%0 Journal Article %A Mario Naranjo %A Valentina Mercurio %A Hussein Hassan %A Noura Alturaif %A Alessandra Cuomo %A Umberto Attanasio %A Nermin Diab %A Sarina K. Sahetya %A Monica Mukherjee %A Steven Hsu %A Aparna Balasubramanian %A Catherine E. Simpson %A Rachel Damico %A Todd M. Kolb %A Stephen C. Mathai %A Paul M. Hassoun %T Causes and Outcomes of ICU Hospitalizations in Patients with Pulmonary Arterial Hypertension %D 2022 %R 10.1183/23120541.00002-2022 %J ERJ Open Research %P 00002-2022 %X Rationale Pulmonary 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.Objectives To describe patient characteristics, causes of ICU hospitalisation, and risk factors for ICU and 1-year mortality.Methods Data 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 Results 102 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).Conclusions RHF 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.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: M. Naranjo has nothing to disclose. V. Mercurio has nothing to disclose. H. Hassan has nothing to disclose. N. Alturaif has nothing to disclose. A. Cuomo has nothing to disclose. U. Attanasio has nothing to disclose. N. Diab has nothing to disclose. S.K. Sahetya has nothing to disclose. M. Mukherjee has nothing to disclose. S. Hsu has nothing to disclose. A. Balasubramanian has nothing to disclose. C.E. Simpson has nothing to disclose. R.L. Damico has nothing to disclose. T.M. Kolb has nothing to disclose. S.C. Mathai has received consulting fees from Actelion, Bayer, Acceleron, and United Therapeutics. P.M. Hassoun served on a scientific advisory board for Merck & Co. %U https://openres.ersjournals.com/content/erjor/early/2022/03/17/23120541.00002-2022.full.pdf