Lung transplantation for cystic fibrosis: 6-year follow-up

Abstract

Lung transplantation is currently the most effective means of improving survival and quality of life in patients with end-stage cystic fibrosis. In reviewing our 6-year experience we sought to evaluate complications and survival after sequential bilateral lung transplantation. Between October 1996 and October 2002, 114 patients with cystic fibrosis were referred to us from 15 Italian regional centers and 2 support centers for cystic fibrosis as possible candidates for lung transplantation. Of these 114 patients, 99 were included in the waiting list and 15 were refused. The mean time spent on the waiting list was 6.8+/-5.2 months (range 1 day-21 months) for those patients receiving lung transplantation, and 5.4+/-4.5 months (range 10 days-18 months) for those 35 patients who died while on the waiting list. A total 55 patients (6 children and 49 adults), mean age 25.6+/-6.6 years (range 9-52 years), 29 males, underwent bilateral sequential lung transplantation. One patient had a second transplantation 14 months after the first. The most frequent medical non-infective complications after transplantation were chronic renal failure (n=27 patients), diabetes (n=31), osteoporosis (n=17), arterial hypertension (n=14), seizures (n=4), transient cerebral ischaemia (n=1), and transient bilateral blindness (n=1). Bacterial lower airways respiratory infections with the organisms that colonized patients' airways before lung transplantation developed in 42 patients; cytomegalovirus (CMV) infection in 41; and opportunistic infections of the lung with Pneumocystis carinii in 3 patients. Cultures of sputum or bronchoalveolar lavage fluid grew Aspergillus fumigatus in nine patients; aspergillosis of right bronchial anastomosis developed in one patient and a lung infection in another. Another patient had a pulmonary infection secondary to Aspergillus niger. An average of 1.3 episodes of acute rejection developed per patient in the first 6 months after lung transplantation. Freedom from bronchiolitis obliterans syndrome was 95% at 1 year, 82.5% at 2 years, 70% at 3 years, and 65% at 4, 5 and 6 years. Actuarial survival rates were 80% at 1 month, 79% at 1 year, 74% at 2 years, 70% at 3 years and 58% at 4, 5 and 6 years. Ten patients (17.8%) died in the early postoperative period (1-30 days) for the following reasons: primary graft failure (n=4), multiorgan failure (n=3), Burkholderia cepacia sepsis (n=1), myocardial infarction (n=1), and pulmonary embolism (n=1). Mortality was accounted for by 9 patients (16%) who died from 9 to 43 months after lung transplantation, for the following reasons: P. carinii infection (n=2), bronchiolitis obliterans syndrome (n=4), A. fumigatus pulmonary infection (n=1), unknown cause (n=1) and suicide (n=1). In conclusion, the leading causes of morbidity after lung transplantation for cystic fibrosis are pulmonary bacterial infection and opportunistic infections. Bronchiolitis obliterans develops in more than half of lung transplant recipients who survive for more than 3 years and is an important cause of...

Clinical Significance of Microbial Infection and Adaptation in Cystic Fibrosis

“…The Aspergillus-positive and -negative groups were fairly equivalent with regard to age and gender distribution, and no difference in % predicted FEV 1 was observed between them. Furthermore CF patients with respiratory cultures positive for Aspergillus species did not appear to be at an increased risk for complications from lung transplantation (194), although other authors have noted anastomotic dehiscence due to Aspergillus in this setting (249,473). On the other hand, Shoseyov and colleagues reported six CF patients with sputum cultures that grew A. fumigatus and who experienced pulmonary exacerbations (542).…”

Renal impairment in children with cystic fibrosis

“…Indeed, chronic renal failure has been shown to be the most frequent non-infectious medical complication after lung transplantation [12]. Bech et al also showed an early and important decrease in measured glomerular filtration rate in transplanted patients [13].…”

Prospective Multicenter Study of Pneumocystis jirovecii Colonization among Cystic Fibrosis Patients in France

“…Several works support a significant association of P. jirovecii colonization with diverse chronic pulmonary diseases (6,12,21). Few severe PcP cases in cystic fibrosis (CF) patients have been reported (23,25), and only some studies have reported Pneumocystis carriage, the clinical significance of which remains practically unexplored (14,(18)(19)(20)(22)(23)(24)(25)(26). The aims of the present study were therefore to evaluate the prevalence of P. jirovecii colonization during a prospective multicenter observational study, to provide information regarding the distribution of Pneumocystis genotypes, and to explore the clinical relevance of Pneumocystis colonization among CF patients in France.…”