A Concise Review of Marfan Syndrome with a Congenital Cardiac Surgery Focus.

Abstract

Marfan syndrome is named after Antoine Marfan, who described a 5-year-old child with congenital elongation of the digits and other skeletal abnormalities in 1896. While Marfan syndrome is a systemic connective tissue disorder predominantly involving the skeletal, cardiovascular, and ocular systems, the cardiovascular system presents the most life-threatening complications. Most cardiovascular pathologies surround the left ventricular outflow tract and aorta, with aortic dissection requiring emergent surgical management to the progression of mitral regurgitation requiring elective surgery. Intensive care management, along with a tailored approach to the surgical management of a patient with Marfan syndrome, is critical to their survival. Current surgical operations for patients include aortic root surgery, valve-sparing root replacements, aortic root replacements with conduits, and mitral valve repairs. Further research is necessary to determine the molecular, endovascular, pharmaceutical, and surgical management of Marfan syndrome. This review attempts to concisely discuss the diagnosis, complications, and medical and intensive care management of Marfan syndrome while further divulging on the surgical management of those with this disease process.