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      The Pulmonary Hypertension Story

      , MD, MACC , 1 , , DO, MPH 1

      Cardiovascular Innovations and Applications

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          Causes and circumstances of death in pulmonary arterial hypertension.

          The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH). We sought to determine the specific reasons and characteristics surrounding the death of patients with PAH. 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. 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. 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.
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            The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries

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              Author and article information

              Journal
              CVIA
              Cardiovascular Innovations and Applications
              CVIA
              Compuscript (Ireland )
              2009-8782
              2009-8618
              May 2018
              May 2018
              : 3
              : 1
              : 9-11
              Affiliations
              1University of Florida Medical School, Gainesville, FL, USA
              Author notes
              Correspondence: C. Richard Conti, MD, MACC, University of Florida Medical School, Gainesville, FL, USA, E-mail: conticr@ 123456medicine.ufl.edu
              Article
              cvia20170031
              10.15212/CVIA.2017.0031
              Copyright © 2018 Cardiovascular Innovations and Applications

              This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 Unported License (CC BY-NC 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See https://creativecommons.org/licenses/by-nc/4.0/.

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