Right Ventricular Systolic Function Parameters in Hypoplastic Left Heart Syndrome

Abstract

Assessment of the systolic function of the right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS) is important. The asymmetric shape and heavy trabeculations make accurate assessment of RV systolic function challenging. Novel measures of RV function could be helpful in distinguishing reduced versus preserved function in HLHS and may also be worse in HLHS with preserved function compared to normal controls. These novel methods offer promise, but research and clinical applicability is hindered as no cut-off values for normal function have been established. We performed a retrospective comparison of functional assessments from echocardiograms of HLHS patients with preserved and reduced RV function along with a control group of normal patients. Measures of function included fractional area change (FAC), tissue motion annular displacement of the tricuspid (TMAD-TV) and pulmonary valves (TMAD-PV), myocardial performance index (MPI), tricuspid tissue Doppler S' velocity, and RV global longitudinal strain (RVGLS). Comparisons were made between three groups: normal patients, HLHS with preserved function, and HLHS with reduced function defined as FAC < 35%. FAC was chosen as the reference as it is a historical standard. 41 HLHS patients were studied. Of these patients, 20 had HLHS with reduced function, and 21 had preserved function. They were compared with 27 age-matched, normal, controls. Comparison between HLHS and normal controls: in HLHS with preserved RV systolic function, compared to normal controls, tissue Doppler S', MPI, and TMAD-TV were all abnormal (all p < 0.05). RVGLS was not statistically different (20.5 ± 3.6% for normal vs. 17.9 ± 2.6% for HLHS with preserved function). TMAD-PV was similar between groups (16.1 ± 4.6% vs. 16.7 ± 5.1%). All measures were significantly worse (all p < 0.05) in the HLHS with reduced function group compared to normal controls. Comparison between HLHS preserved vs reduced function: in HLHS with reduced function defined by FAC < 35%, all measures were significantly worse compared to HLHS with preserved function (all p < 0.05). The cut-off values that correspond to a FAC of > 35% were 14.5% for TMAD-TV and 16% for RVGLS. All measures except RVGLS and TMAD-PV estimated worse function than controls even for HLHS with preserved function. Each of the functional measures was able to identify preserved vs reduced function in HLHS with FAC as the reference standard. Cut-off values between preserved and reduced function in HLHS were estimated for TMAD-TV and RVGLS based on a relatively small cohort. These cut-off values will aid in the research design of future studies.

Longitudinal changes in echocardiographic measures of ventricular function after Fontan operation

“…This retrospective observational study of patients with single ventricle heart disease after Fontan operation demonstrates that markers of ventricular performance are decreased compared to known normative values of healthy patients and that global longitudinal strain rate and global circumferential strain rate worsen over the decade following the Fontan operation, even without an overt change in the qualitative assessment of ventricular function 9,16,18–20 . Not surprisingly, the aggregate mean values for most functional parameters were worse than what would be expected for a healthy age‐matched population without heart disease 9,11,15,18,19,21 . While there was a marginal improvement in the s/d ratio improved over time, this change was most likely related to the known decrease in heart rate as these children get older, and not related to a true change in ventricular function 17 .…”

Atrial and Ventricular Deformation Analysis in Normal Fetal Hearts Using Two-Dimensional Speckle Tracking Echocardiography

“…Furthermore, these data illustrate the potential of atrial deformation parameters, which may act as predictors for the necessity of therapeutic interventions in adult cardiology [20]. Besides already investigated potential function of 2D-STE to distinguish between healthy fetuses and fetuses with congenital heart diseases (CHD), the function of myocardial strain as a predictor for the success of cardiosurgical interventions may be a potential usage for 2D-STE [21,22]. To enable future research in these areas, the aim of our study was to evaluate atrial and ventricular MF using 2D-STE in normal fetal cardiac 4-chamber view (4CV), which is a common standard in basic sonographic screening, and test reproducibility of our collected deformation values.…”

New Aspects in the Diagnosis and Therapy of Fetal Hypoplastic Left Heart Syndrome

“…In contrast, a recently published work showed a significant association between RV function before and after stage I of the Norwood operation. Zaidi et al observed a significant reduction in echocardiographic functional parameters after surgery as well as a correlation with the length of hospital stay, the need for extracorporeal membrane oxygenation, and the mortality rate of HLHS children 37 , 38 .…”