PMID- 1863023
OWN - NLM
STAT- MEDLINE
DCOM- 19910830
LR  - 20220409
IS  - 0003-4819 (Print)
IS  - 0003-4819 (Linking)
VI  - 115
IP  - 5
DP  - 1991 Sep 1
TI  - Survival in patients with primary pulmonary hypertension. Results from a national 
      prospective registry.
PG  - 343-9
AB  - 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.
FAU - D'Alonzo, G E
AU  - D'Alonzo GE
AD  - Division of Lung Diseases, National Heart, Lung, and Blood Institute, Bethesda, 
      MD 20892.
FAU - Barst, R J
AU  - Barst RJ
FAU - Ayres, S M
AU  - Ayres SM
FAU - Bergofsky, E H
AU  - Bergofsky EH
FAU - Brundage, B H
AU  - Brundage BH
FAU - Detre, K M
AU  - Detre KM
FAU - Fishman, A P
AU  - Fishman AP
FAU - Goldring, R M
AU  - Goldring RM
FAU - Groves, B M
AU  - Groves BM
FAU - Kernis, J T
AU  - Kernis JT
AU  - et al.
LA  - eng
GR  - N01-HR-14000/HR/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Ann Intern Med
JT  - Annals of internal medicine
JID - 0372351
SB  - IM
MH  - Adult
MH  - Analysis of Variance
MH  - Female
MH  - Follow-Up Studies
MH  - Hemodynamics
MH  - Humans
MH  - Hypertension, Pulmonary/drug therapy/*mortality/physiopathology
MH  - Life Tables
MH  - Male
MH  - Middle Aged
MH  - Multivariate Analysis
MH  - Odds Ratio
MH  - Prospective Studies
MH  - Registries
MH  - Respiratory Function Tests
MH  - Survival Rate
MH  - United States/epidemiology
EDAT- 1991/09/01 00:00
MHDA- 1991/09/01 00:01
CRDT- 1991/09/01 00:00
PHST- 1991/09/01 00:00 [pubmed]
PHST- 1991/09/01 00:01 [medline]
PHST- 1991/09/01 00:00 [entrez]
AID - 10.7326/0003-4819-115-5-343 [doi]
PST - ppublish
SO  - Ann Intern Med. 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343.