Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.



Published on

  • Be the first to comment


  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