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Case presentation ethan
- 1. Controversies in Hypertrophic Cardiomyopathy: CasePresentation Ethan Rowin, MD Associate Director, Hypertrophic Cardiomyopathy Center Tufts Medical Center, Boston MA Chanin T. Mast Center for Hypertrophic Cardiomyopathy Morristown Medical Center, Morristown NJ
- 2. HCM Case Presentation •40-year-old male with HCM referred for management of progressive heart failure symptoms over the past 3 years. • Limiting exertional dyspnea (stairs and gradual inclines; NYHA Class II) and atypical chest pain despite Toprol XL 150mg. • In addition, severe post-prandial SOB resulting in significant limitation, even walking on level ground (class III); and 30 pound weight loss • One syncopal episode, 1 year prior, occurred with exertion, 30 minutes after ingestion of a large meal
- 3. Past Medical History •Atypical chest pain prompted cardiac catheterization which demonstrated no CAD • No other significant medical history • Employed as a transporter of raw materials • No family history of HCM or unexplained sudden death • 14 year-old asymptomatic daughter underwent a recent screening echocardiogram without LV hypertrophy • No NSVT on 24 hour Holter monitoring
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- 5. Echocardiogram at Rest ParasternalLong-Axis Apical 4-Chamber
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- 8. 16mm LV RV LV RV 2% LGE Basal Short-AxisContrast Enhanced CMR
- 9. HCM Case • MaximumLV wall thickness of 16mm • Ejection Fraction of 60% • SAM at rest and with exercise but no LVOT obstruction • Trace mitral regurgitation • Normal BP response to exercise • Minimal LGE on CMR (2% of LV myocardium) • MYPC3 Arg502Trp positive pathogenic mutation
- 10. What is thenext step? 1. Evaluation/ management of his symptoms 2. Risk stratification 3. Family Screening
- 11. Exercise echo was repeatedseveral days later off medications for 24 hours and one hour after a small meal…
- 12. 6:00 minutes onBruce Protocol… Mild MR 130 mmHg LV LA Ao
- 13. Management • Initially notinterested in moving forward with invasive option • Started on Disopyramide… • Limiting symptoms improved for several months before eventually recurred • An exercise echocardiogram was repeated which confirmed prior findings • At this point patient elected to move forward with invasive option…
- 14. Management • Alcohol SeptalAblation • Surgical Myectomy • Mitral valve replacement
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- 17. Post-operative Myectomy +MV plication Isuprel MR
- 18. One Year Later… Asymptomatic…Able to eat all foods without symptoms. Very happy with his improved quality of life.
- 19. Symptomatic HCM patient without obstructionat rest Exercise Echocardiography Provocable obstruction (≥30mmHg) Nonobstructive (<30mmHg) Myectomy/ Alcohol Ablation Uncertainty Persists BB/CCB/Norpace Pharmacologic Provocation Transplant BB/CCB
- 20. Provocable obstruction n=220 I/II 80% III/IV 20%NYHA Class I/II 6.5 ± 2 Rateof Heart Failure progression = 3.2%/year Exercise Echocardiogram Predicts Progressive Heart Failure Symptoms in HCM Patients with No/Mild Symptoms
- 21. What is thenext step? 1. Evaluation/ management of his symptoms 2. Risk stratification 3. Family Screening
- 22. Sudden Death RiskAssessment • Maximum LV wall thickness = 16mm • Ejection Fraction of 60% • Unexplained Syncope 2 years ago • Normal BP response to exercise • No FH of SD due to HCM • No NSVT on Holter • MYPC3 Arg502Trp positive pathogenic mutation • CMR with 2% LGE
- 23. Family evaluation • Underwentgenetic testing which returned positive for MYPC3 Arg502Trp positive pathogenic mutation • He has an asymptomatic 14 year old daughter who has had a recent echocardiogram without LV hypertrophy • Next step in the approach to screening his family?
- 25. 0.50 0.60 0.70 0.80 0.90 1.00 FreedomfromProgressionto NYHAClassIII/IV 0 2 46 8 10 Time from Initial Visit (years) p=.003 Nonobstructive Provocable Obstruction 1.5%/yr 3.2%/yr 0.40 Exercise Echocardiogram Predicts Progressive Heart Failure Symptoms in HCM Patients with No/Mild Symptoms
- 26. Family evaluation • Underwentgenetic testing which returned positive for MYPC3 Arg502Trp positive pathogenic mutation • He has a 14 year old daughter who has had a recent echocardiogram without LV hypertrophy • Next step in the approach to screening his family?
- 27. Non- obstructive Provocable obstruction n=220 I/II 90% III/IV 10% I/II 80% III/IV 20% NYHA Class n=249 NYHA Class I/II NYHA Class I/II 6.5 ±2 6.5 ± 2 1.5%/year 3.2%/year p=0.003 Exercise Echocardiogram Predicts Progressive Heart Failure Symptoms in HCM Patients with No/Mild Symptoms
- 28. HCM patients without LVoutflow obstruction at rest (<30mmHg) and NYHA class I or II n=469 Exercise Echocardiogram Provocable Obstruction (≥ 30mmHg) n=220 Progression to Class III/IV n=43 (20%) Myectomy or Alchol Septal Ablation n=32/43 Class I/II N=30/32 (94%) Nonobstructive (<30mmHg) n=249 Progression to Class III/IV N=24 (10%) Heart Transplant n=10/24 (42%) Follow-up 6.5 ± 2 yrs Rate of heart failure progression to NYHA class III/IV = 1.5%/year Rate of heart failure progression to NYHA class III/IV = 3.2%/year
- 29. Outflow obstruction canbe effectively relieved with myectomy even in HCM patients with minimal hypertrophy without need for MV replacement… Pre-Op Gradient/MR 100±35 mmHg Mild MR (n=9) Post-Op Complications Post-Op Gradient/MR Complete Heart Block (n=3*) 6±2 mmHg Mild MR (n=9) *1 patient with pre-op right bundle branch block 93±34 mmHg Mild-Mod MR (n=10) Severe MR (n=2) Complete Heart Block (n=1) Tamponade (n=2) 6±1 mmHg Mild MR (n=12) 60 mmHg Mild MR None 9 mmHg Mild MR
- 30. Should this patientbe considered for primary prevention ICD placement?
- 31. Surgical Approach? • ExtendedMyectomy • Myectomy plus MV repair (plication) • Mitral valve replacement