RATIONALE: The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH). OBJECTIVES: We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH. METHODS: All deaths of patients with pulmonary hypertension (PH) followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the PH team. A total of 84 patients with PAH (age 58 ± 14 yr; 73% females) who died between June 2008 and May 2012 were included. MEASUREMENTS AND MAIN RESULTS: PH was determined to be the direct cause of death (right heart failure or sudden death) in 37 (44%) patients; PH contributed to but did not directly cause death in 37 (44%) patients; and the death was not related to PH in the remaining cases (n = 7; 8.3%). In three (3.6%) patients the final cause of death could not be adequately assessed. Most patients died in a healthcare environment and most received PH-specific therapies. In our cohort, 50% of all patients with PAH and 75.7% of those who died of right heart failure received parenteral prostanoid therapy. Less than half of patients had advanced healthcare directives. CONCLUSIONS: Most patients with PAH in our cohort died of their disease; however, right ventricular failure or sudden death was the sole cause of death in less than half of patients.
RATIONALE: The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH). OBJECTIVES: We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH. METHODS: All deaths of patients with pulmonary hypertension (PH) followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the PH team. A total of 84 patients with PAH (age 58 ± 14 yr; 73% females) who died between June 2008 and May 2012 were included. MEASUREMENTS AND MAIN RESULTS: PH was determined to be the direct cause of death (right heart failure or sudden death) in 37 (44%) patients; PH contributed to but did not directly cause death in 37 (44%) patients; and the death was not related to PH in the remaining cases (n = 7; 8.3%). In three (3.6%) patients the final cause of death could not be adequately assessed. Most patients died in a healthcare environment and most received PH-specific therapies. In our cohort, 50% of all patients with PAH and 75.7% of those who died of right heart failure received parenteral prostanoid therapy. Less than half of patients had advanced healthcare directives. CONCLUSIONS: Most patients with PAH in our cohort died of their disease; however, right ventricular failure or sudden death was the sole cause of death in less than half of patients.
Authors: David B Badesch; Hunter C Champion; Miguel Angel Gomez Sanchez; Marius M Hoeper; James E Loyd; Alessandra Manes; Michael McGoon; Robert Naeije; Horst Olschewski; Ronald J Oudiz; Adam Torbicki Journal: J Am Coll Cardiol Date: 2009-06-30 Impact factor: 24.094
Authors: L J Rubin; J Mendoza; M Hood; M McGoon; R Barst; W B Williams; J H Diehl; J Crow; W Long Journal: Ann Intern Med Date: 1990-04-01 Impact factor: 25.391
Authors: Marius M Hoeper; Nazzareno Galié; Srinivas Murali; Horst Olschewski; Melvyn Rubenfire; Ivan M Robbins; Harrison W Farber; Vallerie McLaughlin; Shelley Shapiro; Joanna Pepke-Zaba; Joerg Winkler; Ralf Ewert; Christian Opitz; Volker Westerkamp; Jean-Luc Vachiéry; Adam Torbicki; Juergen Behr; Robyn J Barst Journal: Am J Respir Crit Care Med Date: 2002-02-01 Impact factor: 21.405
Authors: G E D'Alonzo; R J Barst; S M Ayres; E H Bergofsky; B H Brundage; K M Detre; A P Fishman; R M Goldring; B M Groves; J T Kernis Journal: Ann Intern Med Date: 1991-09-01 Impact factor: 25.391
Authors: Vallerie V McLaughlin; Stephen L Archer; David B Badesch; Robyn J Barst; Harrison W Farber; Jonathan R Lindner; Michael A Mathier; Michael D McGoon; Myung H Park; Robert S Rosenson; Lewis J Rubin; Victor F Tapson; John Varga; Robert A Harrington; Jeffrey L Anderson; Eric R Bates; Charles R Bridges; Mark J Eisenberg; Victor A Ferrari; Cindy L Grines; Mark A Hlatky; Alice K Jacobs; Sanjay Kaul; Robert C Lichtenberg; Jonathan R Lindner; David J Moliterno; Debabrata Mukherjee; Gerald M Pohost; Robert S Rosenson; Richard S Schofield; Samuel J Shubrooks; James H Stein; Cynthia M Tracy; Howard H Weitz; Deborah J Wesley Journal: Circulation Date: 2009-03-30 Impact factor: 29.690
Authors: Hubert Chen; Stephen C Shiboski; Jeffrey A Golden; Michael K Gould; Steven R Hays; Charles W Hoopes; Teresa De Marco Journal: Am J Respir Crit Care Med Date: 2009-06-11 Impact factor: 21.405
Authors: Adriano R Tonelli; Mostafa K Ahmed; Laith Alkukhun; Frank Cikach; Kulwant Aulak; Raed A Dweik Journal: Am J Respir Crit Care Med Date: 2015-10-15 Impact factor: 21.405