肥厚型心肌病or运动员心脏 课件.pptVIP

Hypertrophic Cardiomyopathy, Athlete’s Heart, or Both A Case of Hypertrophic Cardiomyopathy Regression From Circ Cardiovasc Imaging. 2015;8: Kebed, MD, Mayo Clinic Case An 18-year-old man was referred for evaluation after a markedly abnormal ECG was discovered at a professional hockey scouting event. He was otherwise healthy and asymptomatic He had no family history of cardiomyopathies or sudden unexplained death. ECG UCG A contrast enhanced transthoracic echocardiogram showed apical HCM with a maximal LV wall thickness of 24 mm and spade-like appearance LV chamber size and systolic function were normal without dynamic obstruction. Diastolic function assessment showed indeterminate diastolic function grade MRI the apex and anterior septum measured 25 and 13 mm, First pass perfusion was normal,but postcontrast images demonstrated patchy areas of late gadolinium enhancement suggestive of fibrosis MRI Holter Exercise ECG and 24-hour Holter monitor showed no ventricular arrhythmias genetic testing On genetic testing, a de novo HCM associated mutation in MYH7-encoded β myosin heavy chain gene at position 530 (p. Ile530Val) was identified. The patient’s parents and 2 siblings, all phenotype negative for HCM, tested negative for the mutation. The patient was counseled on the natural history of HCM and the risk of sudden death with competitive athletics. None of the standard sudden death risk markers were present at the initial evaluation, and therefore there was no indication for an implantable cardioverter defibrillator. The patient discontinued his rigorous athletic training, but continued to lead a relatively active lifestyle He was seen for follow-up 14 months after the initial diagnosis. Repeat cardiac MRI showed complete resolution of the LV hypertrophy with maximal wall thickness of 10 mm at the apex However, the patchy areas of late gadolinium enhancement were unchanged On repeat noncontrast echocardiogram, maximum thickness was 12 mm at the apex. The ST