Clinical Investigation
Cardiac Structure and Function in Fetuses, Neonates, and Infants
Right Ventricular Mechanics in the Fetus with Hypoplastic Left Heart Syndrome

https://doi.org/10.1016/j.echo.2013.02.001Get rights and content

Background

Right ventricular mechanics influence outcomes in patients with hypoplastic left heart syndrome (HLHS). The aim of this study was to determine whether differences in right ventricular performance have their origins in fetal life and if the architectural character of the hypoplastic left ventricle affects right ventricular mechanics.

Methods

The first complete fetal echocardiograms after 17 weeks' gestation were reviewed in 84 fetuses with HLHS and in 115 gestational age-matched normal controls. Inflow, outflow, and myocardial tissue Doppler velocities were measured. E/A and E/e′ ratios and right ventricular myocardial performance index were calculated.

Results

In fetuses with HLHS, there were lower tricuspid E/A ratios (mean, 0.6 ± 0.1 vs 0.7 ± 0.1; P < .001), higher E/e′ ratios (mean, 8.1 ± 2.6 vs 7.0 ± 1.3; P = .006), and higher right ventricular myocardial performance indices (mean, 0.47 ± 0.14 vs 0.40 ± 0.10; P < .001) compared with controls. Among fetuses with HLHS grouped according to left ventricular architecture, those with left ventricular endocardial fibroelastosis had the most striking differences in right ventricular mechanics.

Conclusions

Right ventricular mechanics are different from normal in fetuses with HLHS and are influenced by the presence of left ventricular endocardial fibroelastosis. These differences precede the imposition of undue loading conditions as a consequence of surgical palliation and may offer clues to the development of later right ventricular failure.

Section snippets

Methods

This cross-sectional retrospective review used the echocardiographic imaging database of the Fetal Heart Program of the Cardiac Center at the Children's Hospital of Philadelphia. Fetal echocardiograms from pregnant women referred for fetal echocardiography between January 2006 and December 2008 were reviewed. Echocardiograms demonstrating diagnoses of HLHS with severe mitral stenosis or atresia and/or severe aortic stenosis or atresia, double-outlet right ventricle, and unbalanced

Results

One hundred two consecutive fetuses with HLHS and HLHS variants presenting for fetal echocardiography between January 2006 and December 2008 were reviewed. Seven of these fetuses had more than mild atrioventricular valve regurgitation, six had restriction across the atrial septum, three had complete heart block, one had hydrops fetalis, and one did not have adequate imaging. All of these fetuses were excluded from analysis. Eighty-four fetuses fulfilled our inclusion criteria, and the first

Discussion

This study demonstrates that in patients with HLHS, RV mechanics differ from normal as early as fetal life, even in the presence of qualitatively normal systolic shortening. First, fetuses with HLHS have enhanced RV noncompliance, reflected by altered tricuspid valve blood pool Doppler velocities. In fetal life, tricuspid valve inflow Doppler patterns normally demonstrate a prominent A wave and a relatively low E/A ratio, reflecting a degree of ventricular impaired ventricular filling.13, 14 In

References (36)

Cited by (29)

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    The septum is often fibrotic and less contractile and abnormalities of septal movement impact global systemic ventricular function [39]. The hypoplastic ventricle in hypoplastic left heart syndrome has been shown to negatively affect diastolic performance of the systemic ventricle [40–42], while hypoplastic ventricle morphology and especially hypertrophy of the interventricular septum are associated with worse outcome [43,44]. The hypoplastic ventricle is often dyskinetic and by only measuring strain in the dominant ventricle, the negative effect of the hypoplastic ventricle on ventricular performance is ignored.

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    Our work contributes to that of multiple investigators who have shown the presence of abnormal cardiac mechanics and time intervals in fetuses with HLHS.4,5,7 Natarajan et al.5 found that fetuses with morphologic LVs and endocardial fibroelastosis had lower E/A ratios, higher E/e′ ratios, and higher Tei indices measured by tissue Doppler than controls. Similarly, Szwast et al.7 showed that fetuses with HLHS had preserved systolic performance and mainly diastolic dysfunction.

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