Causes and outcomes of ICU hospitalisations in patients with pulmonary arterial hypertension

Abstract

RationalePulmonary arterial hypertension (PAH) is a rare disease characterised by limited survival despite remarkable improvements in therapy. The causes, clinical burden, and outcomes of patients admitted to the intensive care unit (ICU) remain poorly characterised.ObjectivesTo describe patient characteristics, causes of ICU hospitalisation, and risk factors for ICU and 1-year mortality.MethodsData from patients enrolled in the Johns Hopkins Pulmonary Hypertension Registry were analyzed for the period between January 2010 to December 2020. Clinical, functional, hemodynamic, and laboratory data were collected.Measurements and Main Results102 adult patients with 155 consecutive ICU hospitalisations were included. The leading causes for admission were right heart failure (RHF, 53.3%), infection (17.4%), and arrhythmia (11.0%). ICU mortality was 27.1%. Mortality risk factors included Na<136 mEq mL−1 (OR: 3.10, 95% CI: 1.41–6.82), elevated proBNP (OR: 1.75, 95% CI: 1.03–2.98), hyperbilirubinemia (OR: 1.40, 95% CI: 1.09–1.80), hyperlactemia (OR: 1.42, 95% CI: 1.05–1.93), and need for vasopressors/inotropes (OR: 5.29, 95% CI: 2.28–12.28), mechanical ventilation (OR: 3.76, 95% CI: 1.63–8.76), and renal replacement therapy (OR: 5.57, 95% CI: 1.25–24.76). Mortality rates at 3, 6 and 12 months were 17.5%, 27.6%, and 39.0%, respectively. Connective tissue disease-associated PAH has lower 1-year survival compared to idiopathic PAH (51.4% versus 79.8%, log-rank test p=0.019).ConclusionsRHF is the most common cause for ICU admission. In-hospital and 1-year mortality remain exceedingly high despite improved ICU care. Recognising specific risk factors on admission can help identifying patients at risk for poor outcomes.

Frequency of acute vasodilator response (AVR) in incident and prevalent patients with pulmonary arterial hypertension: Results from the pulmonary vascular disease phenomics study

“…Survival from diagnosis and from PVDOMICS enrollment is worse for CTD‐PAH compared to IPAH, consistent with multiple previous publications 2,4,8 . The present study reveals that PAH prevalent patients who display AVR response, as defined herein, present with less functional impairment and more preserved RV function (lower RAP, less RV dilation and better TAPSE) and have superior survival, from diagnosis and from PVDOMICS enrollment, compared with nonresponders.…”

Approach to the hospitalized patient with pulmonary arterial hypertension

“…Several groups have reported on in-hospital and postdischarge outcomes, focusing on ICU-admitted patients for RV failure, while other single-center studies have included non-ICU admissions of PAH patients, with clinical details depicted in Table 2[6 ▪ ,7 ▪ ,8 ▪ ,9 ▪ ,10 ▪ ,11 ▪ ,12 ▪ ,13,14]. Taken together, this data demonstrates that RV failure is a frequent cause of hospitalization requiring ICU level of care in 16–18% of patients.…”

Management of Acutely Decompensated Pulmonary Hypertension

“…While systemic hypotension at admission has been identified as a clear predictive factor of mortality in patients managed for acute decompensated PAH, normal blood pressure should not be considered as a nonthreatening condition. 3,6 Indeed, end-organ dysfunction caused by RHF can be induced by both elevation in central venous pressure (CVP) and circulatory insufficiency. 11 The physiological mechanisms leading to renal dysfunction during an episode of acute RHF are likely similar to those observed in type I cardiorenal syndrome.…”

“…Fig.2Intensive care unit (ICU) mortality in acute decompensated pulmonary hypertension [3][4][5][6][19][20][21]. …”