Survey of how pediatric cardiologists noninvasively evaluate patients with hypoplastic left heart syndrome.

BACKGROUND: The evaluation of right ventricular (RV) function is important in patients with hypoplastic left heart syndrome (HLHS). Echocardiographic qualitative grading has been the prevalent method used in the past, but newer technologies allowing for quantitative assessment of RV function may have changed this fact. The goal of this study was to determine the current routine noninvasive evaluation of patients with HLHS and what, if any, methods are used to assess systolic and diastolic function in this population. METHODS: Web-based survey was conducted using various listservs. Timing of echocardiograms between surgical stages was assessed. Methods of assessing systolic and diastolic function were evaluated. RESULTS: Two hundred seventy-seven physicians who averaged 12.8 ± 9.6 years removed from training responded. Largest percentage of respondents was echocardiographers (44.2%) in a university-based practice (73.3%) from North America (91.7%). There were 54.3% of respondents who performed echocardiograms monthly between stages I and II, 48.8% who performed echocardiograms every 6 months between stages II and III, and 67.0% who performed echocardiograms annually after stage III procedure. The main method for systolic grading was qualitative grading (95.5%) and for diastolic grading were tricuspid blood inflow velocities (56.8%). Qualitative grading was considered the method of choice for systolic grading for 38.8% of respondents and tissue Doppler velocities was the method of choice for diastolic grading for 35.3% of respondents. There were 4.0% of respondents who routinely perform a cardiac magnetic resonance imaging (cMRI) between stages I and II, 8.0% between stages II and III, and 24.2% after stage III procedure. CONCLUSION: Variability in the noninvasive assessment of the RV in patients with HLHS continues to exist. Qualitative RV systolic assessment was still the predominant method used to assess function despite newer imaging techniques to allow for quantification. Future studies are needed to determine which values are most useful in reviewing function in this complex patient population.

Diastolic Dyssynchrony Differences in Patients with Single Right Ventricles vs. Control Patients.

AIMS: Diastolic dyssynchrony is increasingly being recognized as another marker for possible adverse cardiac events. Minimal data exist in hypoplastic left heart syndrome (HLHS) patients. The goal of this study was to determine if there were differences in diastolic dyssynchrony in patients with HLHS vs. control patients. METHODS/RESULTS: Tissue Doppler imaging (TDI) and strain rate (SR) analysis of the right ventricle in HLHS and control patients were performed. Time interval from onset of QRS complex on electrocardiography to peak TDI e' wave was obtained. Differences in intervals were calculated: QRSe'(RV) - QRSe'(IVS) and QRSe'(RV) - QRSe'LV). Time interval from onset of QRS to peak strain rate early diastolic wave (SRe) was obtained for the six-segment model RV. Standard deviation of the six SRe time intervals was calculated. t-tests were performed to determine if differences were present between groups. Sixty patients were studied (35 HLHS, 25 control). There were no significant differences between HLHS and control patients in age (6.5 ± 3.2 years vs. 6.1 ± 2.6 years) or heart rate (91 ± 22 bpm vs. 91 ± 13 bpm), respectively. There were no significant differences between HLHS and control patients in QRSe'(RV) - QRSe'(IVS) (19.9 ± 15.4 ms vs. 23.3 ± 13.6 ms) and QRSe'(RV) - QRSe'(LV) (20.7 ± 13.8 vs. 22.8 ± 14.6 ms), respectively. There was a significant difference in SRe standard deviation between the HLHS and control patients (25.3 ± 12.4 ms vs. 15.5 ± 6.9 ms, P = .0007), respectively. CONCLUSION: Patients with HLHS had increased diastolic dyssynchrony compared with control patients as measured via deformation analysis. Future studies are needed to determine the significance of these findings.

Evaluation of the fetal heart at 14 to 18 weeks' gestation in fetuses with a screening nuchal translucency greater than or equal to the 95th percentile.

OBJECTIVES: The purpose of this study was to determine whether normal fetal cardiac anatomy could be successfully demonstrated and congenital heart disease detected transabdominally at 14 to 18 weeks' gestation in fetuses with a nuchal translucency greater than or equal to the 95th percentile. METHODS: In this retrospective chart review, grayscale images, Z scores, and Doppler evaluations, including pulsed, color, and spectral Doppler imaging, were reviewed to determine whether fetal heart evaluation findings at 14 to 18 weeks' gestation were normal or abnormal. RESULTS: Normal cardiac anatomy was successfully evaluated in 32 of 33 normal cases; only an aortic arch and a ductal arch were not successfully visualized in 1 case. Major congenital heart disease was detected prenatally in 4 abnormal cases. CONCLUSIONS: The fetal heart can be successfully evaluated at an earlier gestational age but may be dependent on the skill of the sonographer and reading physician. Maternal decisions can be made earlier in gestation, before the pregnancy is obvious, and can allow planning for a pregnancy that will need to be delivered at a medical center that has a level 3 nursery.