ECMO for Lung Transplantation in Cystic Fibrosis Patients

Study Overview
=================
Brief Summary
-----------------
Cystic fibrosis (CF) is the most common genetically inherited disease in the Caucasian population. Bilateral lung transplantation (LUTX) is a viable option for these patients. Frequently, the surgical operation of LUTX is complicated by hemodynamic instability, intractable hypoxia and respiratory acidosis. For these reasons, Intraoperative extracorporeal life support - ECLS- is required. Data on predictors of use of intraoperative ECLS in CF patients undergoing LUTX is scarce. Aim of this retrospective observational study was 1) to find possible risk factors at the time of enlistment associated with the intraoperative use of ECLS and 2) to compare the outcomes of CF patients treated with ECLS during LUTX or not.

Detailed Description
-----------------
Cystic fibrosis (CF) is the most common genetically inherited disease in the Caucasian population and damages multiple organ systems (i.e., upper and lower respiratory tract, pancreas, liver). Respiratory manifestations include reduction of mucus clearance, chronic pulmonary infections and bronchiectasis, causing progressive respiratory failure that is the primary cause of death in CF patients. Moreover, advanced CF is complicated by pulmonary hypertension, right ventricular hypertrophy and right heart failure. Bilateral lung transplantation (LUTX) is a viable option for these patients, providing a significant survival benefit as compared to no-LuTX. Frequently, the surgical operation of LUTX is complicated by acute heart failure (due to sequential pulmonary artery cross-clamping and/or hemodynamic instability), severe intractable hypoxia and respiratory acidosis. For these reasons, extracorporeal life support - ECLS - (either in the form of cardiopulmonary bypass -CBP- or extracorporeal membrane oxygenation -ECMO) is frequently required. To now, literature data on predictors of use of intraoperative ECLS in CF patients undergoing LUTX is scarce. Notably, the use of ECLS during LUTX has been associated with a higher risk of primary graft dysfunction (PGD). Moreover, knowing that a patient has a high risk for the use of ECLS may allow appropriate clinical planning of the procedure with eventual elective ECMO connection. The investigator's Institution (Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico) is an Italian tertiary referral center for CF and LUTX, as well as for respiratory failure and ECMO support. Aim of this retrospective observational study was 1) to find possible risk factors at the time of enlistment associated with the intraoperative use of ECLS and 2) to compare the outcomes of CF patients treated with ECLS during LUTX or not.

Official Title
-----------------
Extracorporeal Membrane Oxygenation for Lung Transplantation in Cystic Fibrosis Patients: Predictors and Impact on Outcome

Conditions
-----------------
Cystic Fibrosis

Intervention / Treatment
-----------------
* Procedure: Extracorporeal Life Support


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: CF undergoing LUTX Exclusion Criteria: single lung transplantation re-transplantation missing medical records.
Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| ECLS group<br>Patients with CF undergoing LUTX. Need for intraoperative extracorporeal life support | Procedure: Extracorporeal Life Support<br>* Any extracorporeal blood treatment technique for respiratory and/or cardiac support<br>* Other names: Cardiopulmonary Bypass;|
| Non-ECLS group<br>Patients with CF undergoing LUTX. No need for intraoperative extracorporeal life support | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Intraoperative use of blood components | Intraoperative use of: 1) packed red blood cells used (units); 2) fresh frozen plasma (units); 3) pooled platelets (units) | Jan 2013 Dec 2018 | 
| Length of mechanical ventilation | Length of invasive mechanical ventilation (days) | Jan 2013 Dec 2018 | 
| Length of intensive care unit (ICU) stay | Intensive care unit stay (days) | Jan 2013 Dec 2018 | 
| Length of Hospital stay | Length of hospital stay (days) | Jan 2013 Dec 2018 | 
| Primary graft dysfunction | Primary graft dysfunction at 72 hours from re-perfusion of grafts (classes) | Jan 2013 Dec 2018 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Survival | survival at 31st March 2019. | Jan 2013 Dec 2018 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
cystic fibrosis, lung transplantation, extracorporeal membrane oxygenation