SESSION TITLE: Topics in ILD and Lung Transplantation
SESSION TYPE: Original Investigation Slide
PRESENTED ON: Wednesday, October 29, 2014 at 02:45 PM - 04:15 PM
PURPOSE: Pulmonary veno-occlusive disease (PVOD) is an uncommon cause of pulmonary arterial hypertension (PAH). However, in contrast to PAH, treatment options for PVOD are usually quite limited, and transplantation has been the only intervention that may prolong life. The impact of the lung allocation score (LAS) on access to transplantation for PVOD patients, and the clinical course of these patients, have not been well-described.
METHODS: Patients with a diagnosis of PVOD and PAH registered on the UNOS waiting list for transplantation from January 1, 2004-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 methods. Proportional hazards regression modeling was used to study the association between diagnosis and time to death on the waiting list, while controlling for confounders.
RESULTS: Of 13,368 patients listed for lung transplantation during the study period, 51 with PVOD and 773 with PAH were identified. PVOD patients were more commonly male (49.0% vs 30.5%, p=0.006) and had a lower mean PA pressure at listing (49.2 vs. 57.7 mmHg, p=0.001). There were no significant differences in LAS between PVOD and PAH patients at listing (33.6 vs. 33.7), transplant (33.9 vs 34.4), or wait list removal for death or too sick for transplant (36.5 vs. 35.7). A diagnosis of PVOD was associated with an increased risk of death while awaiting transplant (HR 1.92, 95%CI 0.96-3.86,p=0.07), and PVOD patients who died or were considered too sick for transplant were removed from the waiting list sooner after listing (21 vs. 120 days, p=0.006). There was no difference in the proportion of PVOD and PAH patients who underwent transplant (49.0% vs 45.0%, p=0.39).
CONCLUSIONS: In the LAS era, a similar proportion of PVOD and PAH patients underwent transplantation. However, PVOD patients may be at higher risk for death or becoming too sick while on the transplant waiting list.
CLINICAL IMPLICATIONS: With limited treatment options, PVOD patients should be referred early for lung transplantation. After wait list registration, close monitoring for disease progression in these patients is advised.
DISCLOSURE: The following authors have nothing to disclose: Tejaswini Kulkarni, Nirmal Sharma, Matthew Lammi, Joseph Barney, Benjamin Wei, Spencer Melby, David McGiffin, Enrique Diaz-Guzman, Keith Wille
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