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Survival in Patients with Primary Pulmonary Hypertension: Results from a National Prospective Registry

Gilbert E. D'Alonzo, DO; Robyn J. Barst, MD; Stephen M. Ayres, MD; Edward H. Bergofsky, MD; Bruce H. Brundage, MD; Katherine M. Detre, MD; DrPH; Alfred P. Fishman, MD; Roberta M. Goldring, MD; Berton M. Groves, MD; Janet T. Kernis, MPH; Paul S. Levy, ScD; Giuseppe G. Pietra, MD; Lynne M. Reid, MD; John T. Reeves, MD; Stuart Rich, MD; Carol E. Vreim, PhD; George W. Williams, PhD; and Margaret Wu, PhD
[+] Article, Author, and Disclosure Information

Grant Support: By contract NO1-HR-14000 from the National Heart, Lung, and Blood Institute, National Institutes of Health.

Requests for Reprints: Carol E. Vreim, PhD, Division of Lung Diseases, National Heart, Lung, and Blood Institute, Westwood Building, Room 6A09, Bethesda, MD 20892.

Current Author Addresses: Dr. D'Alonzo: Pulmonary Division, University of Texas Medical Center, Suite 1274, 6431 Fannin Street, MSMB, Houston, TX 77030.

Dr. Barst: Baby's Hospital South, 3959 Broadway, 102A, New York, NY 10032.

Dr. Ayres: Medical College of Virginia, 401 N. 12th Street, Richmond, VA 23219.

Dr. Bergofsky: Pulmonary Critical Care Division, Health Sciences Center, Room 040, SUNY Stoney Brook, Stoney Brook, NY 11794-8172.

Dr. Brundage: Harbor UCLA Medical Center, 1000 West Carson Street, Torrance, CA 90509.

Dr. Detre: Department of Epidemiology, A531 Crabtree Hall, Graduate School of Public Health, University of Pittsburgh, 130 DeSoto Street, Pittsburgh, PA 15261.

Dr. Fishman: Department of Physical Medicine and Rehabilitation, University of Pennsylvania, 196 John Morgan Hall, 36th and Harrison Walk, Philadelphia, PA 19104.

Dr. Goldring: Department of Medicine, New York University, 550 1st Avenue, New York, NY 10016.

Dr. Groves: University Hospital, Division of Card gy, Campus Box B-130, 4200 East Ninth Avenue, Denver, CO 80262

Dr. Levy and Ms. Kernis: School of Public Health, University of Illinois at Chicago, P. O. Box 6998, Chicago, Illinois 60680.

Dr. Pietra: Department of Pathology and Medicine, Hospital of University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104.

Dr. Reid: Department of Pathology, Children's Hospital Medical Center, 300 Longwood Avenue, Boston, MA 02115.

Dr. Reeves: Cardiovascular Pulmonary Research Laboratory, University of Colorado Health Sciences Center, Box B133, 4200 East Ninth Avenue, Denver, CO 80262.

Dr. Rich: Chief, Section of Cardiology, Department of Medicine, University of Illinois at Chicago, PO Box 6998, Chicago, IL 60680.

Dr. Vreim: Division of Lung Diseases, National Heart, Lung, and Blood Institute, National Institutes of Health, Westwood Building, Room 6A16, 5333 Westbard Avenue, Bethesda, MD 20892.

Dr. Williams: The Cleveland Clinic Foundation, 1 Clinic Center, 9500 Euclid Avenue, Cleveland, OH 44195-5196.

Dr. Wu: Mathematical and Applied Statistics Branch, Division of Heart, Lung, and Blood Institute, National Institutes of Health, Federal Building 2A11, 7550 Wisconsin Avenue, Bethesda, MD 20892.

Ann Intern Med. 1991;115(5):343-349. doi:10.7326/0003-4819-115-5-343
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Objective: To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival.

Design: Registry with prospective follow-up.

Setting: Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute.

Patients: Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988.

Measurements: At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals.

Main Results: The estimated median survival of these patients was 2. 8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration.

Conclusions: Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.





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