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Evaluation of right ventricular function in fetal hypoplastic left heart syndrome using spatio-temporal image correlation (STIC).

ABSTRACT: Postnatal outcome of fetuses with hypoplastic left heart syndrome (HLHS) is mainly determined by right ventricular function. In the present study we used spatio-temporal image correlation (STIC) to assess right ventricular function of fetuses with HLHS.Three-dimensional ultrasound with STIC technique was used to acquire heart images from fetuses that had HLHS and the normal controls, between 24(+0) and 37(+6) weeks of gestation. Right ventricular end-diastolic volume (RVEDV) and right ventricular end-systolic volume (RVESV) were determined using the virtual organ computer-aided analysis software, and the parameters of right ventricular function were calculated.Both RVEDV and RVESV were found to be significantly higher in fetuses with HLHS as compared to that in normal controls (P < 0.001). There were no significant differences in the parameters between fetuses with and without a visible left ventricular cavity (P > 0.05). Compared to fetuses with HLHS plus mild tricuspid regurgitation (TR), fetuses with HLHS plus severe TR exhibited lower right ventricular stroke volume (RVSV), right ventricular cardiac output (RVCO) and standardized RVCO (P < 0.05). The right ventricular ejection fraction (RVEF) was significantly lower in HLHS fetuses that had severe TR (P < 0.001).As the right ventricle is solely responsible for maintenance of circulation, the right ventricular systolic function undergoes compensatory enhancement in fetuses with HLHS and mild TR, compared to that in normal controls. Size of the left ventricle does not significantly affect the right ventricular function in HLHS. However, right ventricular systolic function may be impaired prenatally in HLHS fetuses that have severe TR.

SUBMITTER: Zhang J

PROVIDER: S-EPMC4827237 | biostudies-other | 2016 Apr

REPOSITORIES: biostudies-other

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Project description:BackgroundThe geometry and heterogeneity of the right ventricle in hypoplastic left heart syndrome makes objective echocardiographic assessment of systolic function challenging. Consequently, subjective echocardiographic assessment of right ventricular (RV) function is still routinely undertaken. The aims of this study were to compare this with magnetic resonance imaging (MRI), investigate the impact of experience and training on the accuracy of subjective assessment, and critically analyze the role of echocardiography to detect impaired systolic function.MethodsA retrospective analysis of prospectively acquired data was performed. Children with hypoplastic left heart syndrome underwent routine preoperative cardiac MRI and echocardiography under the same general anesthetic. Echocardiograms were reviewed, and members of the congenital heart disease team with differing echocardiography experience subjectively graded RV systolic function (good, moderate, or poor). This was compared with MRI-derived ejection fraction.ResultsTwenty-eight patients at different palliative stages were included. Twenty-eight observers were divided into five experience categories (congenital heart disease junior trainees to attending cardiologists). Median agreement was 47.6% (range, 31.4%-58.2%), with the lowest agreement among junior trainees and the highest among attending cardiologists. When used as a screening test for poor RV systolic function, the median sensitivity of echocardiography was 0.89 (range, 0.86-0.96), and median specificity was 0.45 (range, 0.26-0.55). The highest sensitivity was observed among junior trainees but with the lowest specificity. The highest specificity was observed among attending cardiologists (0.55).ConclusionsAgreement between echocardiographic and MRI RV ejection fraction improves with experience but remains suboptimal. When used as a screening test for poor RV function, echocardiography is sensitive, but specificity is heavily influenced by operator experience.

Project description:IntroductionHypoplastic left heart syndrome (HLHS) is a congenital condition with an underdeveloped left ventricle (LV) that provides inadequate systemic blood flow postnatally. The development of HLHS is postulated to be due to altered biomechanical stimuli during gestation. Predicting LV size at birth using mid-gestation fetal echocardiography is a clinical challenge critical to prognostic counseling.HypothesisWe hypothesized that decreased ventricular filling in utero due to mitral stenosis may reduce LV growth in the fetal heart via mechanical growth signaling.MethodsWe developed a novel finite element model of the human fetal heart in which cardiac myocyte growth rates are a function of fiber and cross-fiber strains, which is affected by altered ventricular filling, to simulate alterations in LV growth and remodeling. Model results were tested with echocardiogram measurements from normal and HLHS fetal hearts.ResultsA strain-based fetal growth model with a normal 22-week ventricular filling (1.04 mL) was able to replicate published measurements of changes between mid-gestation to birth of mean LV end-diastolic volume (EDV) (1.1-8.3 mL) and dimensions (long-axis, 18-35 mm; short-axis, 9-18 mm) within 15% root mean squared deviation error. By decreasing volumetric load (-25%) at mid-gestation in the model, which emulates mitral stenosis in utero, a 65% reduction in LV EDV and a 46% reduction in LV wall volume were predicted at birth, similar to observations in HLHS patients. In retrospective blinded case studies for HLHS, using mid-gestation echocardiographic data, the model predicted a borderline and severe hypoplastic LV, consistent with the patients' late-gestation data in both cases. Notably, the model prediction was validated by testing for changes in LV shape in the model against clinical data for each HLHS case study.ConclusionReduced ventricular filling and altered shape may lead to reduced LV growth and a hypoplastic phenotype by reducing myocardial strains that serve as a myocyte growth stimulus. The human fetal growth model presented here may lead to a clinical tool that can help predict LV size and shape at birth based on mid-gestation LV echocardiographic measurements.

Project description:Background As right ventricular dysfunction is a major cause of adverse outcome in patients with hypoplastic left heart syndrome, the aim was to assess right ventricular function and deformation after Fontan completion by performing 2-dimensional cardiovascular magnetic resonance feature tracking in serial cardiovascular magnetic resonance studies. Methods and Results Cardiovascular magnetic resonance examinations of 108 patients with hypoplastic left heart syndrome (female: 31) were analyzed. Short-axis cine images were used for right ventricular volumetry. Two-dimensional cardiovascular magnetic resonance feature tracking was performed using long-axis and short-axis cine images to measure myocardial global longitudinal, circumferential, and radial strain. All patients had at least 2 cardiovascular magnetic resonance examinations after Fontan completion and 41 patients had 3 examinations. Global strain values and right ventricular ejection fraction decreased from the first to the third examination with a significant decline in global longitudinal strain from the first examination to the second examination (median, first, and third quartile: -18.8%, [-20.5;-16.5] versus -16.9%, [-19.3;-14.7]) and from the first to the third examination in 41 patients (-18.6%, [-20.9;-15.7] versus -15.8%, [-18.7;-12.6]; P-values <0.004). Right ventricular ejection fraction decreased significantly from the first to the third examination (55.4%, [49.8;59.3] versus 50.2%, [45.0;55.9]; P<0.002) and from the second to the third examination (53.8%, [47.2;58.7] versus 50.2%, [45.0;55.9]; P<0.0002). Conclusions Serial assessment of cardiovascular magnetic resonance studies in patients with hypoplastic left heart syndrome after Fontan completion demonstrates a significant reduction in global strain values and right ventricular ejection fraction at follow-up. The significant reduction in global longitudinal strain between the first 2 examinations with non-significant changes in right ventricular ejection fraction suggest that global longitudinal strain measured by 2-dimensional cardiovascular magnetic resonance feature tracking might be a superior technique for the detection of changes in myocardial function.

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Evaluation of right ventricular function in fetal hypoplastic left heart syndrome using spatio-temporal image correlation (STIC).

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