Marfan syndrome is a genetic disorder of connective tissue caused by a mutation in the fibrillin gene. The most characteristic changes caused by the syndrome affect the musculoskeletal system, the organ of vision, the heart, and the blood vessels. Due to the complexity of the disease, patients with Marfan syndrome require multidisciplinary treatment. Prognosis is largely dependent on the degree of cardiovascular involvement. This is the most common cause of premature death among patients affected by the syndrome.
- In recent years, tremendous progress has been made in both the diagnosis and treatment of patients with Marfan syndrome. The David procedure, Yacoub method, or pectus excavatum surgeries were introduced - says prof. Mariusz Kuśmierczyk, the main surgeon. The operation lasted 10 hours. - During the procedure, aortic root reconstruction was performed using the David procedure, with 30 mm and 24 mm dacron prostheses. The aortic root was excised leaving the coronary orifices and valve with its connections. Then the top of the right lung was resected. Finally, after partial resection of the sternum, a 38 cm plate was implanted using the Nuss technique to correct the deformed chest. The Nuss procedure is one of the surgical treatment options for funnel chest deformity. The procedure involves inserting a properly curved metal plate under the sternum. The size and degree of bending of the plate is adjusted by the surgeon, adequately to the anatomical conditions of the patient - explains dr Michał Buczyński.
The operation was performed by a team of specialists from the Department of Cardiac Surgery of UCC MUW and the Department of Cardiac and General Pediatric Surgery consisting of: surgeons - Prof. Mariusz Kuśmierczyk, Dr Michał Buczyński, Prof. Karolina Szymczak, anesthesiologist - Andrzej Trzebuchowski, MD, perfusionist - Michał Lewicki, instrument nurse - Marlena Banasiewicz.
The collaboration between the two Departments opens up new therapeutic opportunities for patients with complex defects that require a multidisciplinary approach.
