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  1. 1. Hypertrophic cardiomyopathy
  2. 2. Hypertrophic cardiomyopathy .2-.5% of population affected Left ventricular hypertrophy with myocardial fiber disarray Interstitial fibrosis 2
  3. 3. Hypertrophic cardiomyopathy Familial (autosomal dominant) or sporadic (50%) Abnormalities in sarcomere proteins  beta-myosin heavy chain (45%--chromosome 14) , cardiac myosin binding protein-C  D/D genotype for ACE alters phenotype with more hypertrophy 3
  4. 4. Hypertrophic cardiomyopathy Distribution of hypertrophy  Asymmetric septal hypertrophy most common (septal/posterior wall thickness > 1.5)  Type 1: Hypertrophy anterior septum  Type II : Hypertrophy of anterior and inferior septum  Type III: Extensive LVH with sparing of posterior wall  Type IV: Apical hypertrophy 4
  5. 5. Hypertrophic cardiomyopathy: May have restingLVOT obstruction, latent obstruction or non-obstructive Normal systolic function but increased afterload if LVOT obstruction Dynamic outflow tract obstruction created by anterior motion of mitral leaflet during systole obstructing the outflow tract  Venturi forces b/o septal hypertrophy  Anterior papillary muscle displacement and abnormally long anterior mitral leaflet Gradient worse with decreased preload, increased contractility Primarily diastolic dysfunction  Decreased relaxation  Increased stiffness 5
  6. 6. Hypertrophic cardiomyopathy:Symptoms  May be asymptomatic  Found on screening ECG, echocardiogram  Dyspnea: High LV diastolic pressure because of impaired LV relaxation and increased stiffness. High afterload if LVOT obstruction  Angina: Inadequate coronary arterial blood supply for degree of hypertrophy and increased demand  Syncope: Arrhythmias or hypotension secondary to LVOT obstruction  Palpitations—AF is common  Sudden cardiac death: Ventricular fibrillation6
  7. 7. Ischemia in HCM Myocardial bridging Impaired vasodilator reserve Decreased capillary density and increased capillary separation Myocardial fibrosis Small vessel disease/microvascular dysfunction 7
  8. 8. Apical hypertrophic cardiomyopathy Apical segment >/=15 mm Good prognosis (95% survival at 15 years) Often need contrast echo or cardiac MR to make diagnosis
  9. 9. Apical hypertrophic cardiomyopathy 1/3 of patients had complications—atrial fibrillation, myocardial infarction, HF, TIA/stroke, VT/VF May have mid cavitary obstruction and apical aneurysm
  10. 10. Hypertrophic cardiomyopathy: Signs Spike and dome carotid pulse Triple ripple apical impulse Fourth heart sound (increased filling with atrial contraction because of decreased LV relaxation) Systolic ejection murmur of LV outflow tract obstruction  Increases if decreased preload ie increases with standing from squat Murmur of mitral regurgitation 10
  11. 11. Hypertrophic cardiomyopathy:Diagnosis Echocardiogram shows left ventricular hypertrophy in the absence of hypertension or aortic stenosis  LVOT obstruction caused by mitral leaflet SAM  Eccentric lateral jet of MR ECG: Usually abnormal  Left ventricular hypertrophy  Abnormal Q waves Family history/genetic testing Cardiac MR: LV/RV thickness, late gadolinium enhancement 11
  12. 12. Increased wall thickness LVH for other reasons: hypertension, aortic stenosis, athlete’s heart (< 14 mm) Sigmoid septum of the elderly Infiltrative : amyloidosis, hypereosinophilic syndrome Children with HCM  Idiopathic (75%)  Inborn errors of metabolism (Fabry, Danon disease)  Malformation syndromes (Noonan’s)  Neuromuscular disorders (Friedrich’s ataxia) 12
  13. 13. Brockenbrough-Braunwald-Morrow signAfter a PVC, there is a decrease in carotid pulse pressurein patients with HOCM. Increased contractility results inincreased LVOT obstruction. 13
  14. 14. Hypertrophic Cardiomyopathy:Management Medical therapy for symptomatic dynamic LVOT obstruction:  Beta-blockers, verapamil, disopyramide Interventions for symptomatic LVOT obstruction: septal myomectomy or septal alcohol ablation Prevent death from ventricular arrhythmias:  Assess all patients with HCM for SCD risk factors  Syncope, non-sustained VT, family history SCD, exercise induced hypotension, septum > 30mm (class IIA for ICD), gradient > 30 mm Hg, high risk mutations 14  Defibrillator if VF or sustained VT (class 1)
  15. 15. Septal myectomy 3-15 gm of septal muscle removed Indicated in severely symptomatic patients with resting LVOT obstruction despite medical therapy Dramatic improvement of symptoms Probable decrease in SCD rates Complications  VSD  LBBB/CHB (5-10%)  Aortic regurgitation 15
  16. 16. Ethanol ablation: Complications CHB 10-25% RBBB Ventricular arrhythmias Coronary dissection Pericardial effusion Large MI Best suited for older patients with comorbidities
  17. 17. Management of asymptomaticpatient with HCM Screen first degree relatives with echo Assess for SCD risk with Holter and stress test Avoid competitive sports. Avoid extreme exertion and dehydration Annual F/U No endocarditis prophylaxis No beta-blocker if no symptoms Pregnancy well tolerated—50% chance of affected child 17
  18. 18. Patient with HCM and PAF Beta-blocker Disopyramide if LVOT obstruction Otherwise amiodarone or sotalol Anticoagulation 18
  19. 19. Patient with HCM, NYHA class III,severe LVOT obstruction Beta-blocker Disopyramide 100 mg QID or 200 mg TID Surgical myectomy (preferred in younger patients—90% long term symptom improvement) Septal alcohol ablation—localized septal infarct DDD pacing—no long term benefits 19
  20. 20. Post-operative severe LVOTobstruction Volume expansion Avoid beta-agonists Phenyephrine iv metoprolol/esmolol 20