PMID- 24074527
OWN - NLM
STAT- MEDLINE
DCOM- 20140930
LR  - 20141120
IS  - 1557-3117 (Electronic)
IS  - 1053-2498 (Linking)
VI  - 32
IP  - 12
DP  - 2013 Dec
TI  - Survival in pulmonary arterial hypertension patients awaiting lung 
      transplantation.
PG  - 1179-86
LID - S1053-2498(13)01412-5 [pii]
LID - 10.1016/j.healun.2013.08.016 [doi]
AB  - BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease with 
      lung transplantation as the only option for those patients refractory to medical 
      therapy. Although several equations have been developed to predict PAH patient 
      survival, it is unclear whether they can predict survival for patients awaiting 
      transplantation. METHODS: Data were analyzed on 827 patients listed since 1991 on 
      the Scientific Registry of Transplant Recipients. Overall survival and survival 
      for patients listed prior to and after January 1, 2006 was estimated using the 
      Kaplan-Meier (K-M) method and compared with predicted survival from the pulmonary 
      hypertension connection (PHC) and lung allocation system (LAS) equations. A new 
      equation using a novel model selection algorithm for correlated covariates and 
      missing data was developed using clinical factors and variables in the LAS score. 
      Model validation statistics were calculated and averaged across 500 bootstrap 
      resamples within each of 5 imputation data sets. K-M with 95% confidence 
      intervals and receiver-operator characteristic (ROC) curves assessed model 
      performance. RESULTS: PHC predicted overall survival but underestimated and 
      overestimated survival for those listed pre- and post-2006, respectively. The 
      best model included baseline 6-minute walk distance (6MWD), invasive cardiac 
      output and resting oxygen requirement (O2). Factors associated with 1-year 
      waitlist survival included: resting O2 amount; invasive hemodynamics; 6MWD; and 
      functional class. The new equation by ROC analysis outperformed the LAS and PHC 
      equations. CONCLUSIONS: Current prediction models overestimate survival for PAH 
      patients listed for transplant in the LAS era. This new survival equation can 
      help guide clinicians caring for PAH patients with progression of disease 
      requiring transplant.
CI  - Copyright (c) 2013 International Society for Heart and Lung Transplantation. 
      Published by Elsevier Inc. All rights reserved.
FAU - Gomberg-Maitland, Mardi
AU  - Gomberg-Maitland M
AD  - Medicine. Electronic address: mgomberg@medicine.bsd.uchicago.edu.
FAU - Glassner-Kolmin, Cherylanne
AU  - Glassner-Kolmin C
FAU - Watson, Sydeaka
AU  - Watson S
FAU - Frantz, Robert
AU  - Frantz R
FAU - Park, Myung
AU  - Park M
FAU - Frost, Adaani
AU  - Frost A
FAU - Benza, Raymond L
AU  - Benza RL
FAU - Torres, Fernando
AU  - Torres F
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130925
PL  - United States
TA  - J Heart Lung Transplant
JT  - The Journal of heart and lung transplantation : the official publication of the 
      International Society for Heart Transplantation
JID - 9102703
SB  - IM
CIN - J Heart Lung Transplant. 2013 Dec;32(12):1165-7. PMID: 24210050
MH  - Cardiac Output/physiology
MH  - Familial Primary Pulmonary Hypertension
MH  - Female
MH  - Hemodynamics/physiology
MH  - Humans
MH  - Hypertension, Pulmonary/*mortality/physiopathology/*surgery
MH  - Kaplan-Meier Estimate
MH  - *Lung Transplantation
MH  - Male
MH  - Models, Statistical
MH  - Registries
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Waiting Lists/*mortality
MH  - Walking/physiology
OTO - NOTNLM
OT  - lung allocation score
OT  - lung transplant
OT  - pulmonary arterial hypertension
OT  - survival prediction model
OT  - waitlist
EDAT- 2013/10/01 06:00
MHDA- 2014/10/01 06:00
CRDT- 2013/10/01 06:00
PHST- 2013/06/13 00:00 [received]
PHST- 2013/08/12 00:00 [revised]
PHST- 2013/08/15 00:00 [accepted]
PHST- 2013/10/01 06:00 [entrez]
PHST- 2013/10/01 06:00 [pubmed]
PHST- 2014/10/01 06:00 [medline]
AID - S1053-2498(13)01412-5 [pii]
AID - 10.1016/j.healun.2013.08.016 [doi]
PST - ppublish
SO  - J Heart Lung Transplant. 2013 Dec;32(12):1179-86. doi: 
      10.1016/j.healun.2013.08.016. Epub 2013 Sep 25.