Abstract | BACKGROUND: 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.
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Authors | Adriano R Tonelli, Sebastian Fernandez-Bussy, Saba Lodhi, Olufemi A Akindipe, Robin D Carrie, Karen Hamilton, Kamal Mubarak, Maher A Baz |
Journal | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
(J Heart Lung Transplant)
Vol. 29
Issue 8
Pg. 865-72
(Aug 2010)
ISSN: 1557-3117 [Electronic] United States |
PMID | 20466565
(Publication Type: Journal Article)
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Copyright | Copyright (c) 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved. |
Topics |
- Adult
- Blood Pressure
(physiology)
- Cardiac Catheterization
- Cystic Fibrosis
(complications, surgery)
- Echocardiography, Doppler
- Female
- Humans
- Hypertension, Pulmonary
(epidemiology, mortality, physiopathology)
- Lung Transplantation
- Male
- Prevalence
- Prognosis
- Retrospective Studies
- Stroke Volume
(physiology)
- Survival Rate
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