and Internal Medicine (N.M.A., J.B.S., A.J.T.), the Section of Biostatistics (K.R.B.), and the Division of Anatomic Pathology (W.D.E.), Mayo Clinic and Mayo Foundation, Rochester, Minn.
Background—Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function.
Methods and Results—Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for idiopathic restrictive cardiomyopathy, mainly dilated atria with nonhypertrophied, nondilated ventricles. None had known infiltrative disease, hypertension of >5 years’ duration, or cardiac or systemic conditions associated with restrictive filling. Nineteen percent were in NYHA class I, 53% in class II, and 28% in class III or IV. Atrial fibrillation was noted in 74% of patients and systolic dysfunction in 16%. Follow-up (mean, 68 months) was complete for 93 patients (99%). At follow-up, 47 patients (50%) had died, 32 (68%) of cardiovascular causes. Four had heart transplantation. The death rate compared with actuarial statistics was significantly higher than expected (P<0.0001). Kaplan-Meier 5-year survival was 64%, compared with expected survival of 85%. Multivariate analysis using proportional hazards showed that the risk of death approximately doubles with male sex (hazard ratio [HR] =2.1), left atrial dimension >60 mm (HR=2.3), age >70 years (HR=2.0), and each increment of NYHA class (HR=2.0).
Conclusions—Idiopathic restrictive cardiomyopathy or nondilated, nonhypertrophic ventricles with marked biatrial dilatation, as defined morphologically by echocardiography, affects predominantly elderly patients but can occur in any age group. Patients present with systemic and pulmonary venous congestion and atrial fibrillation and have a poor prognosis, particularly men >70 years old with higher NYHA class and left atrial dimension >60 mm.
Key Words: cardiomyopathy • echocardiography • prognosis • survival
This article has been cited by other articles:
 |
|
 |
|
  P. Nihoyannopoulos and D. Dawson Restrictive cardiomyopathies Eur J Echocardiogr, December 1, 2009; 10(8): iii23 - iii33. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Rossi, P. L. Temporelli, M. Quintana, F. L. Dini, S. Ghio, G. S. Hillis, A. L. Klein, N. Ajmone Marsan, D. L. Prior, C. M. Yu, et al. Independent relationship of left atrial size and mortality in patients with heart failure: an individual patient meta-analysis of longitudinal data (MeRGE Heart Failure) Eur J Heart Fail, October 1, 2009; 11(10): 929 - 936. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 I. M. Robbins, J. H. Newman, R. F. Johnson, A. R. Hemnes, R. D. Fremont, R. N. Piana, D. X. Zhao, and D. W. Byrne Association of the Metabolic Syndrome With Pulmonary Venous Hypertension Chest, July 1, 2009; 136(1): 31 - 36. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J P Kaski, P Syrris, M Burch, M-T Tome-Esteban, M Fenton, M Christiansen, P S Andersen, N Sebire, M Ashworth, J E Deanfield, et al. Idiopathic restrictive cardiomyopathy in children is caused by mutations in cardiac sarcomere protein genes Heart, November 1, 2008; 94(11): 1478 - 1484. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Rouf, S. Greytak, E. C. Wooten, J. Wu, J. Boltax, M. Picard, E. C. Svensson, W. H. Dillmann, R. D. Patten, and G. S. Huggins Increased FOG-2 in Failing Myocardium Disrupts Thyroid Hormone-Dependent SERCA2 Gene Transcription Circ. Res., August 29, 2008; 103(5): 493 - 501. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. R. Pinto, M. S. Parvatiyar, M. A. Jones, J. Liang, and J. D. Potter A Troponin T Mutation That Causes Infantile Restrictive Cardiomyopathy Increases Ca2+ Sensitivity of Force Development and Impairs the Inhibitory Properties of Troponin J. Biol. Chem., January 25, 2008; 283(4): 2156 - 2166. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Kubo, J. R. Gimeno, A. Bahl, U. Steffensen, M. Steffensen, E. Osman, R. Thaman, J. Mogensen, P. M. Elliott, Y. Doi, et al. Prevalence, Clinical Significance, and Genetic Basis of Hypertrophic Cardiomyopathy With Restrictive Phenotype J. Am. Coll. Cardiol., June 26, 2007; 49(25): 2419 - 2426. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. I. vanden Driesen, R. E. Slaughter, and W. E. Strugnell MR findings in cardiac amyloidosis. Am. J. Roentgenol., June 1, 2006; 186(6): 1682 - 1685. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. V. Gomes, J. Liang, and J. D. Potter Mutations in Human Cardiac Troponin I That Are Associated with Restrictive Cardiomyopathy Affect Basal ATPase Activity and the Calcium Sensitivity of Force Development J. Biol. Chem., September 2, 2005; 280(35): 30909 - 30915. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L M Russo and S A Webber Idiopathic restrictive cardiomyopathy in children Heart, September 1, 2005; 91(9): 1199 - 1202. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Seth, D. Thatai, S. Sharma, P. Chopra, and K.K. Talwar Clinico-pathological evaluation of restrictive cardiomyopathy (endomyocardial fibrosis and idiopathic restrictive cardiomyopathy) in India Eur J Heart Fail, October 1, 2004; 6(6): 723 - 729. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Rossi, M. Cicoira, L. Zanolla, R. Sandrini, G. Golia, P. Zardini, and M. Enriquez-Sarano Determinants and prognostic value of left atrial volume in patients with dilated cardiomyopathy J. Am. Coll. Cardiol., October 16, 2002; 40(8): 1425 - 1430. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Agmon, B. K. Khandheria, F. Gentile, and J. B. Seward Clinical and Echocardiographic Characteristics of Patients With Left Atrial Thrombus and Sinus Rhythm: Experience in 20 643 Consecutive Transesophageal Echocardiographic Examinations Circulation, January 1, 2002; 105(1): 27 - 31. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R A Nishimura Constrictive pericarditis in the modern era: a diagnostic dilemma Heart, December 1, 2001; 86(6): 619 - 623. [Full Text] [PDF]
|
|
|
|
|
|
E W. Hancock CARDIOMYOPATHY: Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis Heart, September 1, 2001; 86(3): 343 - 349. [Full Text] [PDF]
|
|
|
|
|
|
D. P. Schutte, M. R. Essop, N. M. Ammash, A. J. Tajik, J. B. Seward, W. D. Edwards, and K. R. Bailey Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy Response Circulation, April 10, 2001; 103 (14): e83 - e83. [Full Text] [PDF]
|
|
|
|
 |
|
 Idiopathic Restrictive Cardiomyopathy -- Rare But Deadly Journal Watch Cardiology, August 18, 2000; 2000(818): 2 - 2. [Full Text]
|
|