BACKGROUND: Limited information is available about the prevalence of pulmonary hypertension diagnosed by right heart catheterization (RHC) in patients with cystic fibrosis being evaluated for lung transplantation. It is unclear whether there are factors that can predict the presence of pulmonary hypertension and whether the presence of pulmonary hypertension influences patient outcomes. METHODS: The study included 57 unique and consecutive adult patients (33 women) with cystic fibrosis who underwent lung transplant evaluation at the University of Florida. RESULTS: The average age at evaluation was 31.8 +/- 10 years. All patients were in New York Heart Association class III. The median (interquartile range) of mean pulmonary artery pressure (PAP) was 26 (24-30) mm Hg. Thirty-six patients (63.2%) had pulmonary hypertension (mean PAP >or= 25 mm Hg) and had a significantly higher degree of hypoxemia and oxygen requirements. Echocardiography evidenced limitations for the diagnosis of pulmonary hypertension. The 5-year mortality rate was similar in patients with or without pulmonary hypertension; however, it was higher in 7 patients identified by cluster analysis and in patients with a left ventricular ejection fraction < 55%. CONCLUSIONS: More than half of our patients with cystic fibrosis and advanced lung disease have elevation of PAP, usually of mild degree. A lower left ventricular ejection fraction, but not the presence of pulmonary hypertension, was associated with worse outcomes. Copyright (c) 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
BACKGROUND: Limited information is available about the prevalence of pulmonary hypertension diagnosed by right heart catheterization (RHC) in patients with cystic fibrosis being evaluated for lung transplantation. It is unclear whether there are factors that can predict the presence of pulmonary hypertension and whether the presence of pulmonary hypertension influences patient outcomes. METHODS: The study included 57 unique and consecutive adult patients (33 women) with cystic fibrosis who underwent lung transplant evaluation at the University of Florida. RESULTS: The average age at evaluation was 31.8 +/- 10 years. All patients were in New York Heart Association class III. The median (interquartile range) of mean pulmonary artery pressure (PAP) was 26 (24-30) mm Hg. Thirty-six patients (63.2%) had pulmonary hypertension (mean PAP >or= 25 mm Hg) and had a significantly higher degree of hypoxemia and oxygen requirements. Echocardiography evidenced limitations for the diagnosis of pulmonary hypertension. The 5-year mortality rate was similar in patients with or without pulmonary hypertension; however, it was higher in 7 patients identified by cluster analysis and in patients with a left ventricular ejection fraction < 55%. CONCLUSIONS: More than half of our patients with cystic fibrosis and advanced lung disease have elevation of PAP, usually of mild degree. A lower left ventricular ejection fraction, but not the presence of pulmonary hypertension, was associated with worse outcomes. Copyright (c) 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
Authors: Kathleen J Ramos; Patrick J Smith; Edward F McKone; Joseph M Pilewski; Amy Lucy; Sarah E Hempstead; Erin Tallarico; Albert Faro; Daniel B Rosenbluth; Alice L Gray; Jordan M Dunitz Journal: J Cyst Fibros Date: 2019-03-27 Impact factor: 5.482
Authors: Don Hayes; Curt J Daniels; Stephen Kirkby; Benjamin T Kopp; Kerri L Nicholson; Ashley E Nance; Mark L Splaingard Journal: Lung Date: 2014-03-27 Impact factor: 2.584
Authors: Christopher N Scipione; Rebecca E Schewe; Kelly L Koch; Andrew W Shaffer; Amit Iyengar; Keith E Cook Journal: J Thorac Cardiovasc Surg Date: 2013-02-10 Impact factor: 5.209
Authors: Begum Akay; Julie A Foucher; Daniele Camboni; Kelly L Koch; Ayushi Kawatra; Keith E Cook Journal: ASAIO J Date: 2012 Jul-Aug Impact factor: 2.872
Authors: Chi Young Kim; Ji Eun Park; Ah Young Leem; Joo Han Song; Song Yee Kim; Kyung Soo Chung; Eun Young Kim; Ji Ye Jung; Young Ae Kang; Young Sam Kim; Joon Chang; Jin Gu Lee; Hyo Chae Paik; Moo Suk Park Journal: J Thorac Dis Date: 2018-03 Impact factor: 2.895