| Keywords [eng] |
Congenital heart disease (CHD), Fontan surgery, Chylothorax, Plastic Bronchitis (PB), Protein- losing enteropathy (PLE), Dynamic-contrast magnetic resonance lymphangiography (DCMRL), Heart transplantation. |
| Abstract [eng] |
Fontan Surgery is established as a definitive palliative medical care for single-ventricle congenital heart disease. This intervention method involves in transporting systemic venous blood to the pulmonary arteries and avoiding the non-functional ventricle. Fontan surgery was first done by Francis Fontan in 1971. Since its invention, the procedure has undergone many changes and alterations in order to enhance and optimize hemodynamic and reduce Fontan surgery-associated morbidity. Even with the advancement of the procedure, long-term survivors often develop severe lymphatic and venous pressure-related complications such as lymphatic hypertension which leads to chylothorax, plastic bronchitis, and protein-losing enteropathy (PLE), these conditions exacerbate morbidity and have an impact in the rate of mortality. This literature review comprehensively reviews the evolution of Fontan surgery, underlining the advancement in Fontan surgical methods, and understanding the complexity of post-Fontan surgical outcomes, as well as the complications related to lymphatic hypertension in Fontan patients. Furthermore, it analyses the challenges of diagnosing and managing lymphatic complications that arise after Fontan surgery. Detailed analysis of lymphatic system’s anatomy and physiology, post-Fontan surgical complications of lymphatic system, assessment of surgical advancements and exploration of diagnostic modalities and treatment of lymphatic hypertension in Fontan patients are included in the objective of this literature review. Despite its important role in the management of univentricular heart disease, the Fontan procedure requires a multidisciplinary approach to manage its multi-system post-surgical complications. Ongoing advances in diagnostic techniques and treatment methods are important to consider in better outcomes in Fontan patients. Additionally, understanding the interplay between increased systemic venous pressure and impaired lymphatic function will be vital for establishing targeted treatment to reduce the post-surgical complications in Fontan patient. |
