Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension

Abstract

The aim of this study was to examine the causes and outcomes of hospitalisation in patients with pulmonary arterial hypertension (PAH).205 consecutive hospitalisations occurring between 2000 and 2009 in 90 PAH patients were studied. The leading causes for hospitalisation were right heart failure (RHF; 56%), infection (16%) and bleeding disorders (8%). For patients with RHF, in-hospital mortality was 14% overall, 46% for patients receiving inotropes and 48% for those admitted to the intensive care unit. The predictors for in-hospital mortality were the presence of connective tissue disease (CTD) (OR 4.92), systolic blood pressure ,100 mmHg (OR 4.32) and Na f136 mEq?L -1 (OR 4.29). Mortality after discharge was 13, 26 and 35% at 3, 6 and 12 months, respectively. World Health Organization functional class prior to admission, renal dysfunction, Charlson comorbidity index, and the presence of CTD were all predictors of mortality after discharge. Hyponatraemia and low systolic blood pressure upon admission and underlying CTD are the main prognostic factors for in-hospital mortality in patients with PAH admitted for RHF. The shortterm outcomes after discharge are poor and remarkably worse in patients with underlying CTD or renal impairment. Early recognition of these factors may guide decisions regarding more aggressive therapy, including consideration for lung transplantation.KEYWORDS: Connective tissue diseases, heart failure, hospital mortality, right ventricular dysfunction, scleroderma, systemic P ulmonary arterial hypertension (PAH) remains a disease with high morbidity and mortality rates despite recent advances in therapy and overall improved survival. PAH is usually progressive, with right ventricular dysfunction being the leading cause of death. Patients often require hospitalisation during the course of their disease, typically for bouts of right heart failure (RHF). In addition, complications related to treatment itself, including PAH-specific therapy, anticoagulation and long-standing indwelling catheters, constitute other potential causes of hospitalisation.In contrast with left heart failure (LHF), the course and outcomes of acute or decompensated RHF have been seldom described [1,2]. Several features differentiate RHF secondary to severe PAH from LHF and make management of RHF particularly challenging. While aggressive diuresis is usually required in both LHF and RHF, the large increase in transpulmonary gradient due to a fixed resistance in the pulmonary vasculature in PAH complicates the treatment of RHF. In addition, right ventricular-left ventricular interdependence often leads to left ventricular dysfunction with low cardiac output and consequent systemic hypotension in RHF, which may require the use of vasopressor and inotropic agents. These haemodynamic perturbations may be further complicated by decreased myocardial perfusion from compromised coronary flow due to right ventricle overload. Ultimately, these processes can lead to distal organ dysfunction and irreversible haemodynamic col...

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