Lung Transplantation in Cystic Fibrosis Patients: Clinical Presentations and Surgical Considerations

Introduction: Lung transplantation has become a critical option for patients with advanced cystic fibrosis, a genetic disorder that causes the production of thick and sticky mucus, leading to severe respiratory complications and impaired lung function. Cystic fibrosis results in recurrent lung infections and significant structural damage, making lung transplantation necessary when conventional treatments are no longer sufficient to manage disease progression. In this context, the surgical approach involves not only the replacement of compromised lungs but also the management of specific complications and the adaptation of the patient to the new organ. Objective: To evaluate clinical practices and surgical considerations associated with lung transplantation in patients with cystic fibrosis, focusing on clinical presentations and surgical challenges. Methodology: The systematic review was conducted following the PRISMA checklist to ensure accuracy and transparency. Searches were performed in the PubMed, Scielo, and Web of Science databases. The descriptors used were "cystic fibrosis," "lung transplantation," "clinical presentations," "surgical considerations," and "postoperative outcomes." Inclusion criteria were articles published in the last 10 years, studies focused on patients with cystic fibrosis and lung transplantation, and publications describing both clinical practices and surgical outcomes. Exclusion criteria included studies without relevant data on cystic fibrosis lung transplantation, articles addressing other pulmonary diseases, and reviews or meta-analyses without original data. Results: The reviewed studies indicated that lung transplantation for cystic fibrosis patients is associated with significant improvements in lung function and quality of life. However, the complexity of the surgical procedure and the need for intensive postoperative management were highlighted as critical factors. The surgical approach requires meticulous planning to avoid complications such as graft rejection and infections. Detailed preoperative assessment and rigorous follow-up were identified as essential for long-term transplant success. Conclusion: Lung transplantation represents an effective option for patients with cystic fibrosis who have reached the advanced stage of pulmonary disease. Clinical and surgical practices must be carefully adapted to the specific needs of these patients to optimize outcomes and minimize complications. The integration of robust assessment and follow-up strategies has proven fundamental for transplant success and substantial improvement in patient quality of life.