Myocardial ischemia 10 years after a modified Cabrol procedure in a 42-year-old patient with Marfan syndrome

BACKGROUND: Marfan syndrome, a genetic disorder of the connective tissue, may cause aortic root dilation with aortic insufficiency, aortic dissection and mitral prolapse with mitral insufficiency. We present a case of a late complication of the modified Cabrol procedure that included replacing the ascending aorta with a composite graft. CASE PRESENTATION: In February 2019, a 42-year-old female patient with Marfan syndrome who presented with chest pain was sent to the Emergency Department. She had undergone the modified Cabrol procedure 10 years prior. Upon presenting, laboratory analysis revealed elevated troponin-I levels. Electrocardiogram showed new inverted T waves over lead I, aVL and V4 to V6. Contrast computed tomography (CT) revealed thrombosis in the Dacron graft. Percutaneous coronary angiography was conducted, and a large thrombus in the graft was noted. Thrombolytic therapy and percutaneous coronary intervention were performed, after which the patient had no more symptoms and was discharged without complications. CONCLUSIONS: Aortic root surgery, including the Cabrol or modified Cabrol procedure, is necessary for complicated cases of aortic dilations, such as in patients with Marfan syndrome, even though the Cabrol or modified Cabrol procedure has a high complication rate. Regarding this case, we were surprised by the timing of the myocardial ischemia and the position of the thrombus, which differed from other cases. To better manage such a patient’s condition and to detect the formation of thrombus early, completeness of the graft and possible stenosis of the anastomosis site to avoid preventable myocardial ischemia, we suggest that patients should have regular image follow-up, even years after the Cabrol or modified Cabrol procedure.