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Cardiovascular Medicine Sudden Cardiac Death Screening Unexplained premature (age <35 years) death or sudden death in a first-degree family member should raise suspicion for an inherited arrhythmia syndrome. Diagnosis SCD is most often associated with structural heart disease or arrhythmogenic substrate, including HCM, abnormal cardiac rhythms or conduction, dilated cardiomyopathy with reduced systolic function, WPW syndrome, Brugada syndrome, and long QT syndrome. Long QT syndrome may be inherited, but most are acquired. Patients may experience syncope or SCD as the result of ventricular arrhythmia. Look for hypokalemia, hypomagnesemia, structural heart disease, medications, and drug interactions. Look spe- cifically for: o macrolide and fluoroquinolone antibiotics (especially moxifloxacin) o terfenadine and astemizole antihistamines o antipsychotic and antidepressant medications . methadone o antifungal medications o class Ia and class III antiarrhythmics Risk is greatest with a QTc interval >500 ms. Brugada syndrome is an inherited condition characterized by a structurally normal heart but abnormal electrical conduction associated with SCD. Classic Brugada syndrome is recognized as an incomplete RBBB pattern with coved ST segment elevation in leads V, and Vr. Testing Select ECG and echocardiography for survivors of SCD to identiff long Ql anatomic abnormalities, impaired ventricular func- tion, and/or myopathic processes. Electrophysiologic studies are indicated for patients with suspected ventricular arrhythmias, episodes of impaired consciousness, and structurally abnormal hearts. Treatment Therapy includes treatment of underlying CAD and a revascularization procedure if anatomically possible. Inherited long QT syndrome may be treated with p-blockers. Select an ICD in the following scenarios o for survivors of cardiac arrest resulting from VF or VT not explained by a reversible cause . after sustained VT in the presence of structural heart disease . after syncope and sustained WVF on electrophysiologz study o for ischemic and nonischemic cardiomyopathy with an EF <35'2,, NYHA class II or III symptoms, with guideline directed medical therapy . for Brugada syndrome with syncope or ventricular arrhythmia o for inherited long QT syndrome not responding to p-blockers . >40 days after MI with an EF <30% 26

Cardiovascular Medicine TESTYOURSELF A S5-year-old man is evaluated 4 months after a large anterior MI. He has no symptoms, and his physical examination is normal' Follow-up echocardiography documents an LVEF of 28o/". ANSWER: For management, choose an ICD, because this patient is at high risk for sudden cardiac death. Prolonged OI Syndrome: Ihe ECG shows a prolonged 0T interyal of 590 ms. Acute Pericarditis Diagnosis Acute pericarditis is diagnosed clinically by the presence ofat least two offour criteria: o chest pain typical for pericarditis o pericardial friction rub (classically three phases) . newECGchanges . new pericardial effusion Chest pain is sharp, severe, and positional; is worse in the supine position; and improves with sitting up and leaning forward. 27