Highlights from EuroEcho 2009 Echo in cardiomyopathies

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1 Highlights from EuroEcho 2009 Echo in cardiomyopathies Bogdan A. Popescu University of Medicine and Pharmacy, Bucharest, Romania ESC Congress 2010

2 Hypertrophic cardiomyopathy To determine the differences in the degree of fibrosis (estimated by late gadolinium enhancement) in patients with obstructive and non obstructive HCM, as well as its correlation with the LV filling pressures. 67 Patients with HCM: 30% obstructive HCM Age: 56 ± 13 years FC I: 70%, FC II: 20%, FC III: 10% (NYHA) Costabel JP, et al. M 388

3 Methods Presence of LGE Percentage Score Fibrosis score: analysis of 17 segments Values: 0-68

4 Fibrosis score Obstructive vs Non-obstructive p < 0.05 HCM HOCM

5 e wave velocity Fibrosis score and e wave velocity 8 p < n = 63 1 quart. 2 quart. 3 quart. 4 quart. Fibrosis score Costabel JP, et al. M 388

6 E/e Fibrosis score and LV filling pressures n = 67 r = 0.52 p < 0.01 Fibrosis score Costabel JP, et al. M 388

7 Hypertrophic cardiomyopathy Aim to evaluate the value of E/e ratio as a non-invasive estimate of LV filling pressure in patients with HOCM Patients and Methods 38 patients (59.0±15.2 yrs) accepted for septal alcohol ablation for symptomatic HOCM underwent TTE within 24 h of cardiac catheterization E/e ratio calculated between E and e septal, e lateral and between E and a mean of e from all four sites of M annulus Invasive measurement of LVEDP using fluid-filled catheters Eriksson MJ, et al. P 930.

8 Results Invasive pressure measurements LV peak systolic pressure (mmhg) ± 30.4 Pressure gradient across the LVOT 41.7 ± 31.1 (mmhg) LV early diastolic pressure (mmhg) 5.9 ± 4.0 LV end-diastolic pressure (mmhg) 22.0 ± 5.6 TTE and TDI results Septal thickness (mm) 21.1 ± 3.0 Left atrial volume (ml) 64.0 ± 24.7 E/A 1.3 ± 0.8 Deceleration time (ms) 248 ± 82 E/e septal 21.6 ± 10.9 E/e lateral 16.1 ± 10.7 E/e mean (sept, lat) 18.9 ± 9.8 E/e mean (sept, lat, ant, inf) 19.0 ± 10.8

9 E/Em E/e (sept, ant, lat, post) Deceleration Decelerationstid time (ms) E/Em E/e (sept) E/Em E/e (lat) Results correlations with LVEDP Korrelation LVEDP och E/Em septalt 60,00 50,00 40,00 30,00 20,00 10,00 0,00 y = 0,1839x + 17,582 R 2 = 0,009 0,0 10,0 20,0 30,0 40,0 Korrelation LVEDP (mmhg) och E/Em septalt, lateralt, anteriort, inferiort Korrelation LVEDP och E/Em lateralt 60,00 50,00 40,00 30,00 20,00 10,00 0,00 y = -0,0084x + 16,29 R 2 = 2E-05 0,0 10,0 20,0 30,0 40,0 LVEDP (mmhg) Korrelation LVEDP och DT 80,00 60,00 40,00 20,00 0,00 y = 0,0051x + 18,882 R 2 = 7E-06 0,0 10,0 20,0 30,0 40,0 LVEDP (mmhg) y = -6,039x + 382,4 R 2 = 0,1669 0,0 10,0 20,0 30,0 40,0 LVEDP (mmhg)

10 Results There was no significant correlation between any of the calculated E/e ratios and invasively measured LVEDP. There was a weak negative correlation between EDT and LVEDP. Conclusion In patients with HOCM none of the calculated E/e ratios correlated significantly with invasively measured LVEDP. Eriksson MJ, et al. P 930.

11 Exercise echocardiography in HCM Aim: to identify determinants of exercise peak LVOT gradient and their impact on outcome 139 patients No obstruction 68 patients (49%) Obstruction at baseline 49 patients (35%) Latent obstruction 22 patients (16%) Follow-up: 2.3 ± 1.5 y Composite end-point: cardiac death; appropriate discharge by ICD/aborted SCD; unplanned hospitalization for a cardiac event related to HCM Deswarte G, et al. M387

12 Echo characteristics No obstruction Obstruction Baseline Latent P* P** Septal thickness, mm < LVEDD, mm LVEF, % Septal S, cm/s Resting peak gradient Exercise peak gradient LAVi, ml/m² E/E sep P* comparison among all groups; P** for comparison between obstruction subgroups

13 Determinants of exercise peak gradient Multivariate analysis β P Rest peak gradient 0.79 < Septal S (TDI) 0.16 < Multivariate analysis Exclusion resting peak gradient β P Sinus rhythm Septal thickness Septal S (TDI) Posterior mitral leaflet length Deswarte G, et al. M387

14 Determinants of outcome HR CI P Sinus rhythm NYHA class Resting peak gradient < LV EF Exercise SBP Deswarte G, et al. M387

15 Dilated cardiomyopathy 88 pts with non-ischemic dilated cardiomyopathy (mean age: 51 ± 14.1 yrs; mean EF: 31 ± 6.9%) Group A High Filling Pressures E/E > 15 (n= 39) Group B Low Filling Pressures E/E < 15 (n= 49) Serum BNP levels Functional status (NYHA class) Left atrial volume index (LAVi) Serum CA-125 levels Karaca O, et al. P911

16 Group A (E/E > 15) (n=39) Group B (E/E < 15) (n=49) p value Serum CA125 (U / ml) Serum BNP (pg / ml) LAVi (ml / m2) 25.5 [ ] 11.2 [ ] < [ ] 69 [9-1545] < [ ] 30.9 [8.8-57] < Serum BNP (r=0.46, p<0.01) Serum CA-125 correlates with LAVi (r=0.42, p<0.01) Karaca O, et al. P911

17 Serum CA-125 levels (U/ml) Serum CA-125 levels (U/ml) CA-125 levels (U/ml) Group A (E/E'>15) (n=39) NYHA III-IV (n=37) U/ml 11.2 U/ml Group B (E/E' <15) (n=49) p < U/ml 11.2 U/ml NYHA I-II (n=51) p <0.001 Serum CA-125 levels according to E/E ratio Serum CA-125 levels according to NYHA classification CONCLUSIONS: 1. Serum CA125 levels are associated with LV filling pressures 2. CA-125 levels correlate with LAVi, BNP levels, and NYHA class 3. CA-125 can be used as a co-marker that reflects hemodynamic and clinical status in patients with non-ischemic DCMP

18 Dilated cardiomyopathy Hypothesis Combining the analysis of the LV torsion (LVtor) and global longitudinal strain (LVε) may provide additive information for the assessment of LV function. 55 pts with dilated cardiomyopathy (DCM) in sinus rhythm (35 men, 62±12 y/o, LVEF: 29±10%) TTE performed simultaneously with NTproBNP determination Mornos C, et al. O974.

19 Results correlations of NTproBNP with: LVtor LVε (r =0.75, p <0.001) LVtor (r =- 0.69, p <0.001) LVtw (r =- 0.67, p <0.001) LVε (r =0.56, p <0.001) E/E (r =0.49, p =0.002) LV ejection fraction (r =- 0.46, p =0.01) Mornos C, et al. O974.

20 AUC for predicting NTproBNP levels >900 pg/ml

21 Conclusions LVtor LVε correlates strongly with plasma NTproBNP and could be used to evaluate LV function with a good accuracy in patients with DCM in sinus rhythm Mornos C, et al. O974.

22 LV twist in non-compaction cardiomyopathy (NCCM) Normal NCCM Van Dalen et al. Eur J Heart Fail 2008;10:

23 Methods LV rotation patterns as determined by speckle tracking echocardiography and defined as: 1A) Completely normal rotation 1B) Partly normal rotation: normal end-systolic rotation, but absence of initial rotation in the other direction 2) Solid body rotation (SBR): rotation at the basal and apical level predominantly in the same direction. Van Dalen, et al. O975.

24 Results 52 subjects with prominent trabeculations 34 patients with NCCM 18 subjects with hypertrabeculation (expert opinion diagnosis)

25 Results All familial NCCM patients showed solid body rotation (SBR) First-degree NCCM relatives from one family had identical LV rotation patterns Diagnostic value of left ventricular solid body rotation for diagnosis of noncompaction cardiomyopathy in 52 patients with prominent trabeculations Sensitivity, % 88 Specificity, % 78 Positive predictive value, % 88 Negative predictive value, % 78 Accuracy, % 85

26 Conclusion Solid body rotation is an objective and reproducible functional criterion with good predictive value for the diagnosis of NCCM as determined by expert opinion In addition to the current more subjective diagnostic criteria for NCCM, solid body rotation, as a functional diagnostic criterion, should be present for a definite diagnosis of NCCM, although the morphologic criteria should be fulfilled as well Van Dalen, et al. O975.

27 Arrhythmogenic Right Ventricular Cardiomyopathy Aim To evaluate the evolution of RV and LV systolic function at mid-term follow-up in patients with ARVC To evaluate the prognostic impact of RV and LV systolic function assessment at mid-term F-U in ARVC RV Dysfunction: RV FAC <33% LV Dysfunction: LVEF <50% Pyxaras S, et al. O973

28 Evolution of LV and RV dysfunction in ARVC

29 Prognostic impact of LV and RV function at mid-term follow-up in ARVC

30 Conclusions In ARVC RV as well LV systolic function can change in a considerable percentage of patients at mid-term follow-up echocardiographic assessment after tailored medical treatment Prediction of evolution of RV as well as LV systolic function at mid-term is not possibile at initial evaluation The persistence or development of a normal RV as well as LV function at mid-term follow-up is predictive of a better prognosis in the long term Pyxaras S, et al. O973

31 Peripartum cardiomyopathy Aim: to evaluate if early echocardiographic findings can predict functional recovery in patients with peripartum cardiomyopathy (PPCM) PPCM was defined according to guidelines: LVEF<45%, FS<30%, no previous evidence of heart disease and absence of an identifiable cause for HF Improvement was defined as a return to a LVEF>50% Kuperstein R, et al. P1133

32 Results Thirteen patients (52%) improved LVEF Twelve patients did not improve LVEF None of the patients died Time to documented full recovery of function was 7±5.6 months (1 to 16) Follow up time was 41±36 months (4-134) Kuperstein R, et al. P1133

33 Results IMPROVERS NON-IMPROVERS P (n=13) (n=12) AGE (years) 35 ± ± LVEF (%) 34 ± 8 23 ± LVEDD (mm) 52 ± 5 58 ± LVESD (mm) 39 ± 6 48 ± LA (mm) 37 ± 7 40 ± E/E S 11 ± ± ± ± E/E L FS (%) 24 ± 9 17 ± 6 LVEF (Follow Up) 58 ± 4 23 ± 8 <0.0001

34 Conclusions Approximately half of the patients (52%) with PPCM fully recovered their LVEF Patients who tend to recover LVEF have smaller LV dimensions and a LVEF>30% at baseline LVESD was the strongest predictor of recovery None of the 5 patients with a LVEDD >60 mm and LVESD >45 mm recovered LVEF Kuperstein R, et al. P1133

35 Fabry cardiomyopathy At least a moderate amount of LVH is needed for the development of myocardial fibrosis in males Female cardiomyopathy was considered rare in the past. Nowadays it is widely accepted that heterozygous females could develop severe cardiomyopathy and other organ manifestations Association between the three cardiomyopathic hallmarks (hypertrophy, function reduction, fibrosis) in female patients remains largely unknown Niemann M, et al. P1135.

36 Study protocol 58 female and 46 male Fabry patients all patients: MRI with late enhancement (LE) all patients: general echo + strain rate imaging Hypertrophy assessment: 1. diastolic left ventricular wall thickness (LVWT) greater than 12 mm 2. myocardial mass using Devereux formula corrected for BSA

37 Results

38 Results Niemann M, et al. P1135.

39 Conclusions Fabry cardiomyopathy in female patients differs from male patients Female patients can develop fibrosis without showing LVH. Half of the female patients with signs of a relevant cardiomyopathy would not have been detected by the guideline proposed pure standard echo and LVH assessment Staging and monitoring Fabry female patients should include the assessment of replacement fibrosis Niemann M, et al. P1135.

40 Thank you!