Clinical Characteristics, Inpatient Management and Variables Associated with Post-Discharge Mortality in Pulmonary Arterial Hypertension; Patients Hospitalized with Right Heart Failure

Background Pulmonary arterial hypertension (PAH) may be associated with acute and chronic right ventricular (RV) failure and confers adverse outcomes in PAH. Given a paucity of data in understanding the management of RV failure in PAH, we sought to evaluate the characteristics of PAH patients admitted with RV failure, inpatient management strategies and the determinants of post-hospitalization mortality. Methods We conducted a retrospective analysis of 41 patients with an established diagnosis of PAH, admitted for management of right heart failure or cardiogenic shock. Demographics, clinical characteristics, echocardiograms, right heart catheterization, medications use and outcome data including length of stay (LOS) and mortality were evaluated. Results Our cohort had a median age of 63 years and 90% were female. Hypertension (65.6%), dyslipidemia (32%), connective tissue disease (34%), and tricuspid regurgitation (71%) were common in this cohort. 17% were admitted directly to the intensive care unit (ICU), 83% to a non-ICU setting with 9% requiring escalation of care to the ICU. Inpatient management included the use of escalating diuretic dosing (80.5%), endothelin receptor antagonists (ERA) (48.8%), phosphodiesterase inhibitors (PDE-I) (61.0%), intravenous (IV) treprostinil (36.6%), spironolactone (41.5%) and digoxin (46.3%) and inhaled nitric oxide (INO) (7.3%). Overall LOS and ICU LOS were a mean of 6 and 3.5 days respectively. Evaluation of medications revealed a 50% increase in the proportion of patients discharged on ERAs, a 21% increase in the use of PDE-I and a 71% increase in IV treprostinil use. Inpatient mortality was 0 but post-discharge mortality was 19.5% over a median follow up period of 743 days. On Cox proportional hazard analysis, age [HR(CI): 1.1 (1.02-1.2), p=0.02], serum creatinine [HR(CI): 1.7 (1.1-2.7), p=0.02] and INO [HR(CI): 39.5 (2.5-631.5), p=0.009] were found to predict post-discharge mortality in all patients. Additionally, higher TAPSE was associated with lower mortality in patients requiring only non-ICU care [HR(CI): 0.9 (0.8-0.97), p=0.01]. Conclusion RV failure treatment often involves simultaneous treatment strategies aimed at improving RV preload, afterload and contractility. Understanding patterns of contemporary management of PAH patients admitted with RV failure and the factors associated with post-hospitalization mortality is important to provide better and individualized care to these patients.