scholarly journals Predictors of right ventricular function and size in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Mateusz Śpiewak ◽  
Mariusz Kłopotowski ◽  
Łukasz Mazurkiewicz ◽  
Ewa Kowalik ◽  
Joanna Petryka-Mazurkiewicz ◽  
...  

AbstractWe investigated factors associated with right ventricular (RV) function and size in hypertrophic cardiomyopathy (HCM) patients. Two hundred fifty-three consecutive HCM patients and 20 healthy volunteers underwent cardiac magnetic resonance examination. In addition to measuring RV function (ejection fraction—RVEF) and size (end-diastolic volume—RVEDV), each image was inspected for the presence of RV and left ventricular (LV) hypertrophy, and the maximal wall thickness of the left and right ventricles was recorded. HCM patients had higher RVEF and lower RVEDV than healthy volunteers and similar RV mass. The mean RV wall thickness was higher in HCM patients than in controls. LV late gadolinium enhancement (LGE) was present in 89.7% of patients, and RV LGE was present in 3.1% of patients (p < 0.0001). Univariate and multivariable analyses revealed that LVEF, peak LV outflow tract gradient, LV LGE, maximal LV wall thickness, and tricuspid regurgitation (TR) volume by magnetic resonance imaging were positive predictors of RVEF. In addition to TR volume, the only independent predictor of RVEF < 45% was LVEF (odds ratio = 0.80, 95% confidence interval 0.67–0.95). Multivariable analysis revealed that LVEDV and TR volume were positive predictors of RVEDV, whereas negative predictors were RVEF, maximal RV wall thickness, LV LGE, and age. Neither estimated systolic pulmonary artery pressure nor TR grade by echocardiography proved to be predictors of RVEF. There were no differences in either the maximal RV wall thickness or the maximal left ventricular (LV) wall thickness in patients stratified according to NYHA functional class (p = 0.93 and p = 0.15, respectively). There were no differences in mean RV wall thickness in patients categorised based on the number of clinical risk factors for sudden cardiac death (SCD), i.e., non-sustained ventricular tachycardia, family history of SCD, or unexplained syncope (p = 0.79). On the other hand, there was a weak positive association between RV hypertrophy and the estimated probability of SCD at 5 years (rho = 0.16, p = 0.01). RV systolic dysfunction measured as decreased RVEF was uncommon in HCM and was associated with poor LV systolic function. LV also had a significant impact on RV size.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Keteepe-Arachi ◽  
A Malhotra ◽  
J Basu ◽  
G Parry-Williams ◽  
B Ensam ◽  
...  

Abstract Background Structural cardiac adaptations due to hypertension (HTN) present a diagnostic challenge when differentiating from hypertrophic cardiomyopathy (HCM), using traditional imaging techniques such as echocardiography (echo). Cardiac magnetic resonance imaging (CMR) offers reproducible anatomical, functional quantification and myocardial tissue characterisation which discriminates between hypertension and HCM. Purpose To identify hypertensive individuals with undiagnosed HCM using CMR imaging. Methods 100 consecutive hypertensive patients underwent CMR at a tertiary centre dedicated blood pressure clinic (55% male, mean age 51 years). In keeping with ESC guidelines, end diastolic wall thickness (EDWT) ≥15mm identified individuals within the “grey zone” between hypertension and with a potential HCM diagnosis. 19 individuals were referred on to the dedicated inherited cardiac conditions clinic for further evaluation. Four patients expressed a definitive LV phenotype and were diagnosed with HCM. CMR parameters were compared in three groups: Hypertensive (HTN), “grey zone” Hypertensive (GZH) and HCM. Results CMR demonstrated end diastolic wall thickness (EDWT) >11 mm in 50% of hypertensives. 73% of the referred patients were Afro-Caribbean (AC) and all 4 HCM patients were AC. All referrals demonstrated EDWTs ≥14mm, 9 (47%) demonstrated late gadolinium enhancement of which 3 (16%) had HCM. Three had asymmetrical septal hypertrophy – 2 were in the HCM cohort and one underwent endomyocardial biopsy confirming HTN. Left ventricular mass index (LVMI) was significantly higher in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0004). EDWT was significantly greater in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0002). There was no significant difference in these parameters between GZH and HCM. Table 1. P-values for CMR data in hypertensive (HTN), gray zone hypertensive (GZH) and hypertrophic cardiomyopathy (HCM) cohorts CMR Parameter HCM vs. HTN GZH vs. HTN GZH vs. HCM EDVI NS p=0.025 NS EDWT p=0.0002 p<0.0001 NS LVMI p=0.0004 p<0.0001 NS Conclusion This study reports a 4% prevalence of HCM among hypertensive patients - 20 x greater than in the general population - which would be left undiagnosed using echo alone. Screening hypertensive individuals with CMR is not routine but we advocate its use in these individuals especially in Afro Caribbeans and in those in the “grey zone”, to identify undiagnosed HCM, which has significant implications for lifestyle modification and family screening. Acknowledgement/Funding Cardiac Risk in the Young


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Jiwon Seo ◽  
Yoo Jin Hong ◽  
Young Jin Kim ◽  
Purevjargal Lkhagvasuren ◽  
Iksung Cho ◽  
...  

AbstractWe sought to investigate the prevalence, functional characteristics, and clinical significance of right ventricular (RV) involvement in patients with hypertrophic cardiomyopathy (HCM). A total of 256 patients with HCM who underwent both cardiac magnetic resonance (CMR) imaging and transthoracic echocardiography within 6 months of each other were retrospectively analysed. RV involvement was defined as an increased RV wall thickness ≥ 7 mm on CMR in the segments of the RV free wall. Primary outcomes were defined as the composite of all-cause death, heart transplantation, and unplanned cardiovascular admission. Thirty-seven (14.4%) patients showed RV involvement. Patients with RV involvement showed a significantly higher left ventricular (LV) maximal wall thickness and left atrial volume index. Multivariate Cox model revealed that RV involvement was independently associated with primary outcomes (HR: 2.30, p = 0.024). In a subgroup analysis of patients with speckle tracking echocardiography (n = 190), those with RV involvement had significantly more impaired RV strain, which was independently associated with primary outcomes. RV involvement in patients with HCM correlated with more advanced LV structure and biventricular dysfunction, suggesting an indicator of severe HCM. RV involvement and impaired RV strain have a prognostic value related to clinical adverse events in patients with HCM.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Ronen Rubinshtein ◽  
James F Glockner ◽  
Bernard J Gersh ◽  
Philip A Araoz ◽  
Michael J Ackerman ◽  
...  

Background: Contrast enhanced magnetic resonance imaging (CE-MRI) is a useful tool to define cardiac morphology, systolic function, and the presence of late gadolinium enhancement (LGE). In patients (pts) with hypertrophic cardiomyopathy (HCM), LGE may represent intra-myocardial fibrosis. We explored the association between left ventricular (LV) morphology and function to the presence and amount of LGE in pts with HCM. Methods: 424 HCM pts (age = 55 ± 16 years, 41% females), without prior history of septal ablation/myectomy, underwent CE-MRI (GE 1.5T) during a 6-year period. LV volumes, diameter, ejection fraction (EF), septal shape and thickness, and the presence of obstructive physiology were assessed from standard steady state free precession pulse sequences. The presence and amount of LGE were traced from a dynamic post contrast inversion recovery pulse sequence. Morphologic and functional findings were then compared with presence and amount of LGE. Results: Over half of the pts [239/424 (56%)] had LGE, most commonly localizing to RV insertion points [174/239 pts, (73%)]. The extent of LGE ranged from 0.4–65% of LV mass. Univariate (morphologic) predictors of LGE were: reverse curve septal shape [OR = 7.8, (95% CI, 3.9–15.6)], septal thickness ≥ 16 mm (OR = 4.5, 2.4–8.2), LV mass ≥ 150 grams (OR = 2.6, 1.7–3.8)], LVEF<50% (OR = 4.6, 1.3–16), and presence of obstructive physiology (OR = 1.8, 1.02–3). The percentage of LV mass involved with LGE increased with higher LV mass, increased septal wall thickness, and decreasing LVEF (Spearman rank = 0.2– 0.4, p<0.0001 for all). Multivariate analysis (adjusting for all morphologic and functional parameters) showed that septal thickness ≥ 16mm (p = 0.0001), LV mass ≥ 150 grams (p = 0.01) and LVEF < 50% (p = 0.02) were independent predictors of LGE on CE-MRI. Conclusions: LGE, as determined by CE-MRI, was seen in over half of the patients with HCM. Decreased LVEF, increased septal thickness, and increased LV mass were associated with the presence of LGE. The clinical and prognostic impact of these morphological features warrants further investigation.


Cardiology ◽  
2015 ◽  
Vol 133 (1) ◽  
pp. 35-43 ◽  
Author(s):  
Xiying Guo ◽  
Chaomei Fan ◽  
Hongyue Wang ◽  
Shihua Zhao ◽  
Fujian Duan ◽  
...  

Objectives: Extreme left ventricular hypertrophy (LVH) is a known risk factor for sudden cardiac death in hypertrophic cardiomyopathy (HCM). Extreme right ventricular hypertrophy (RVH) is rare, and whether it is linked to a poor outcome is unknown. This study was designed to investigate differences between HCM patients with extreme RVH and those with extreme LVH. Methods: Among 2,413 HCM patients, 31 with extreme RVH (maximum right ventricular wall thickness ≥10 mm) and 194 with extreme LVH (maximum left ventricular wall thickness ≥30 mm) were investigated. The main clinical features and natural history were compared between the 2 groups. Results: The prevalence of extreme RVH and extreme LVH was 1.3 and 8.0%, respectively. Patients with extreme RVH tended to be younger and female (p < 0.01). Cardiovascular-related mortality and morbidity within 10 years were significantly greater in the extreme RVH group (p < 0.05). Multivariate analysis demonstrated 3 independent predictors for cardiovascular mortality - extreme RVH, left ventricular end-diastolic dimension ≥50 mm, and age ≤18 years at baseline - and 2 for morbidity - extreme RVH and presyncope. Conclusions: Compared with extreme LVH, extreme RVH was quite uncommon in HCM and had a worse prognosis. A right ventricle examination should be performed in routine HCM evaluation.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J.C Peteiro Vazquez ◽  
J Peteiro ◽  
R Barriales-Villa ◽  
J Larranaga-Moreira ◽  
C Martinez-Veira ◽  
...  

Abstract Background Exercise echocardiography (ExE) may assess left ventricular (LV) systolic and diastolic function, LV outflow tract (LVOT) obstruction and mitral regurgitation (MR). We aimed to assess the feasibility and prognostic value of the assessment of all these issues during exercise in patients with hypertrophic cardiomyopathy (HCM). Methods LV systolic and diastolic function, LVOT gradients, and MR were evaluated during ExE in 285 patients with HCM (age 60±14 years, 168 men) and preserved LVEF (≥50%). Recordings were obtained at rest and peak exercise for regional/global LV systolic function and at rest and within 1.5 min after exercise for the rest of assessments: LVOT gradients, MR and ratio of early LV inflow velocity to early tissue Doppler septal annulus velocity (E/e'). Results Feasibility was 100%, 97%, 98% and 98% for LV systolic function, E/e', LVOT gradients, and MR assessments at exercise, respectively. Thirty-seven patients (13%) had LVOT obstruction at rest, and 76 (27%) developed exercise-induced LVOT obstruction. Mean resting LVEF was 63±3%. New wall motion abnormalities (WMAs) were detected in 38 patients (13%). E/e'&gt;15 was observed in 108 patients at rest (38%) and in 119 at exercise (42%). Corresponding figures for significant MR (moderate or severe) were 20 (7%) and 17 (6%). During follow-up of 3.9±2.5 years, 21 patients had a hard event (cardiac death or transplantation, appropriate discharge of a defibrillator, stroke, myocardial infarction, hospitalization for heart failure), 33 a combined event (hard plus new atrial fibrillation or syncope), and 53 a combined event plus any interventionism. After adjustment, LV wall thickness, resting LVEF, maximal workload in Metabolic Equivalents (METs), and E/e' post-exercise resulted independent predictors of hard events (HR=1.45, 95% CI: 1.21–1.74, p&lt;0.001; HR=0.80, CI: 0.71–0.89, p&lt;0.001; HR=0.73, 95% CI: 0.62–0.86, p&lt;0.001; HR=1.08, 95% CI: 1.02–1.14, p&lt;0.009, respectively). Independent predictors of combined events included also LV wall thickness, resting LVEF, and METs, along with therapy with beta-blockers at the time of ExE (HR=1.29, 95% CI: 1.12–1.50, p=0.001; HR=0.89, CI: 0.81–0.97, p=0.012; HR=0.83, 95% CI: 0.74–0.93, p=0.001; HR=2.51, 95% CI: 1.20–5.25, p=0.015), whereas the model for combined events+any interventionism consisted of beta-blockers, LV wall thickness, LA dimension, METs and new WMAs. (HR=2.15, 95% CI: 1.20–3.86, p=0.01; HR=1.17, 95% CI: 1.03–1.32, p=0.02; HR=1.07, CI: 1.02–1.11, p=0.005; HR=0.90, 95% CI: 0.82–0.98, p=0.01; HR=2.33, 95% CI: 1.17–4.63, p=0.016) Conclusions A comprehensive assessment during ExE is feasible for patients with HCM and provides significant incremental prognostic information Funding Acknowledgement Type of funding source: None


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Hirasawa ◽  
M Izumo ◽  
K Mizukoshi ◽  
T Suzuki ◽  
Y Sato ◽  
...  

Abstract Background Hypertrophic cardiomyopathy (HCM) is a heterogeneous condition that may present crucial complication including life-threatening arrhythmia and sudden cardiac death. However, the risk stratification of HCM without left ventricular outflow tract (LVOT) obstruction had not been fully elucidated. Moreover, although recent studies have revealed the right ventricle (RV) involvement of HCM, the prognostic importance of RV function during exercise is unclear. Purpose To investigate the prognostic significance of RV function in patients with non-obstructive HCM using exercise stress echocardiography (ESE). Methods and results This study conducted on 100 HCM patients (age 62.9±13.6 years, 63% men) with preserved left ventricular ejection fraction who underwent ESE using semi-supine bicycle ergometer. Ten patients with significant LVOT obstruction (≥30mmHg) were excluded and 9 were also excluded because of the inadequate imaging quality or insufficiency of data. Among remaining 81 non-obstructive HCM patients, 9 patients suffered from HCM related cardiac events including cardiac death, unexpected hospitalization, life-threatening arrhythmias, and new-onset of syncope during the mean follow up period of 2.6±1.6 years. A multivariate Cox Hazard analysis revealed that low tricuspid annular plane systolic excursion during exercise (Ex-TAPSE, cut-off: 24mm) was an independent predictor of cardiac events. (hazard ratio: 18.66, 95% confidence interval: 3.66–338.46, P<0.001) The estimated cumulative cardiac event free survival using the Kaplan-Meier method was significantly lower in patients with reduced Ex-TAPSE (<24mm) than those with preserved Ex-TAPSE (Log-rank, P<0.01). K-M curve according to Ex-TAPSE Conclusion Ex-TAPSE had a strong predictive value of clinical outcomes in non-obstructive HCM patients. Right ventricular function during exercise may have crucial role in the risk stratification of non-obstructive HCM.


Author(s):  
Laura Houard ◽  
Sebastian Militaru ◽  
Kaoru Tanaka ◽  
Agnès Pasquet ◽  
David Vancraeynest ◽  
...  

Abstract Aims  Reproducible evaluation of left (LV) and right ventricular (RV) function is crucial for clinical decision-making and risk stratification. We evaluated whether speckle-tracking echocardiography (STE) and cardiac magnetic resonance feature-tracking (cMR-FT) global longitudinal (GLS) and circumferential strains allow better test–retest reproducibility of LV and RV systolic function than conventional cMR and echocardiographic parameters. Methods and results  Thirty healthy volunteers and 20 chronic heart failure patients underwent cMR and STE twice on separate days to evaluate test–retest coefficient of variation (CV), intraclass correlation coefficient (ICC) and estimated sample sizes for significant changes in LV and RV function. Among LV parameters, cMR-left ventricular ejection fraction (LVEF) had the highest reproducibility (CV = 6.7%, ICC = 0.98), significantly better than cMR-FT-GLS (CV = 15.1%, ICC = 0.84), global circumferential strains (CV = 11.5%, ICC = 0.94) and echocardiographic LVEF (CV = 11.3%, ICC = 0.93). STE-LV-GLS (CV = 8.9%, ICC = 0.94) had significantly better reproducibility than cMR-FT-LV-GLS. Among RV parameters, STE-RV-GLS (CV = 7.3%, ICC = 0.93) had significantly better CV than cMR-right ventricular ejection fraction (RVEF) (CV = 13%, ICC = 0.82). cMR-FT-RV-GLS (CV = 43%, ICC = 0.39) performed poorly with significantly lower reproducibility than all other RV parameters. Owing to their superior interstudy reproducibility, cMR-LVEF (n = 12), cMR-RVEF (n = 41), STE-LV-GLS and STE-RV-GLS (both n = 14) were the parameters allowing the lowest calculated sample sizes to detect 10% change in LV or RV systolic function. Conclusion  STE-LV-GLS and STE-RV-GLS showed higher test–retest reliability than other echocardiographic measurements of LV and RV function. They also allowed smaller calculated sample sizes, supporting the use of STE-LV and RV-GLS for longitudinal follow-up of LV and RV function.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Kovacs ◽  
Y Nabeshima ◽  
B Lakatos ◽  
Y Nagata ◽  
M Tokodi ◽  
...  

Abstract Three main mechanisms contribute to right ventricular (RV) pump function: (1) shortening of the longitudinal axis with traction of the tricuspid annulus towards the apex; (2) inward (radial) movement of the RV free wall, which is often referred as the “bellows effect”; and (3) bulging of the interventricular septum into the RV during the left ventricular contraction and stretching of the free wall over the septum (causing shortening in the anteroposterior direction). Despite the established clinical value of the functional changes among the aforementioned components, their relative importance remains to be quantified during physiological conditions. Accordingly, the aim of our study was to evaluate the relative contribution of the different RV motion directions to global RV function on both global and segmental level in a large set of healthy individuals. We have recruited 231 healthy volunteers (44% female, with a wide and balanced age range from 8 to 81 years) from two experienced centers performing transthoracic 3D echocardiography (3DE) on a routine basis. 3DE datasets focused on the RV were obtained using multi-beat acquisition. We determined RV volumes and subsequent ejection fraction (RVEF) by dedicated software. Using the ReVISION method, we have decomposed the motion of the RV to determine longitudinal (LEF), radial (REF) and anteroposterior ejection fraction (AEF). Their ratio to RVEF quantifies the relative contribution of the given component to global RV function. Moreover, regional subvolumes were also analyzed in a 15-segment model. Mean value of RVEF was significantly higher in female subjects compared to male subjects (60±7 vs. 56±7%, p<0.001). The relative contributions of LEF and REF to RVEF were comparable, while the contribution of AEF was significantly lower (LEF/RVEF vs. REF/RVEF vs. AEF/RVEF: 0.48±0.08 vs. 0.49±0.07 vs. 0.39±0.11, p<0.001) in the pooled population as well as in the genders separately. In line with higher RVEF found in women, female gender was associated with a higher longitudinal and radial contribution compared to males, however, AEF was similar (women vs. men; LEF/RVEF: 0.49±0.08 vs. 0.47±0.07, p<0.05; REF/RVEF: 0.50±0.07 vs. 0.48±0.06, p<0.01; AEF/RVEF: 0.38±0.12 vs. 0.40±0.10, p=NS). Interestingly, AEF/RVEF showed a significant deterioration with age (r=−0.354, p<0.001), while age-dependency of the longitudinal and radial contributions were not observed concerning both genders. An age-related decrease could be demonstrated by the volume fractions of the 5 septal volumetric segments (r=−0.229, p<0.001). Motion decomposition and the 15 segments In physiological conditions, the relative importance of longitudinal and radial wall motions is similar in determining global RV function. Aging accompanied by a decrease in anteroposterior shortening, which may point to a deteriorating systolic LV-RV interaction. Our results may facilitate further research concerning the alterations of RV mechanical pattern in various disease states.


Sign in / Sign up

Export Citation Format

Share Document