title: Characteristics of patients with pulmonary venoocclusive disease awaiting transplantation.
abstract: BACKGROUND
  Pulmonary venoocclusive disease (PVOD) is an uncommon cause of pulmonary arterial hypertension (PAH). However, unlike PAH, treatment options for PVOD are usually quite limited. The impact of the lung allocation score on access to transplantation for patients with PVOD and the clinical course of these patients have not been well-described.
  
  
  OBJECTIVE
  To examine the association between the diagnosis of PVOD and lung transplantation for patients on the transplant waiting list.
  
  
  METHODS
  Patients with a diagnosis of PVOD and PAH registered on the United Network for Organ Sharing wait list for transplantation from May 4, 2005 to May 3, 2013 were included. Lung transplantation was the primary outcome measure. Multivariable analyses were performed to determine the odds of dying or receiving a lung transplant after listing. Survival was compared using Kaplan-Meier and competing risks methods.
  
  
  RESULTS
  Of 12,251 patients listed for lung transplantation, 49 with PVOD and 647 with PAH were identified. There were no significant differences in the lung allocation score between patients with PVOD and PAH at listing, transplant, or wait list removal for death/too sick for transplant. By 6 months, 22.6% of patients with PVOD had been removed from the wait list due to death, compared with 11.0% of patients with PAH (Chi-square P = 0.03). Patients with PVOD who died or were considered too sick for transplant were removed from the waiting list sooner after listing (22 vs. 105 d, P = 0.08). There was no difference in the proportion of patients with PVOD and PAH transplanted (50.0 vs. 47.6%, P = 0.60).
  
  
  CONCLUSIONS
  In the lung allocation score era, patients with PVOD may be at higher risk for death while on the transplant waiting list. After wait list registration, close monitoring for disease progression is advised.
authors.affiliation: 1 Department of Medicine, and.
authors.forename: Keith M
authors.initials: KM
authors.lastname: Wille
authors.forename: Nirmal S
authors.initials: NS
authors.lastname: Sharma
authors.forename: Tejaswini
authors.initials: T
authors.lastname: Kulkarni
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Lammi
authors.forename: Joseph B
authors.initials: JB
authors.lastname: Barney
authors.forename: S Christopher
authors.initials: SC
authors.lastname: Bellot
authors.forename: Ryan S
authors.initials: RS
authors.lastname: Cantor
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.forename: Enrique
authors.initials: E
authors.lastname: Diaz-Guzman
authors.forename: David C
authors.initials: DC
authors.lastname: McGiffin
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000601:surgery; D016040:Lung Transplantation / Q000706:statistics & numerical data*; D008297:Male; D015999:Multivariate Analysis; D011668:Pulmonary Veno-Occlusive Disease / Q000401:mortality* / Q000601:surgery; D012737:Sex Factors; D014850:Waiting Lists
keywords: pulmonary hypertension; pulmonary venoocclusive disease; transplant; venoocclusive