RT Book, Section A1 Brahmbhatt, Darshan H. A1 Tweed, Katharine A1 Williams, Lynne K. A2 Baliga, R. R. A2 Abraham, William T. SR Print(0) ID 1161015944 T1 Hypertrophic Cardiomyopathy T2 Color Atlas and Synopsis of Heart Failure YR 2018 FD 2018 PB McGraw-Hill Education PP New York, NY SN 9780071749381 LK accesscardiology.mhmedical.com/content.aspx?aid=1161015944 RD 2024/03/28 AB A 17-year-old adolescent boy presented to the general cardiology clinic for family screening after a maternal aunt had been diagnosed with hypertrophic cardiomyopathy (HCM). He had no history of chest pain, breathlessness, or syncope. He reported mild symptoms of postural hypotension. Examination revealed normal first and second heart sounds with an audible and palpable fourth heart sound, but no murmurs. His electrocardiogram (ECG) demonstrated left ventricular hypertrophy (LVH) with repolarization abnormalities, and echocardiographic and cardiac magnetic resonance imaging (MRI) features of severe asymmetric LVH are noted in this patient (Figure 10-1). He had no evidence of arrhythmia on ambulatory Holter monitoring, but exercise testing demonstrated an abnormal blood pressure response on exercise. Familial genetic testing was performed and identified a pathogenic mutation in the sarcomere gene MYL3. Given his risk factor profile for sudden cardiac death (SCD), an automatic implantable cardioverter defibrillator (AICD) was implanted for primary prevention.