Steel vs Alcohol. Or Neither. Management of Hypertrophic Cardiomyopathy. Josh Doll, MD January 24, 2015

Transcription

1 Steel vs Alcohol Or Neither Management of Hypertrophic Cardiomyopathy Josh Doll, MD January 24, 2015

2 47yo Male, Mr. L Severe progressive dyspnea on exertion and weight gain Previous avid Cross-Fit participant History of heart murmur since childhood Echo obtained LVOT gradient 57mmHg at rest, 183mmHg with valsalva

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8 Mr. W, 41yo Male

9 Affects 1 in 500 Pathogenesis Most prevalent heritable cardiovascular disease Genotypic, phenotypic and symptomatic heterogeneity Hundreds of mutations within 27 genes identified most commonly involving myofilament genes Autosomal dominant with incomplete penetrance Bos et al, JACC 2009

10 Histology

11 Types of HCM Concentric Apical Septal (Obstructive and Nonobstructive) Sigmoidal

12 Bos et al, JACC 2009 Types of HCM

13 Management Overview Genetic Counseling and screening relatives Assess Risk of SCD (ICD or not) Symptom control

14 Risk of SCD (Who Gets an ICD?)

15 Risk of SCD (Who Gets an ICD?) Major Risk Factors (one of these) Personal history of survived SCD Spontaneous sustained VT Other Risk Factors (two of these) First degree relative with SCD < 45yrs old Exertional Syncope NSVT on ambulatory monitoring Failure to increase SBP > 25mmHg or Drop in SBP > 15mmHg during exercise Septal thickness 3cm Resting gradient > 30mmHg Frenneaux, Heart 2003; McKenna, Heart 2002

16 Diastolic Dysfunction Dyspnea Edema Fatigue Myocardial ischemia Angina Symptom Control Arrhythmia (atrial fibrillation, VT) Palpatations Presyncope and Syncope Dyspnea (exacerbation of CHF) Stroke LV Outflow Obstruction Presyncope and Syncope Dyspnea Fatigue

17 Diastolic Dysfunction Beta Blockers and Non-dihydropyridine CCB Decrease heart rate Diuretics Use with caution if LV outflow obstruction Reduced preload can lead to increased outflow obstruction

18 Myocardial Ischemia Beta Blockers Decrease myocardial oxygen demand Non-dihydropyridine CCB Improve microvascular function Avoid nitrates May drop preload and increase outflow obstruction if present

19 Arrhythmias Yearly ambulatory monitoring to assess for NSVT Atrial fibrillation Very common in association with HCM Usual treatment May prefer rhythm management due to exacerbating symptoms of CHF and outflow obstruction Amiodarone preferred If stroke without identifiable cause OAC

20 Arrhythmias Sustained VT ICD +/- BB or amiodarone Nonsustained VT Low risk Beta Blocker Amiodarone High risk ICD +/- BB or amiodarone No evidence to support prophylactic medical therapy

21 LV Outflow Obstruction Affects 70% of pts with HCM (Maron et al, Circulation 2006) 5-30% of pts require invasive treatment (Spirito et al, NEJM 1997) Beta blockers and Non-dihydropyridine Calcium Channel Blockers (Verapamil) Negative inotropes/chronotropes Increase LV size (filling) Decrease contraction and impingement on LVOT, reduce SAM

22 LV Outflow Obstruction

23 LV Outflow Obstruction Beta blockers and Non-dihydropyridine Calcium Channel Blockers (Verapamil) Negative inotropes/chronotropes Increase LV size (filling) Decrease contraction and impingement on LVOT, reduce SAM Disopyramide Negative inotrope Target 600mg per day Follow QTc

24 Other Special Problems Syncope/hypotension May be due to increased obstruction (vs. arrhythmia) Rule out arrhythmia Increase BB or CCB Volume Bradycardia Add Disopyramide? Careful of QTc If asymptomatic don t overtreat (don t stop BB or CCB)

25 LV Outflow Obstruction Invasive treatment for those whose symptomatically refractory or intolerant of medical therapy RV pacing Inconsistent response (significant placebo effect acutely) Symptoms often recur after initial response Surgical Septal Myectomy Alcohol Septal Ablation

26 LV Outflow Obstruction

27 Surgical Myectomy Pros Lower rate of repeat procedures (Alam et al, Eur Heart J 2009) Can address concominant abnormalities of papillary muscles or mitral valve Can address mid-ventricular obstruction Possibly improved symptom control in young pts (Van der Lee et al, Circulation 2005) Lower risk of pacemaker Cons Sternotomy/cardiopulmonary bypass (associated risks) Longer recovery Higher cost Results confirmed only in experienced hands (?availability good flights to Rochester, MN, Cleveland, OH, or Toronto?) Higher risk of VSD (2%) (Maron et al, JACC 2004)

28 Alcohol Septal Ablation Pros Short hospitalization and recovery Less expensive Operator needs judgement but less technical skill Lower risk of VSD Symptom improvement similar (Alam et al, Eur Heart J 2009) More available Cons Less control of affected myocardium (use of contrast echo helps) Higher pacer rate (10% vs. 5%) Risk of coronary dissection, EtOH leak, pericardial effusion Cannot address other anatomic abnormalities and mid-ventricular obstruction 5% of pts may require second procedure

29 Invasive Treatment Experienced physician familiar with pros and cons of each procedure Myectomy preferred for thick septum, septal ablation in older pts, etc Appropriate EP support for ICD and arrhythmia issues Full discussion with pt regarding options and tayloring to their particular needs

30 Mr. L Intolerant to BB No benefit of increasing doses of CCB Not candidate for disopyramide Selected invasive treatment

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43 Mr. W Symptoms resistant to BB + CCB Selected invasive treatment

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