Objective Restrictive cardiomyopathy (RCM) is among the five major cardio- myopathies. The purpose of this study was to demonstrate the clinical features of RCM patients and evaluate the outcome and prognostic predictors of these patients.

Methods Between 2000 and 2009, 107 patients (48% male, age 55±18 years² mean follow up 35±28 months) which met strict morphological echocardiographic and/or cardiac magnetic resonance imaging (CMRI) criteria for RCM were included. The clinical profile was reviewed in detail, and the follow up data were used to assess risk factors and overall survival of patients.

Results The most common symptom of this study cohort was dyspnoea (82 patients), followed by oedema and palpitation. 73% of patients were in NYHA class III/IV. At follow up, 49 out of 107 patients (46%, 27 male) died, and cardiovascular system–related death accounted for 77% of the mortality (38 patients). There were seven patients (6.5%) underwent heart transplantation. Multivariate Cox regression analysis demonstrated that the risk of all cause mortality was independently associated with age>65 (hazard ratio 2.449) and LVEF<50% (hazard ratio 2.661), but was not related to gender, left atrial diameter >60 mm, atrial fibrillation, fragmented QRS, ventricular tachycardia, or tricuspid regurgitation.

Conclusions RCM is characterised by progressive heart failure, and the overall prognosis is relatively poor. Age>65 and LVEF<50% are predictors of adverse prognosis in RCM.