Association of Right Ventricular Dysfunction with Risk of Neurodevelopmental Impairment in Infants with Pulmonary Hypertension

Abstract

(1) Background: Pulmonary hypertension (PH) increases pulmonary vascular resistance and right ventricular (RV) afterload. Assessment of RV systolic function in PH using RV fractional area change (RV FAC) as a marker directly correlates with mortality and the need for extracorporeal membrane oxygenation (ECMO). However, few studies have assessed neurodevelopmental outcomes. We hypothesize that cardiac RV systolic dysfunction with lower RV FAC is associated with worse neurodevelopmental impairment (NI). (2) Methods: Retrospective study of 42 subjects with PH to evaluate neurodevelopmental outcomes in the first two years of life based on (i) subjective assessment of RV systolic function and (ii) RV FAC, a specific echocardiographic marker for RV function. (3) Results: Subjects from the initial study cohort (n = 135) with PH who had long-term follow-up were divided into RV dysfunction (study, n = 20) and non-RV dysfunction (control, n = 22) groups. RV FAC in the study vs. control group (0.18 vs. 0.25) was lower (p = 0.00017). There was no statistically significant difference in NI either with RV dysfunction or lower RV FAC. Although not significant, RV dysfunction was associated with longer mean duration of mechanical ventilation, time on ECMO, and length of stay. In the initial cohort (135), mortality was 16.3% and the percentage of NI was 62%. (4) Conclusions: Neonatal pulmonary hypertension is associated with a high degree of neurodevelopment impairment. Early RV systolic dysfunction, as identified by RV FAC, was not an optimal predictive biomarker for infants with PH and neurodevelopmental impairment.

Keywords: neurodevelopmental impairment (NI); pulmonary hypertension (PH); pulmonary vascular resistance (PVR); right ventricle (RV).

Figure 1

Flow diagram of study cohort.

Figure 2

Correlation of the RV FAC with RV systolic dysfunction, N = 42. The horizontal axis represents the presence or absence of RV systolic dysfunction as determined by a subjective assessment based on additional measurements apart from the RV FAC; the vertical axis represents the RV FAC measurement (<0.35 is considered low in term infants).

Figure 3

RV FAC vs neurodevelopmental outcome quantile-quantile plot. The horizontal axis represents the RV FAC measurement (<0.35 is considered abnormally low). The vertical axis represents ND test Z-scores.