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SCD 2014: Hypertrophic and Other Cardiomyopathies
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SCD 2014: Hypertrophic and Other Cardiomyopathies

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Andrew Wang, MD

Andrew Wang, MD

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  • Figure 1. Distribution of Maximal Left-Ventricular-Wall Thicknesses in the 947 Elite Athletes. Shaded bars indicate wall thicknesses within the normal range, and solid bars those within a range compatible with the diagnosis of hypertrophic cardiomyopathy (≥13 mm).
  • Distribution of left ventricular wall thickness in 300 highly trained black male athletes and 300 white male athletes of similar age, size, and sporting calibre demonstrating that a significantly higher proportion of black athletes exhibit a wall thickness >12 mm compared with white athletes (8 vs. 3%). Reproduced from Basavarajaiah et al.7 with permission from the American College of Physicians.
  • In this article, which highlighted relatively elite athletes found to have HCM, the focus was on the risk of SCD
  • Illustrates in a large unselected HCM patient population (N=744), that sudden death occurs most commonly in young patients < 25 years old, but in addition, this risk of sudden death also persists into mid-life and beyond.
  • In HCM the magnitude of hypertrophy is directly related to the risk of sudden death and is a strong independent predictor of prognosis. Over a mean follow-up of 6.5 years, 65 of 480 patients (14%) died: 23 suddenly, 15 of heart failure, and 27 of non cardiac causes or stroke. In the 23 persons who died suddenly, the risk of sudden death increased progressively and in direct relation to wall thickness (p = .001)
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    • 1. Andrew Wang, MD Professor of Medicine April 12, 2014 Hypertrophic Cardiomyopathy
    • 2. All Rights Reserved, Duke Medicine 2008 Hypertrophic Cardiomyopathy • Clinical definition and prevalence • Diagnosis • Differentiation from athlete’s heart • Sudden cardiac death in HCM – Risk factors – Prevention
    • 3. All Rights Reserved, Duke Medicine 2008 Hypertrophic Cardiomyopathy • Prevalence 1 in 500 persons; autosomal dominant • Left ventricular hypertrophy >14 mm in any region not due to other condition (aortic stenosis, HTN) • Only 30% have resting LV outflow obstruction; another 40% have provokable obstruction with exercise • Clinical presentation: dyspnea, chest pain, syncope, palpitaitions, sudden death
    • 4. All Rights Reserved, Duke Medicine 2008 20 year old male college athlete who had dizziness during sprint training exercise.
    • 5. All Rights Reserved, Duke Medicine 2008 Echo showed mild LVH. CMR performed: • Max wall thickness 14 mm • No LVOT obstruction • Normal RV • No delayed hyperenhancement
    • 6. All Rights Reserved, Duke Medicine 2008 Athlete with dizziness • Family history: – Negative • EKG: – Abnormal (ST changes) • Echo: – LVH with maximum wall thickness 14 mm – No systolic anterior motion of mitral valve – LVOT velocity 2 m/sec • MRI: – Max septal wall thickness 14 mm; no fibrosis Does he have hypertrophic cardiomyopathy?
    • 7. All Rights Reserved, Duke Medicine 2008 Driest et al. Mayo Clin Proc 2005;80:463-469. Yield of Genetic Testing ~50-60%
    • 8. All Rights Reserved, Duke Medicine 2008 Athlete’s Heart vs. HCM • LVH <15 mm • Increased LV volume • Normal diastolic fxn Cardiac Disease in Young Trained Athletes: Insights Into Methods for Distinguishing Athlete's Heart From Structural Heart Disease, With Particular Emphasis on Hypertrophic Cardiomyopathy. Maron, Barry; Pelliccia, Antonio; Spirito, Paolo Circulation. 91(5):1596-1601, March 1, 1995.
    • 9. All Rights Reserved, Duke Medicine 2008 . LV Wall Thickness in Elite Athletes Pelliccia A et al. N Engl J Med 1991;324:295-301.
    • 10. All Rights Reserved, Duke Medicine 2008 LV Hypertrophy in 23% of NFL Players JACC 2003;41:280–4
    • 11. All Rights Reserved, Duke Medicine 2008 Athlete’s Heart is Associated with Mild LV Dilation JACC 2003;41:280–4
    • 12. All Rights Reserved, Duke Medicine 2008 Rawlins J et al. Eur J Echocardiogr 2009;10:350-356 Greater LVH in Male and Black Athletes
    • 13. All Rights Reserved, Duke Medicine 2008 De-Training Reverses Changes of Athlete’s Heart Circulation. 2002;105:944-949
    • 14. All Rights Reserved, Duke Medicine 2008 Sports Illustrated 12/2007.
    • 15. All Rights Reserved, Duke Medicine 2008 0 2 4 6 8 10 12 14 16 Sudden death Heart failure Stroke %Mortality Age at Death or Most Recent Evaluation (years) 5-15 16-25 26-35 36-45 46-55 56-65 66-75 >75 Maron BJ et al. Circulation 2000;102:858-864.
    • 16. All Rights Reserved, Duke Medicine 2008 Risk Factors for SCD in HCM • Cardiac arrest/sustained VT • Family history of sudden death • Recurrent, unexplained syncope • Extreme LVH (max thickness >30 mm) • Non-sustained VT • Abnormal blood pressure response during exercise • LVOT gradient >30 mm Hg
    • 17. All Rights Reserved, Duke Medicine 2008 Spirito P. et al. NEJM 2000:342;1781. Wall Thickness and Sudden Death 0 2 4 6 8 10 12 14 16 18 20 Maximal Left Ventricular Wall Thickness (mm) 0 2.6 7.4 11.0 18.2 <15 16 - 19 20 - 24 25 - 29 > 30 IncidenceofSuddenDeath (per1000person–yr)
    • 18. All Rights Reserved, Duke Medicine 2008 ICDs for the Prevention of SCD in HCM • Multicenter; 506 unrelated pts 1986-2003 • 75% primary prevention ICD • 20% had appropriate discharge over 3.7 yr follow-up – 3.6% per year primary prevention – 10.6% per year secondary prevention • 27% had first discharge >5 years after implant • 27% had inappropriate discharge JAMA. 2007;298(4):405-412
    • 19. All Rights Reserved, Duke Medicine 2008 ICD for Prevention of SCD in HCM JAMA. 2007;298(4):405-412
    • 20. All Rights Reserved, Duke Medicine 2008 ICD Appropriate Discharge: No Difference Based on Number of SCD Risk Factors JAMA. 2007;298(4):405-412
    • 21. All Rights Reserved, Duke Medicine 2008 Similar Rates of Therapies for Specific SCD Risks JAMA. 2007;298(4):405-412 Family history of SCD Syncope Massive LVH NSVT % with appropriate ICD therapy 13% 14% 12% 14% ICD therapy per 100 pt-yr 3.3 3.8 3.6 4.2
    • 22. All Rights Reserved, Duke Medicine 2008 Circulation published online November 8, 2011
    • 23. All Rights Reserved, Duke Medicine 2008 Exercise Recommendations in Patients with HCM (36th Bethesda Conference) Level of Exercise Examples Recommendation High Basketball, soccer, singles tennis, sprinting Not recommended Moderate Jogging, surfing, sailing, skiing Individual basis Low Biking, hiking, walking, doubles tennis, bowling, skating Acceptable Competitive exercise acceptable for patients with genetic mutation but no symptoms or phenotypic expression (LVH). Circulation. 2004;109:2807-16.
    • 24. All Rights Reserved, Duke Medicine 2008 Back to our patient… • De-conditioned for 3 months • Repeat cardiac MRI: Slight regression of LVH (max septal thickness now 13 mm)
    • 25. All Rights Reserved, Duke Medicine 2008 ECG after Deconditioning
    • 26. All Rights Reserved, Duke Medicine 2008 HCM in Athletes • Diagnosis should exclude athlete’s heart as cause of LVH. – Normal exercise capacity, no family history – Mild concentric hypertrophy with normal diastolic fxn – LV dilation – Regression changes with deconditioning • Exercise in HCM should be limited to moderate (non- competitive) levels. • Primary prevention ICD recommended for HCM patients with risk factors, esp massive hypertrophy, family history of SCD, recurrent syncope.