Mortality patterns among patients with restrictive cardiomyopathy referred for CMR in a tertiary care center in Latin-America

Abstract

Background Restrictive Cardiomyopathies (R-CMP) represent a wide array of conditions characterized by diastolic heart failure with important impairment in left ventricle filling pressures. These entities require different treatments, rendering etiological diagnosis a key component during the evaluation of these patients. Purpose Report the mortality of a select group of patients with R-CMP referred to CMR in a tertiary care center in Latin-America. Methods We analyzed all patients with CMR performed in our institution, including hospitalized and ambulatory patients, between 2016 and 2021. We extracted data of patients diagnosed by CMR with amyloidosis, hypertrophic cardiomyopathy (HCM), and endomyocardial fibrosis (EMF). All-cause mortality was extracted from national census data and analyzed using Kaplan-Meier survival estimates and log-rank test. Results Between June 1st, 2016, and December 31st, 2021, 5000 CMRs were performed at our institution. Among these, 317 (6.3%) patients were diagnosed with R-CMP by CMR as follows: 52 patients (16.4%) had amyloidosis, 250 patients had HCM (78.9%) and 15 patients (4.7%) had EMF. Of these, 40.7% were female (n=129), the median age was 63 yar-old (IQR 51–71) and 64.7% (n=205) were out-patients. At a mean follow-up of 33.3 months, there was a statistically significant difference in mortality between patients with amyloidosis (49,0%) and those with HCM and EMF (7,1% and 13,3%, respectively; P<0.001), as described in Figure 1. Conclusions R-CMP represent a meaningful percentage of patients referred for CMR in our institution with different survival rates. Amyloidosis patients had a significantly higher mortality rate compared to HCM and EMF, at a relatively short follow-up. Increased access to CMR in our region for optimal diagnosis and early treatment may optimize prognosis in these types of patients. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Self funding