Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly

Prota, Costantina; Salvo, Giovanni Di; Sabatino, Jolanda; Josen, M; Paredes, Josefa; Sirico, Domenico; Pernia, M Uy; Hoschtitzky, Andreas; Michielon, Guido; Citro, Rodolfo; Fraisse, Alain; Ghez, Olivier

doi:10.1016/j.ijcard.2018.10.046

Cited by 23 publications

(16 citation statements)

References 42 publications

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“…The prognosis of AL patients is often a paramount clinical concern. Some studies have demonstrated that NT-proBNP, troponin T, RV dysfunction and RV-LGE have prognostic value in AL patients (7,(30)(31)(32)(33). The prognostic value of CMR tissue tracking in AL patients has not yet been analysed.…”

Section: Discussionmentioning

confidence: 99%

The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients

Fu¹,

Guo²,

Yang³

et al. 2020

Cardiovasc Diagn Ther

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Background: Early detection of right ventricular (RV) dysfunction is vital for determining the prognosis of light-chain amyloidosis (AL) patients. While few studies focused on RV deformation due to the limitation of research methods. The aim of this study was to determine the prognostic significance of RV myocardial strain in AL patients assessed by cardiac magnetic resonance (CMR) tissue tracking. Methods: Sixty-four AL patients (28 females and 36 males, mean age 58±12.8 years old; range 25-81 years old) were enrolled from 1 October 2014 through 31 March 2017 and compared with 20 age-and sex-matched controls. Fifty-one AL patients met the criteria for cardiac amyloidosis (CA). Deformation parameters of both RV and left ventricle (LV) were measured by the CMR tissue tracking technique including myocardial global radial peak strain (GRPS), global circumferential peak strain (GCPS), and global longitudinal peak strain (GLPS). The follow-up time was 20 months or until the occurrence of death. Results: Thirty-two (50%) had preserved RV ejection fraction (RVEF ≥45%). AL patients had significantly lower RV-GRPS (20.3±2.12 vs. 31.31±7.61), GCPS (−2.12±0.88 vs. −13.71±2.53), and GLPS (−5.33±0.64 vs. −14.239±2.99) than controls even RVEF remain preserved (all P<0.001). Compared with controls and patients without CA, RV-GRPS (12.26±1.26 vs. 29.72±3.54, P<0.001) and RV-GLPS (−3.78±2.25 vs. −5.66±2.08, P<0.05) were significantly lower in patients with CA. Cox multivariate analyses demonstrated that RV-GRPS [hazard ratio (HR) =0.93, 95% CI: 0.88-0.98, P=0.007] and Mayo stage were (HR =3.11, 95% CI: 1.30-7.41, P=0.01) predictors of mortality in AL patients.Conclusions: CMR tissue tracking is a feasible and highly reproducible technique for the analysis of RV deformation and could aid in the early diagnosis of RV involvement in AL patients. RV-GRPS of RV strain and Mayo stage provides prognostic information about mortality in AL patients.

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Section: Discussionmentioning

confidence: 99%

The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients

Fu¹,

Guo²,

Yang³

et al. 2020

Cardiovasc Diagn Ther

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“…Longitudinal strain (LS) analysis of the left ventricle, through 2D STE analysis, was performed offline using GE EchoPac Software (GE Healthcare, Chicago, IL), as previously described [ 17 ]. Briefly, the best apical four-, two- and three-chamber views to visualize the left ventricle segments were selected.…”

Section: Methodsmentioning

confidence: 99%

Early Echocardiographic and Cardiac MRI Findings in Multisystem Inflammatory Syndrome in Children

Sirico

Basso

Reffo

et al. 2021

JCM

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Multisystem Inflammatory Syndrome in Children (MIS-C) is a known severe condition affecting children previously exposed to SARS-CoV-2. The aim of our study was to describe the early cardiac abnormalities in patients with MIS-C, evaluated by speckle tracking echocardiography (STE) and cardiac MRI (CMR). Clinical, laboratory and microbiological data were measured for all patients. All children underwent standard transthoracic echocardiography, STE with analysis of left ventricle global longitudinal strain (GLS). Seventeen (75%) of the children were evaluated with CMR. Twenty-three patients (13M, 10F) were recruited, mean age was 8.1 ± 4 years. Cardiovascular symptoms were present in 10 (43.5%). Nine children (39.1%) shared Kawasaki Disease-like symptoms. Four patients (17.4%) needed ICU admission. In-hospital survival was 100%. TnI was elevated in 15 (65.2%) and BNP in 20 (86.9%) patients. The median time to STE evaluation was 8 days and to CMR was 18 days after fever onset. Mean LVEF was 59 ± 10%. Coronary dilation was observed in six (26.1%) patients. STE showed a reduced mean LVGLS (−17 ± 4.3%). LGE with a non-ischemic pattern was evident in six out of seventeen patients (35.2%). The elevation of myocardial necrosis markers, the reduction of LVGLS and the presence of LGE on CMR in about a quarter of MIS-C patients supports the hypothesis of a post-viral immune-mediated myocarditis-like pathogenesis.

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“…Nonetheless, each patient has its own characteristics, mainly manifested in the differences among the tricuspid septal lobe, posterior lobe lesions, and occasionally anterior lobular abnormalities that shift down or block the right ventricular out ow tract. Additionally, the right ventricle looks abnormal, the right ventricle cavity below the tricuspid valve is signi cantly reduced, and the trabecular part also becomes smaller [6][7] . The funnel part can be narrowed by residual valve tissue or abnormal muscle bundles or ber bundles 8 .…”

Section: Discussionmentioning

confidence: 99%

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

et al. 2020

Preprint

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Objective: The aim of this study was to describe the fetal echocardiographic features of Ebstein anomaly (EA) and also to evaluate the prognosis of those fetuses.Methods: We retrospectively reviewed the clinical records of 53 fetuses with EA who were enrolled from January 2011 to December 2018 in Beijing Anzhen Hospital, and analyzed the ultrasonic features and prognosis of the fetuses. Results: In this single-institutional study, 53 fetuses were screened after 23~39 weeks of gestation with EA. In these fetuses, patients with complications accounted for a greater percentage (69.81%). Ultrasound imaging showed that the posterior lobe shifted down further than the septal leaflets, and the anterior lobe changed less than 50%. Severe tricuspid valve (TV) regurgitation showed the highest proportion in these fetuses (56.60%). Among the enrolled fetuses, 20 cases were classified into grade Ⅱ. 40 cases (75.47%) were inducted, and 13 cases (24.5%) were selected for continued pregnancy. However, only one of the fetuses developed till successful delivery. Conclusion: Echocardiography has become an important diagnostic tool in identifying the fetuses with EA, a rare and heterogeneous congenital heart disorder. The prognosis of the fetuses with EA is relatively poor. Therefore, understanding the echocardiographic characteristics and the prognosis of the fetuses with EA is of great value for pregnancy consultations during the perinatal period.

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Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly

Cited by 23 publications

References 42 publications

The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients

The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients

Early Echocardiographic and Cardiac MRI Findings in Multisystem Inflammatory Syndrome in Children

Echocardiographic Characteristics and Prognosis of the Fetuses with Ebstein Anomaly in Pregnancy

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