Zeitschrift für metabolisches Syndrom

Extent: a completely new method of treatment of patients with Marfan syndrome

Abstract

Jan Pirk

Marfan syndrome is the most common genetic disorder of connective tissue. One complication that threatens the lives of patients is progressive dilatation of the ascending aorta with the development of aortic valve regurgitation or the emergence of dissection, often leading to sudden death. Until now, these patients were operated on only after dilatation of the ascending aorta causing hemodynamically significant regurgitation of the aortic valve. The surgery consisted of the replacement of the ascending aorta and aortic valve or valve-sparing procedure. This new method is a preventive operation. The method involves creating a custom made external support of the root and the ascending portion of the aorta. Based on the CT examination, a prosthesis Extent is created. The surgery is performed from the longitudinal median sternotomy without cardiopulmonary bypass. The entire aortic root is dissected to its origin from the left ventricle, Ostia of the coronary arteries are encircled, the prosthesis is pulled underneath and fixed to the root and then sutured longitudinally. It is interesting that the prosthesis was developed and as the world’s first have it sewn on himself (Mr. Tal Golesworthy), 13 years ago in Oxford. Neither he nor the other 100 patients operated in this department with this disease had dilation or dissection throughout the study. It is because the prosthesis grows over time into the aortic wall thereby enforces it while maintaining the elastic properties of the wall. At our institute, we have so far experience with operations of 20 patients, with good results. This operation moves the care of patients with Marfan syndrome to a qualitatively higher level.