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Transcript
Lab/ECG/Xray Rounds The EM Resident Provisionally Known as Sean Caine CCFP-EM March 5, 2008 Case 29 yo male presents with severe lightheadedness and palpitations during sexual activity Symptoms persisted x 10-15 min Spontaneous resolution Case continued Additional hx: 2 prev visits to ED in past month for similar symptoms 1st episode Lightheaded while mowing the lawn D/C home from ED after ECG and normal labs 2nd episode Syncopal watching TV Admitted for observation Had serial cardiac enzymes and cardiac monitoring Discharged home after negative work up Instructed to f/u with GP Case Normal vitals and physical exam ECG is obtained…. Hypertrophic Cardiomyopathy (HCM) Objectives Background Etiology Pathophysiology Clinical Presentation ECG Findings Treatment Thank you… Adam Davidson and Jeff Booker Background aka Idiopathic hypertrophic subaortic stenosis, obstructive cardiomyopathy Notable cause of sudden cardiac death among athletes Accounting for 2-36% SCD among athletes Prevalence of 0.1-0.2% of general population Accounts for up to 60% of unexplained LVH MOST COMMON genetic cardiovascular disorder Background SCD most common for those <35 yrs of age1 – No age is immune from SCD Annual mortality rates as high as 3-6%3 Etiology 30-50% due to familial inheritance1,5 Autosomal dominant inheritance with variable penetration mutations of 1 of 11 possible genes encoding proteins for myocardial sarcomere 15% 15% 35% Pathophysiology Hypertrophied, nondilated left ventricle in the absence of another cause Histology: – Hypertrophied muscle cells – Cellular disorganization in “whorled pattern” – Abnormal fibrous tissue – Scarring mimics healed MI Schematic Asymmetric septal hypertrophy (ASH) Systolic motion of MV anteriorleaflet (SAM) LVOT gradient Mitral regurgitation (MR) 2 Chamber Echo Systole Diastole Morphologic Variants of HCM Clinical Presentation Can be completely asymptomatic Presenting Symptoms – Dyspnea – Exercise intolerance – Dizziness – Chest pain – Presyncope/syncope – Sudden Cardiac Death Clinical Presentation Average age at diagnosis is 30-40 yrs 2% diagnosed before the age of 5 7% before the age of 10 Key features on history Previous hx of syncope Family history of SCD or early onset of symptoms Physical Examination S4 gallop Harsh crescendo-decrescendo midsystolic murmur loudest at apex – LOUDER with valsalva and standing – SOFTENS with lying, squatting, or isometric exercise Bifid arterial pulse,double/triple apical impulse,palpable atrial gallop THE ECG Abnormal in 90% ECG Findings LVH: 30% – and associated ST and T wave changes Deep NARROW Q waves (typically I, aVL, V5, V6) Left atrial enlargement: 25-50% 30 yo male with 20 min of lightheadedness running to bus 31 yo female syncope preceded by palpitations during physical activity ????? ????? Diagnosis Ultimately made by doppler Echo Complications Increased incidence of WPW Chronic or paroxysmal afib (10-40%) Moderate risk for infective endocarditis SCD Pharmacotherapy β blockers are first line – Verapamil or disopyramide prescribed with caution when β blocker poorly tolerated Afterload reducing agents with systolic dysfunction/CHF – ACEI, diuretics, digoxin Amiodarone should be used for ventricular dysrhythmias Avoid nitrates (decrease preload and LVOT) Nonpharmacologic therapy Septal myomectomy Alcohol septal ablation ICD Take Home Points Be suspicious of unexplained LVH on ECG – Especially in symptomatic patients Think HCM with narrow qwaves in lateral leads References 1. 2. 3. 4. 5. Ramaraj R. Hypertrophic Cardiomyopathy: Etiology, Diagnosis, Treatment. Cardiology in Review. 2008; 14(4): 172-179. Dovgalyuk J, Holstege C, Mattu A, Brady WJ. The electrocardiogram in the patient with syncope. American Journal of Emergency Medicine. 2007; 25: 688-701. Kelly BS, Mattu A, Brady WJ. Hypertrophic cardiomyopathy: electrocardiographic manifestations and other important considerations for the emergency physician. American Journal of Emergency Medicine. 2007; 25:72-79. Jouriles NJ. Hypertrophic Cardiomyopathy. Marx: Rosen’s Emergency Medicine: Concepts and Clinical Practice. 6th ed. 2006. Niemann JT. Hypertrophic Cardiomyopathy. Emergency Medicine: A comprehensive study guide. 6th ed. 2004: 379-380. Sudden Cardiac Death Risk factors Sustained VT Recurrent syncope (esp w/exertion) Family hx of 1 or more SCD Extreme LVH (>30mm wall thickness) LVOT gradient >30mmHg Abnormal BP response to exercise Nonsustained VT ambulatory monitor 0-1 RF = 1% annual SCD rate 2 or more= increased risk of SCD and prophylactic ICD should be considered Distinguishing hypertrophic cardiomyopathy (HCM) from "Athlete's Heart" Parameter Findings in HCM Findings in Athlete's Heart LV wall thickness and morphology Can be >16 mm; can be heterogeneous or asymmetric across segments Typically <16 mm, especially in women; symmetric Diastolic LV cavity <45 mm (except in late, dilated phase) >55 mm LA size Enlarged Normal LV diastolic filling pattern Impaired relaxation (E:A ratio <1, prolonged diastolic deceleration time) Normal Response to deconditioning None LV wall thickness decreases EKG findings Very high QRS voltages; Q waves; deep negative T waves Criteria for LVH but without unusual features Family history of HCM Present (except de novo mutations) Absent LV: left ventricle; LA: left atrium; LVH: left ventricular hypertrophy. Adapted from Maron, BJ, Pellicia, A, Spirito, P. Circulation 1995; 91:1596. 25 yo male severe palpitations, lightheadedness, presyncope playing basketball