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Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report.

ABSTRACT: Background:Ebstein's anomaly (EA) is mainly thought of as a right heart condition, however, congenital left-sided lesions can co-exist. Therefore, it is paramount to include the left side of the heart as part of a routine investigation in these patients. We present a 57-year-old symptomatic patient with EA and progressive tricuspid regurgitation (TR) associated with acquired left ventricular outflow obstruction (LVOTO). Case summary:A 57-year-old women, known to have severe EA presented with shortness of breath and chest pain on exertion secondary to progression of the tricuspid valve regurgitation and right ventricle dilatation leading to a dynamic compression of the left outflow tract requiring surgical intervention. Discussion:Left ventricular obstruction secondary to severe TR and dilation of the right ventricle can present and remain silent at rest but becoming significant on exertion. Therefore, we recommend that all patients with EA and significant TR undergo exercise echocardiography at regular intervals to specifically look for acquired dynamic LVOTO.

SUBMITTER: Ordonez MV

PROVIDER: S-EPMC7501933 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature

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Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report.

Ordoñez Maria Victoria MV Bedair Radwa R Curtis Stephanie L SL

European heart journal. Case reports 20200629 4

<h4>Background</h4>Ebstein's anomaly (EA) is mainly thought of as a right heart condition, however, congenital left-sided lesions can co-exist. Therefore, it is paramount to include the left side of the heart as part of a routine investigation in these patients. We present a 57-year-old symptomatic patient with EA and progressive tricuspid regurgitation (TR) associated with acquired left ventricular outflow obstruction (LVOTO).<h4>Case summary</h4>A 57-year-old women, known to have severe EA pre ...[more]

PMID: 32974476

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Project description:BackgroundDisease progression and heart failure development in Ebstein's Anomaly (EA) of the tricuspid valve is characterized by both right and left ventricular (LV) deterioration. The mechanisms underlying LV dysfunction and their role in heart failure development are incompletely understood. We hypothesized that LV dyssynchrony and impaired torsion and recoil mechanics induced by paradoxical movement of the basal septum may play a role in heart failure development.Methods31 EA patients and 31 matched controls underwent prospective cardiovascular magnetic resonance (CMR). CMR feature tracking (CMR-FT) was performed on apical, midventricular and basal short-axis and 4D-volume analysis was performed using three long-axis views and a short axis cine stack employing dedicated software. Circumferential uniformity ratio estimates (CURE) time-to-peak-based circumferential systolic dyssynchrony index (C-SDI), 4D volume analysis derived SDI (4D-SDI), torsion (Tor) and systolic (sysTR) and diastolic torsion rate (diasTR) were calculated for the LV. QRS duration, brain natriuretic peptide, NYHA and Total R/L-Volume Index (R/L Index) were obtained.ResultsEA patients (31.5 years; controls 31.4 years) had significantly longer QRS duration (123.35 ms ± 26.36 vs. 97.33 ms ± 11.89 p < 0.01) and showed more LV dyssynchrony (4D-SDI 7.60% ± 4.58 vs. 2.54% ± 0.62, p < 0.001; CURE 0.77 ± 0.05 vs. 0.86 ± 0.03, p < 0.001; C-SDI 7.70 ± 3.38 vs. 3.80 ± 0.91, p = 0.001). There were significant associations of LV dyssynchrony with heart failure parameters and QRS duration. Although torsion and recoil mechanics did not differ significantly (p > 0.05) there was an association of torsion and recoil mechanics with dyssynchrony parameters CURE (sysTR r = -0.426; p = 0.017, diasTR r = 0.419; p = 0.019), 4D-SDI (sysTR r = 0.383; p = 0.044) and C-SDI (diasTR r = -0.364; p = 0.044).ConclusionsEA is characterized by LV intra-ventricular dyssynchrony, which is associated with heart failure and disease severity parameters. Markers of dyssynchrony can easily be quantified from CMR-FT, and may have a role in the assessment of altered cardiac function, carrying potential management implications for EA patients.

Dataset Information

Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report.

Publications

Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report.

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