The Effect of Left Ventricular Size on Right Ventricular Hemodynamics in Pediatric Survivors with Hypoplastic Left Heart Syndrome

Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic left heart syndrome after Fontan surgery

“…In our patients, there was no difference between groups. RV FAC was at the lower end of the spectrum reported as normal in the literature for the subpulmonary RV [14] but in the same range as reported for children with HLHS after Fontan [10]. RVs were larger in the MA/AA group in both end-diastole and end-systole when indexed to body surface area, however.…”

“…Much attention has recently focussed on the subgroup of patients with HLHS that has a significant LV, implying disadvantageous properties of this "piggyback" ventricle on ventricular function and outcome [9][10][11].…”

Left ventricular morphology influences mortality after the Norwood operation

“…Of note, LV morphology as measured in this study was unrelated to mitral and/or aortic valve anatomy (data not shown). Recent work by Wisler showed no relationship between LV size and RV function in a cohort of patients with HLHS who survived stage I Norwood palliation, but IVS was not examined, nor was the relationship of LV size to either mortality or transplantation 17. This study also modelled echocardiographic indices of LV size, IVS and RV function as independent predictors of outcome.…”

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

“…Miller et al [20] used Velocity Vector Imaging (VVI) technology to study the regional and global myocardial motion of right ventricle in fetuses with HLHS, and concluded that the left ventricular volume and absence of left ventricular cavity in fetuses with HLHS did not affect the right ventricular myocardial motion. However, whether the size of underdeveloped left ventricle could affect the right ventricular function in children with HLHS is still controversial [21, 22]. In a recent study by Wisler et al [21], the size of the left ventricle in patients with HLHS had an impact on the right ventricular function only before Fontan procedure; and that it did not affect the right ventricular function either before Norwood and Glenn procedure, or after Fontan procedure.…”

“…However, whether the size of underdeveloped left ventricle could affect the right ventricular function in children with HLHS is still controversial [21, 22]. In a recent study by Wisler et al [21], the size of the left ventricle in patients with HLHS had an impact on the right ventricular function only before Fontan procedure; and that it did not affect the right ventricular function either before Norwood and Glenn procedure, or after Fontan procedure. Considering the high risk of mortality, the high cost of surgical treatment and the unfavorable post-surgical prognosis, all parents in our study opted for termination of pregnancy after counseling.…”