title: Lung transplantation for cystic fibrosis: outcome of 101 single-center consecutive patients.
abstract: Bilateral sequential lung transplantation (BSLT) is nowadays considered a valid therapeutic option for patients with end stage cystic fibrosis. We report our experience with 104 BSLTs in 101 patients. The overall survivals at 1, 3, 5, 10 years were 79%, 65%, 58%, and 42%, respectively. Perioperative mortality was 14.8% (n = 15). The leading causes of perioperative mortality were primary graft dysfunction and sepsis. Three patients were retransplanted owing to obliterative bronchiolitis. In 70 cases (69%), patients displayed ≥ 1 additional risk factors: previous lung resections, colonization by Burkholderia cepacia, diabetes, pneumothorax, or noninvasive ventilatory support. The mean preoperative 1-second forced expiratory volume of 0.69 ± 0.2 L (22%) increased to 85% at 1 year after the operation. The mean time on the waiting list was 12 ± 5 months. The 5 patients treated with extracorporeal membrane oxygenation before urgent transplantation were operated after 3, 5, 6, 30, and 3 days respectively. During the procedure, cardiopulmonary bypass was required in 33 patients (32%). Lung transplantation represents a unique opportunity to ameliorate the quality and improve the survival of patients affected by cystic fibrosis. Timing of referral and patient selection remain crucial for success.
authors: University of Rome Sapienza, Rome, Italy. daniele.diso@uniromal.it
authors: D
authors: D
authors: Diso
authors: M
authors: M
authors: Anile
authors: M
authors: M
authors: Patella
authors: Y
authors: Y
authors: Pecoraro
authors: E A
authors: EA
authors: Rendina
authors: C
authors: C
authors: Carillo
authors: E
authors: E
authors: Russo
authors: I
authors: I
authors: Onorati
authors: D
authors: D
authors: Angioletti
authors: F
authors: F
authors: Ruberto
authors: G
authors: G
authors: Mazzesi
authors: A G M
authors: AG
authors: Marullo
authors: G
authors: G
authors: Frati
authors: F
authors: F
authors: Venuta
mesh_terms: D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000601:surgery*; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016040:Lung Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D016896:Treatment Outcome; D014850:Waiting Lists; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents