Right Ventricular Remodeling, Its Correlates, and Its Clinical Impact in Hypertrophic Cardiomyopathy

Roşca, Monica; Călin, Andreea; Beladan, Carmen C.; Enache, Roxana; Mateescu, A.; Gurzun, Maria-Magdalena; P, Varga; Băicuș, Cristian; Coman, Ioan Mircea; Jurcuţ, Ruxandra; Ginghină, Carmen; Popescu, Bogdan A

doi:10.1016/j.echo.2015.07.015

Cited by 64 publications

(58 citation statements)

References 31 publications

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“…This study demonstrated reduced LV volumes in HCM LVH+ patients compared with HCM LVH− patients and healthy individuals in line with previous studies 20. The reduced LV volumes may have several explanations.…”

Section: Discussionsupporting

confidence: 92%

The systolic paradox in hypertrophic cardiomyopathy

et al. 2017

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ObjectiveWe explored cardiac volumes and the effects on systolic function in hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (HCM LVH+) and genotype-positive patients without left ventricular hypertrophy (HCM LVH−).MethodsWe included 180 HCM LVH+, 100 HCM LVH− patients and 80 healthy individuals. End-Diastolic Volume Index (EDVI), End-Systolic Volume Index (ESVI) and ejection fraction (EF) were assessed by echocardiography. Left ventricular (LV) global longitudinal strain (GLS) was measured by speckle tracking echocardiography.ResultsEDVI and ESVI were significantly smaller in HCM LVH+ compared with HCM LVH− patients (41±14 mL/m2 vs 49±13 mL/m2 and 16±7 mL/m2 vs 19±6 mL/m2, respectively, both p<0.001) and in healthy individuals (41±14 mL/m2 vs 57±14 mL/m2 and 16±7 mL/m2 vs 23±9 mL/m2, respectively, both p<0.001). HCM LVH− patients had significantly lower EDVI and ESVI compared with healthy individuals (49±13 mL/m2 vs 57±14 mL/m2 and 19±6 mL/m2 vs 23±9 mL/m2, both p<0.001). EF was similar (61%±7% vs 60%±8% vs 61%±6%, p=0.43) in the HCM LVH+, HCM LVH– and healthy individuals, despite significantly worse GLS in the HCM LVH+ (−16.4%±3.7% vs −21.3%±2.4% vs −22.3%±3.7%, p<0.001). GLS was worse in the HCM LVH− compared with healthy individuals in pairwise comparison (p=0.001). Decrease in ESVI was closely related to EF in HCM LVH+ and HCM LVH− (R=0.45, p<0.001 and R=0.43, p<0.001) as expected, but there was no relationship with GLS (R=0.02, p=0.77 and R=0.11, p=0.31). Increased maximal wall thickness (MWT) correlated significantly with worse GLS (R=0.58, p<0.001), but not with EF (R=0.018, p=0.30) in the HCM LVH+ patients.ConclusionHCM LVH+ had smaller cardiac volumes that could explain the preserved EF, despite worse GLS that was closely related to MWT. HCM LVH− had reduced cardiac volumes and subtle changes in GLS compared with healthy individuals, indicating a continuum of both volumetric and systolic changes present before increased MWT.

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Section: Discussionsupporting

confidence: 92%

The systolic paradox in hypertrophic cardiomyopathy

et al. 2017

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“…Right ventricular hypertrophy is associated with increased risk of hospitalization for congestive heart failure (CHF), thromboembolic complications, ventricular tachyarrhythmias, and sudden death;9, 10 and, severe RV hypertrophy, albeit rare, is associated with worse prognosis in humans with HCM 11. The impact of RV performance in the clinical status and outcome of humans affected with HCM also has been studied 12, 13, 14, 15.…”

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confidence: 99%

Echocardiographic Assessment of Right Ventricular Size and Function in Cats With Hypertrophic Cardiomyopathy

Visser

Sloan

Stern

2017

Veterinary Internal Medicne

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BackgroundStudies evaluating right ventricular (RV) structural and functional abnormalities in feline hypertrophic cardiomyopathy (HCM) are limited.HypothesisRight ventricular structural and functional abnormalities are present in cats with HCM and are associated with clinical severity.AnimalsEighty‐one client‐owned cats.MethodsRetrospective 2‐dimensional (2D) echocardiographic study. Right atrial diameter (RAD), RV free wall thickness (RVFWd), RV internal dimension (RVIDd), RV fractional area change (FAC), and tricuspid annular plane systolic excursion (TAPSE) were measured in control cats (n = 26), cats with subclinical HCM (subclinical HCM; n = 31), and cats with HCM and congestive heart failure (HCM + CHF; n = 24).ResultsRight heart size (RAD, RVFWd, and RVIDd) and RV function (FAC and TAPSE) significantly (all P < .05) increased and decreased, respectively, in the HCM + CHF group compared with controls. In the subclinical HCM group, only RVFWd was significantly (P < .05) higher than in controls. Compared with reference intervals derived from controls, 29% of cats with HCM had increased RVFWd. Increased left ventricular free wall thickness, increased RVIDd and decreased TAPSE independently correlated with increased left atrial size. Cats with HCM and pleural effusion were significantly more likely to have increased RVFWd and had increased RAD and decreased TAPSE compared with cats without pleural effusion.Conclusions and Clinical ImportanceRight ventricular remodeling and dysfunction occur in some cats with HCM and may be associated with clinical severity. Our results support involvement of RV in the pathophysiology of HCM in some cats and support echocardiographic assessment of the RV in cats with HCM.

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“…10,15 A value of RVWT > 5 mm was used to define the presence of RV hypertrophy. 10,15 A value of RVWT > 5 mm was used to define the presence of RV hypertrophy.…”

Section: Transthoracic Echocardiographymentioning

confidence: 99%

Echocardiographic features of Fabry cardiomyopathy—Comparison with hypertrophy‐matched sarcomeric hypertrophic cardiomyopathy

et al. 2019

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Background The concept of “red flags” has been particularly useful in the etiologic diagnosis of cardiomyopathies such as Fabry disease, as early detection is often essential for treatment response and outcomes. The present study sought to describe the echocardiographic features that may differentiate Fabry cardiomyopathy from sarcomeric hypertrophic cardiomyopathy (HCM). Methods Forty patients with left ventricular (LV) hypertrophy were prospectively included and divided into two groups: the Fabry group (20) and the sarcomeric HCM group (20). The two groups were matched for LV hypertrophy (similar maximum wall thickness and indexed LV mass) and age. All patients underwent full echocardiographic evaluation including ventricular strain analysis. Results The Fabry group had significantly lower LV ejection fraction (63 ± 7 vs 72 ± 7%, P = .001) and higher LV end‐systolic diameter (28 ± 7 vs 22 ± 5 mm, P = .004). LV hypertrophy in Fabry patients was more often concentric, with a significantly lower interventricular septum/posterior wall ratio (1.22 ± 0.63 vs 1.55 ± 0.66, P = .001). Fabry patients had more reduced regional longitudinal strain in the inferolateral part of the LV (−9 ± 5 vs –16 ± 7%), and RV free wall longitudinal strain was also worse in Fabry patients (−23 ± 6 vs –28 ± 5%, P = .027). Conclusion These parameters are promising echocardiographic features to identify patients with Fabry cardiomyopathy and may help for the detection and subsequent management of these patients.

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Right Ventricular Remodeling, Its Correlates, and Its Clinical Impact in Hypertrophic Cardiomyopathy

Cited by 64 publications

References 31 publications

The systolic paradox in hypertrophic cardiomyopathy

The systolic paradox in hypertrophic cardiomyopathy

Echocardiographic Assessment of Right Ventricular Size and Function in Cats With Hypertrophic Cardiomyopathy

Echocardiographic features of Fabry cardiomyopathy—Comparison with hypertrophy‐matched sarcomeric hypertrophic cardiomyopathy

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