Abstract
This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery. Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 ± 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts. Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) pati...Continue Reading
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