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Lab/ECG/Xray Rounds

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:

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Lab/ECG/Xray Rounds

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  1. Lab/ECG/Xray Rounds The EM Resident Provisionally Known as Sean Caine CCFP-EM March 5, 2008

  2. Case 29 yo male presents with severe lightheadedness and palpitations during sexual activity Symptoms persisted x 10-15 min Spontaneous resolution

  3. 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

  4. Case Normal vitals and physical exam ECG is obtained….

  5. Hypertrophic Cardiomyopathy (HCM)

  6. Objectives • Background • Etiology • Pathophysiology • Clinical Presentation • ECG Findings • Treatment

  7. Thank you… Adam Davidson and Jeff Booker

  8. 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

  9. 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

  10. 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

  11. 15% 15% 35%

  12. 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

  13. Asymmetric septal hypertrophy (ASH) Systolic motion of MV anteriorleaflet (SAM) LVOT gradient Mitral regurgitation (MR) Schematic

  14. 2 Chamber Echo Systole Diastole

  15. Morphologic Variants of HCM

  16. Clinical Presentation • Can be completely asymptomatic • Presenting Symptoms • Dyspnea • Exercise intolerance • Dizziness • Chest pain • Presyncope/syncope • Sudden Cardiac Death

  17. Clinical Presentation • Average age at diagnosis is 30-40 yrs • 2% diagnosed before the age of 5 • 7% before the age of 10

  18. Key features on history Previous hx of syncope Family history of SCD or early onset of symptoms

  19. 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

  20. 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%

  21. 30 yo male with 20 min of lightheadedness running to bus

  22. 31 yo female syncope preceded by palpitations during physical activity

  23. ????? ?????

  24. Diagnosis • Ultimately made by doppler Echo

  25. Complications • Increased incidence of WPW • Chronic or paroxysmal afib (10-40%) • Moderate risk for infective endocarditis • SCD

  26. 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)

  27. Nonpharmacologic therapy • Septal myomectomy • Alcohol septal ablation • ICD

  28. Take Home Points • Be suspicious of unexplained LVH on ECG • Especially in symptomatic patients • Think HCM with narrow qwaves in lateral leads

  29. References • 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.

  30. 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

  31. Distinguishing hypertrophic cardiomyopathy (HCM) from "Athlete's Heart" LV: left ventricle; LA: left atrium; LVH: left ventricular hypertrophy.Adapted from Maron, BJ, Pellicia, A, Spirito, P. Circulation 1995; 91:1596.

  32. 25 yo male severe palpitations, lightheadedness, presyncope playing basketball

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