Outcome of Patients with Cystic Fibrosis Awaiting Lung Transplantation

Abstract

Cystic fibrosis is a common indication for lung transplantation. Under the current organ allocation system, donor lungs are distributed to patients based solely on their accrued waiting time, and the death rate on the waiting list has been high. Physiologic parameters have been used to guide the referral, but risk factors for death while awaiting transplantation have not been well defined. This study aimed to identify factors at the time of evaluation that were associated with death on the waiting list. A consecutive cohort of 146 patients with cystic fibrosis who were listed for lung transplantation was retrospectively reviewed. Characteristics of patients who died awaiting transplantation were compared with those of patients who survived until transplantation or the end of the study. Thirty-seven patients died while waiting, 76 underwent transplantation, and 33 were alive and still waiting. Actuarial survival rates for the entire cohort were 81% at 1 yr, 67% at 2 yr, and 59% at 3 yr. Although a multivariate Cox proportional hazards model (chi(2) = 29.6; p < 0.001) identified shorter six-minute walk distance (50 m increments; RR, 0.69; 95% CI, 0.57 to 0.84), higher systolic pulmonary artery pressure (5 mm Hg increments; RR, 1.41; 95% CI, 1.11 to 1.80), and diabetes mellitus (RR, 1.57; 95% CI, 1.06 to 2.32) as significant risk factors for death on the waiting list, these factors and other features overlapped considerably between the group of patients who died waiting and the group who lived until transplantation or the end of the study. The transplant evaluation selects a rather homogeneous cohort of patients for the waiting list. Unless outcome on the waiting list can be reliably predicted, establishing criteria to allocate donor lungs according to medical urgency may not be feasible.

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

“…Pese a estos resultados esperanzadores, entre un 19% y un 41% de los pacientes con FQ fallecen en la lista de espera de TP. [56][57][58] Se ha descrito en la literatura que la calidad del injerto pulmonar tiene un papel importante en la supervivencia posterior al TP (donantes con criterios extendidos en comparación con donantes considerados estándar, la utilización de ventilación protectora y la preservación pulmonar). [59][60][61][62][63] La implementación de la perfusión retrógrada ha demostrado mejorar la calidad del injerto al mantener la baja temperatura y eliminar los detritos o material embolico que se puedan haber formado durante la extracción.…”

“…53,54,56,184,185 Aunque algunas de las publicaciones son en base a series de números reducidos de casos. En nuestro país solo se han encontrado dos publicaciones sobre TP en pacientes con FQ, una de ellas en las que se engloba a los pacientes con FQ dentro de las enfermedades supurativas y otra en la que se sugiere que la CEC, entre otras causas, disminuye la supervivencia de los pacientes pediátricos con FQ que reciben un TP.…”

“…186 El estudio publicado por Vizza y cols, el 6WT es considerado un factor de riesgo independiente de mortalidad previo al TP, siendo de 343 metros de media los pacientes que fallecieron mientras esperaban un TP. 56 Dadas las características de la función respiratoria, los pacientes incluidos en nuestro estudio cumplían los criterios establecidos en los consensos nacionales para el TP. 45 Aunque existen publicaciones en las cuales la decisión para incluir a los pacientes FQ en listas de espera de TP no se base exclusivamente en factores espirométricos como el FEV 1 menor del 30 %, sino también, en otras consideraciones como la necesidad de oxígeno, el deterioro físico, la necesidad de antibióticos EV y la calidad de vida.…”

Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?

“…FEV1 is indirectly related to structural lung damage and has been shown to correlate with survival [11,[78][79][80][81][82][83][84][85][86][87][88]. From these studies we learned that CT scores are more sensitive than PFTs in detecting and monitoring onset and progression of CF lung disease [51][52].…”

Indications, patient selection and timing of referral for lung transplantation

“…To overcome these limitations that preclude generalisation to the entire CF population, several investigators have developed larger inclusive models using multivariate Cox regression analysis to predict survival in adults and children with CF [57,[68][69][70][71][72]. The models included a variety of anthropometric, physiological, laboratory and clinical characteristics (e.g.…”