Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy

Abstract

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 Ͼ5 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 (PϽ0.0001). 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] ϭ2.1), left atrial dimension Ͼ60 mm (HRϭ2.3), age Ͼ70 years (HRϭ2.0), and each increment of NYHA class (HRϭ2.0). 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 Ͼ70 years old with higher NYHA class and left atrial dimension Ͼ60 mm. (Circulation.

Extracardiac Medical and Neuromuscular Implications in Restrictive Cardiomyopathy

“…Thus, the most com- (39,46) Biopsy None Amyloidosis familial (39,46) Biopsy Liver transplantation (48) Amyloidosis senile (39,46) Biopsy None Churg-Strauss syndrome (18) Endomyocardial biopsy (18) Steroids, cyclophosphamide (18) Cystinosis (26) Cystine levels corneal, slit-lamp investigation (26) Cysteamine (26) Sarcoidosis (46,49) Cardiac magnetic resonance, nuclear medicine (49) Steroids (49) Lymphoma, multiple myeloma (50,51) Endomyocardial biopsy (50) Chemotherapy (50) Gaucher's disease (46) Glucocerebrosidase levels (21) Enzyme-replacement therapy (21) Hemochromatosis (52) Transferrin saturation, serum ferritin (53) Venesection (53) Fabry's disease (25,46) Alpha-galactosidase A levels (21) Enzyme-replacement therapy (21) Endomyocardial fibrosis (19,46,54) Endomyocardial biopsy (54) Surgery (55) Pseudoxanthoma elasticum (56) Endomyocardial biopsy (56) None Hypereosinophilic syndrome (17,19,46,57) Peripheral blood eosinophilia (57) Steroids, imatinib mesylate (17, 58) Carcinoid (46) Urinary 5-HIAA, tissue examination (59) Octreotide analogues, interferon-alpha (59) Noonan's syndrome (8,37) Multiple congenital abnormalities (8, 37) None Pericardial mesothelioma a (60) Pericardiocentesis, endomyocardial biopsy (60) Surgery, chemotherapy (60) Reactive arthritis (61) Blood cultures, serum CRP levels, blood Antibiotics, anti-inflammatory sedimentation rate (61) drugs ( mon symptoms are dyspnea (71%), edema (46%), and palpitations (33%). 4 Clinical history should determine other symptoms or disorders indicative of an underlyin...…”

“…The most common clinical signs are jugular venous distension (52%), systolic murmur (46%), pulmonary rales (18%), ascites (15%), and edema (15%). 4 Signs suggesting an underlying disorder should be noted (Table IV).…”

“…4 Atrioventricular block or a tachybradycardia syndrome requiring pacemaker implantation suggests RCMP due to neuromuscular disorders such as desminopathy, myofibrillar myopathy, distal myopathy with rimmed vacuoles, unspecified myopathy, or chloroquine myopathy. 5,7,9,22,28,29 …”

“…1). 4 Doppler echocardiography shows mild-to-moderate mitral and tricuspid regurgitation in 70-84%. 4 The transmitral Doppler spectrum shows a high E-wave with a shortened deceleration time (< 150 ms) and an E/A ratio of > 2.…”

“…4 Doppler echocardiography shows mild-to-moderate mitral and tricuspid regurgitation in 70-84%. 4 The transmitral Doppler spectrum shows a high E-wave with a shortened deceleration time (< 150 ms) and an E/A ratio of > 2. 4,30 At cardiac catheterization, the right atrial and ventricular end-diastolic pressures, as well as the pulmonary capillary wedge pressure and end-diastolic left ventricular pressure, are increased in 80% of the patients.…”