Incidence and outcomes of hospital treated acute myocarditis from 2003 to 2015 in Spain.

BACKGROUND: There are no data on population-based epidemiological changes in acute myocarditis in Europe. Our aim was to evaluate temporal trends in incidence, clinical features and outcomes of hospital treated acute myocarditis (AM) in Spain from 2003 to 2015. METHODS: We conducted a retrospective longitudinal study using information of all hospital discharges of the Spanish National Health System. All episodes with a discharge diagnosis of AM from 1 January 2003 to 31 December 2015 were included. The risk-standardized in-hospital mortality ratio (RSMR) was calculated using a multilevel risk-adjustment model developed by the Medicare and Medicaid Services. Temporal trends for in-hospital mortality were modelled using Poisson regression analysis. RESULTS: A total of 11 147 episodes of AM were analysed, most of them idiopathic (94.7%). The rate of AM discharges increased along the period, from 13 to 30/million inhabitants/year (2003-2015), and this increase was statistically significant when weighted by age and sex (incidence rate ratio, IRR 1.06, 95% CI 1.04-1.08, P = .001). In-hospital crude mortality rate was 3.1%, diminishing significantly along 2003-2015 (IRR 0.95, 95% CI 0.92-0.99, P = .02). RSMR also significantly diminished along the period (IRR 0.95, 95% CI 0.92-0.99, P = .01). Renal failure (OR 7.03, 5.38-9.18, P = .001), liver disease (OR 4.61, 2.59-8.21, P = .001), pneumonia (OR 4.13, 2.75-6.20, P = .001) and heart failure (OR 1.91, 95% CI 1.47-2.47, P = .001) were the strongest independent factors associated with in-hospital mortality. CONCLUSIONS: Acute myocarditis is an uncommon entity, although hospital discharges have increased in Spain along the study period. Most of AM were idiopathic. Adjusted mortality was low and seemed to decrease from 2003 to 2015, suggesting an improvement in AM management.

Thirteen-year trends in hospitalization and outcomes of patients with heart failure in Spain.

BACKGROUND: Heart failure is one of the most pressing current public health concerns. However, in Spain there is a lack of population data. We aimed to examine thirteen-year nationwide trends in heart failure hospitalization, in-hospital mortality and 30-day readmission rates in Spain. METHODS: We conducted a retrospective observational study of patients discharged with the principal diagnosis of heart failure from The National Health System' acute hospitals during 2003-2015. The source of the data was the Minimum Basic Data Set. Temporal trends were modelled using Poisson regression analysis. The risk-standardized in-hospital mortality ratio was calculated using a multilevel risk adjustment logistic regression model. RESULTS: A total of 1 254 830 episodes of heart failure were selected. Throughout 2003-2015, the number of hospital discharges with principal diagnosis of heart failure increased by 61%. Discharge rates weighted by age and sex increased during the period [incidence rate ratio (IRR): 1.03; 95% confidence interval (95% CI): 1.03-1.03; P < .001)], although this increase was motivated by the increase in older age groups (≥75 years old). The crude mortality rate diminished (IRR: 0.99; 95% CI: 0.98-1, P < .001), but 30-day readmission rate increased (IRR: 1.05; 95% CI: 1.04-1.06; P < .001). The risk-standardized in-hospital mortality ratio did not change throughout the study period (IRR: 0.997; 95% CI: 0.992-1; P = .32). CONCLUSIONS: From 2003 to 2015, heart failure admission rates increased significantly in Spain as a consequence of the sustained increase of hospitalization in the population ≥75 years. 30-day readmission rates increased, but the risk-standardized in-hospital mortality ratio did not significantly change for the same period.

Variable Expressivity of a Founder Mutation in the EIF2AK4 Gene in Hereditary Pulmonary Veno-occlusive Disease and Its Impact on Survival.

INTRODUCTION AND OBJECTIVES: Hereditary pulmonary veno-occlusive disease (PVOD) has been associated with biallelic mutations in EIF2AK4 with the recent discovery of a founder mutation in Iberian Romani patients with familial PVOD. The aims of this study were phenotypical characterization and survival analysis of Iberian Romani patients with familial PVOD carrying the founder p.Pro1115Leu mutation in EIF2AK4, according to their tolerance to pulmonary vasodilators (PVD). Familial genetic screening was conducted, as well as assessment of sociocultural determinants with a potential influence on disease course. METHODS: Observational study of Romani patients with familial PVOD included in the Spanish Registry of Pulmonary Arterial Hypertension. Genetic screening of EIF2AK4 was performed in index cases and relatives between November 2011 and July 2016 and histological pulmonary examination was carried out in patients who received a lung transplant or died. The patients were divided into 2 groups depending on their tolerance to PVD, with comparison of baseline characteristics and survival free of death or lung transplant. RESULTS: Eighteen Romani patients were included: 9 index cases and 9 relatives. The biallelic founder mutation in EIF2AK4 was found in all affected cases and 2 unaffected relatives. Family screening showed 34.2% of healthy heterozygotes, high consanguinity, young age at childbirth, and frequent multiparity. Prognosis was bleak, with significant differences depending on tolerance to PVD. CONCLUSIONS: We describe 2 phenotypes of hereditary PVOD depending on tolerance to PVD, with prognostic impact and familial distribution. Consanguinity may have a negative impact on the transmission of PVOD, with familial genetic screening showing high effectiveness.