Pembelajaran echo tentang Hypertrophy Cardiomyopathy.pptx

Echo

1. HYPERTROPHY CARDIOMIOPATHY & WALLMOTION ANALYSIS PRESENTER : RIVHAN FAUZAN SUPERVISOR : DR. T. WINDA ARDHINI, SPJP ECHO CLUB – DIVISI ECHOCARDIOGRAPHY & NON INVASIF

2. INTRODUCTION Hypertrophic cardiomyopathy (HCM) is defined by the presence of increased left ventricular (LV) wall thickness that is not solely explained by abnormal loading conditions HCM is the most common genetic cardiomyopathy. Across multiple geographies and ethnicities, the prevalence is approximately 0.2%. In up to 60% of adolescents and adults with HCM, the disease is an autosomal dominant trait caused by mutations in cardiac sarcomere protein genes Echocardiography is of vital importance in the diagnosis, assessment and follow-up of patients with known or suspected HCM.

3. CLASSIFICATION (EACVI 2015) I (anterior septum) II (anterior + inferior septum) III (anterior + inferior septum + lateral wall) IV (apical, etc.)

4. 65% 35% 10%

5. Binder J, et al. Mayo Clin Proc 2006; 81: 459. 181(47%) Gene + (8%) 132(35%) Gene + (79%) 37(10%) Gene + (32%) 32(8%) Gene + (41%) Sigmoid Septum Rev e r s e Septum Neutral Septum Apical V a r i a nt

6. LVH in HCM: Sigmoid Septum

7. LVH in HCM: Neutral Septum

8. LVH in HCM: Reversed Septum

9. Apical HCM

10. SYSTEMATIC APROACH

11. Confirming LV Hypertrophy Although HCM is typically characterised by asymmetric septal hypertrophy (ASH), almost any myocardial segment may be involved.  The following (2D) echocardiographic criteria are used to aid diagnosis: Unexplained maximal wall thickness >15 mm in any myocardial segment, or  Septal/posterior wall thickness ratio >1.3 in normotensive patients, or Septal/posterior wall thickness ratio >1.5 in hypertensive patients. 

12. Confirming LV Hypertrophy Assessing the extent and severity of hypertrophy must include the measurement of maximal wall thickness in all LV segments from base to apex, ensuring that the wall thickness is recorded at mitral, mid-LV and apical levels. In first-degree relatives, lower cut-off values are used, and a WT ≥ 13 mm in the anterior septum or posterior wall suggests the diagnosis

13. Associated Abnormalities of LVOT

14. MR CW Doppler ( Δ P  4V 2 ) LVOT

15. Resting LVOT gradient = 12 mmHg

16. Valsalva : LVOT gradient = 34 mmHg

17. Assesment of latent Obstruction Exercise stress echocardiography is recommended in symptomatic patients if bedside manoeuvres fail to induce LVOTO ≥50 mm Hg. Pharmacological provocation with Dobutamine is not recommended, as it is not physiological and can be poorly tolerated. nitrates do not reproduce exercise-induced gradients and should be reserved for patients who cannot perform physiologically stressful procedures.

18. Other Echo features on Obstruction HCM Mid-systolic notching coarse systolic fluttering of the aortic valve fibrotic septal changes at the level of leaflet-septal contact

19. Mitral Valve in HCM leaflets: anterior leaflet elongation; dysplasia, MVP; chordae: elongation, laxity, hypermobility; papillary muscles: hypertrophy, bifidity, abnormal anterior position, abnormal insertion in the anterior leaflet

20. Mitral Valve in HCM Systolic anterior motion (SAM) of the mitral valve nearly always results in failure of normal leaflet coaptation and mitral regurgitation, which is typically mid-to-late systolic and inferolaterally oriented

21. Systolic Anterior Motion (SAM)

22. LV Systolic Function (Early) Ejection fraction usually is preserved despite significant impairment of longitudinal contractile function, evidenced by attenuation in systolic annular velocities, strain and strain rate Strain imaging  identify regional heterogenity in contractile function

23. LV Systolic Function (Late) Myocardial fibrosis  progressive impairment of systolic function  end-stage HCM Deterioration of systolic function  Increased mortality (11% year) and sudden cardiac death

24. Left Atrial Enlargement Left atrial (LA) volume is largely determined by the presence of diastolic dysfunction, mitral regurgitation, and atrial myopathy. Although LA enlargement as assessed from linear dimensions was shown to independently predict long-term prognosis in patients with HCM. ASE / EACVI recommends using LAVI = 22 ± 6 ml/m2). LA volume is long-term independent indicator of functional capacity and an LA volume index of >34 ml/m2 has been shown to be predictive of a greater degree of LVH, severity of diastolic dysfunction, and adverse cardiovascular outcomes.

25. LV Diastolic Function Patients with HCM often have diastolic dysfunction, mostly indicating impaired myocardial relaxation, regardless of symptoms or presence of LV outflow obstruction Assessment of LV filling pressures is helpful in the evaluation of symptoms and disease staging  Patients with a restrictive LV filling pattern may be at higher risk for adverse outcome, even with a preserved ejection fraction (EF)

26. Tissue Doppler Imaging (TDI) TDI has become standard in most tertiary centres managing patients with cardiomyopathies. DI may also help in the differentiation of various conditions resulting in LVH, with demonstrable differences in TDI velocities between conditions of physiological hypertrophy (athlete’s heart) and pathological hypertrophy. Mean systolic annular motion Sa <9 cm/s is the parameter for differentiating pathological LVH (HCM/hypertensive LVH) from physiological LVH (diagnostic accuracy of 92%)

27. MOTION ANALYSIS – REGIONAL / SEGMENTAL Normokinetik Systolic increase in free wall thickness >40% Hypokinetik Increase in systolic wall thickness <40% Akinetik Increase in systolic wall thickness <10% Dyskinesia Outward movement of wall during systole with associated wall thinning Aneurismal Dilatation and outward movement of wall during systole with associated systolic wall thinning and diastolic deformation

28. REGIONAL WALL MOTION SCORE INDEX (CAD) Each wall segment:1. Normal (>40%),2. Hypokinesis (10-30%), 3.Severe Hypokinesis – akinesis (<10%), 4. Dyskinesis , and 5.Aneurism

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