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A previously healthy 26-year-old man is seen for evaluation of palpitations. He reports \u201cskipped beats\u201d since age 18, with occasional short runs of fast, strong beats that are precipitated by alcohol use and marked exertion. He denies associated symptoms such as syncope, chest pain, and shortness of breath.\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 <\/span><\/p>\n His medical history is unremarkable, and he takes no medications. He is currently a graduate student and an avid long-distance runner, having run several marathons. He denies tobacco and illicit drug use and drinks alcohol socially.<\/p>\n The young man has an extensive family history of cardiac disease related to lamin A\/C deficiency. His father was the index patient, diagnosed as heterozygous for the mutation at age 54, following cardiac resynchronization therapy (CRT) upgrade of an implantable cardioverter-defibrillator (ICD). The father had been diagnosed with atrial fibrillation and high-degree atrioventricular block at age 46, requiring pacemaker placement. He experienced ventricular tachycardia (VT) at age 50, requiring ICD placement with the subsequent CRT upgrade at 54. Four of the father\u2019s six siblings carry the lamin A\/C deficiency: One suffered a sudden cardiac death, one had a heart transplant, two received ICDs, two received pacemakers, and three have undergone catheter ablation for atrial fibrillation. Average onset for cardiac involvement has been age 40.<\/p>\n The young patient\u2019s baseline ECG shows sinus arrhythmia, a PR interval of 204 msec, low-voltage P waves, and right-axis deviation. An ambulatory ECG monitor showed multiple episodes of nonsustained VT. \u00a0<\/span><\/p>\n Genetic analysis reveals lamin A\/C deficiency.<\/p>\n A transthoracic echocardiogram shows low-normal systolic function (LV ejection fraction, 53%) and high-normal LV size. Cardiac MRI shows significantly delayed enhancement in the mid myocardium of the basal to mid septum, and in the basal inferior and inferolateral walls.<\/p>\n Questions:<\/b><\/p>\n 1. How would you manage this patient?<\/p>\n 2. What are your considerations for device therapy (permanent pacemaker, ICD)?<\/p>\n 3. Would you initiate pharmacologic therapy? If so, what drugs?<\/p>\n 4. How would you advise the patient about participating in competitive sports, specifically long-distance running?<\/p>\n