Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension.

Evaluation for right ventricular (RV) dysfunction is an important part of risk assessment in care of patients with pulmonary hypertension (PH) as it is associated with morbidity and mortality. Echocardiography provides a widely available and acceptable method to assess RV function. RV global longitudinal strain (RVGLS), a measure of longitudinal shortening of RV deep muscle fibers obtained by two-dimensional echocardiography, was previously shown to predict short-term mortality in patients with PH. The purpose of the current study was to assess the performance of RVGLS in predicting 1-year outcomes in PH. We retrospectively identified 83 subjects with precapillary PH and then enrolled 50 consecutive prevalent pulmonary arterial hypertension (PAH) subjects into a prospective validation cohort. Death as well as combined morbidity and mortality events at 1 year were assessed as outcomes. In the retrospective cohort, 84% of patients had PAH and the overall 1-year mortality rate was 16%. Less negative RVGLS was marginally better than tricuspid annular plane systolic excursion (TAPSE) as a predictor for death. However, in the prospective cohort, 1-year mortality was only 2%, and RVGLS was not predictive of death or a combined morbidity and mortality outcome. This study supports that RV strain and TAPSE have similar 1-year outcome predictions but highlights that low TAPSE or less negative RV strain measures are often false-positive in a cohort with low baseline mortality risk. While RV failure is considered the final common pathway for disease progression in PAH, echocardiographic measures of RV function may be less informative of risk in serial follow-up of treated PAH patients.

Pulmonary hypertension mortality trends in United States 1999-2019.

PURPOSE: Pulmonary hypertension (PH) is a heterogenous, often progressive disorder leading to right heart failure and death. Previous analyses show stable PH mortality rates from 1980 to 2001 but increasing from 2001 to 2010 especially among women and non-Hispanic (NH) Black. This study seeks to identify recent trends in PH mortality in the United States from 1999 to 2019. METHODS: Mortality rates among individuals more than or equal to 15 years of age were obtained from the Centers for Disease Control and Prevention's (CDC) Wide-Ranging Online Data for Epidemiology Research (WONDER) database. ICD-10 codes were used to identify individuals with PH. RESULTS: Between 1999 and 2019, PH was included as a cause on 429,105 recorded deaths. The average age-adjusted PH mortality rate was 7.9 per 100,000 individuals and increased by 1.9% per year. Higher age-adjusted mortality rates were experienced by females and NH Black persons. The crude mortality rate was 105.4 per 100,000 among those decedents 85 or older. From 1999 to 2019, mortality in PH and left heart disease co-occurrence increased at nearly double the annual rate of the overall PH group. CONCLUSIONS: Despite therapeutic advances for selected PH subgroups, the overall age-adjusted PH mortality rate increased significantly from 1999 to 2019 and previously reported racial disparities have persisted. These findings emphasize the need for additional study to improve outcomes in PH.

Influence of Body Weight and Diabetes Mellitus in Patients With Pulmonary Hypertension.

Pulmonary hypertension (PH) is a complex condition that arises due to pulmonary vascular disease, heart disease, lung disease, chronic thromboembolism, or several rare causes. Regardless of underlying cause, PH increases mortality, yet there are no directed treatments for the most common forms of PH due to left heart or lung disease. Because metabolic factors have been implicated in the pathogenesis of PH, we used a large administrative cohort to assess diabetes and weight, potentially modifiable risk factors, on PH outcome. We analyzed 110,495 veterans diagnosed with PH from January 1, 2003 to September 30, 2015 in the Veterans Health Affairs system. Veterans with PH survived an average of 3.88 [IQR 3.85, 3.92] years after PH diagnosis. Diabetes occurred in 36% and increased risk of death by 31% (95% confidence interval 28% to 33%, multivariate adjusted). Higher body mass index was associated with lower mortality in a J-shaped pattern with highest risk in underweight and normal weight veterans. Improved survival in obesity has been referred to as the obesity paradox in heart failure and other diseases. These data show that lower weight and diabetes are strong risk factors for mortality in PH. Our results underscore the importance of systemic conditions on outcome in PH.