Ventricular–ventricular interaction variables correlate with surrogate variables of clinical outcome in children with pulmonary hypertension

Abstract

Determination of biventricular dimensions, function, and ventricular–ventricular interactions (VVI) is an essential part of the echocardiographic examination in adults with pulmonary hypertension (PH); however, data from according pediatric studies are sparse. We hypothesized that left and right heart dimensions/function and VVI variables indicate disease severity and progression in children with PH. Left heart, right heart, and VVI variables (e.g. end-systolic LV eccentricity index [LVEI], right ventricular [RV]/left ventricular [LV] dimension ratio) were echocardiographically determined in 57 children with PH, and correlated with New York Heart Association (NYHA) functional class (FC), N-terminal-pro brain natriuretic peptide (NT-proBNP), and invasive hemodynamic variables (e.g. pulmonary vascular resistance index [PVRi]). Clinically sicker patients (higher NYHA FC) had lower LV ejection fraction (LVEF) and higher LVEI – a surrogate of LV compression. In PH children, the ratio of systolic pulmonary arterial pressure divided by systolic systemic arterial pressure (sPAP/sSAP) and the PVRi correlated well with the LVEI ( P < 0.001). Patients with more severe PH (sPAP/sSAP ratio, PVRi) had increased RV/LV and right-to-left atrial dimension ratios ( P < 0.01). When stratified using NYHA-FC, sicker PH children had greater RV and right atrial dimensions with lower exercise capacity, while the tricuspid annular plane systolic excursion as surrogate for longitudinal systolic RV function decreased. Consistent with previous studies, serum NT-proBNP correlated with both, sPAP/sSAP ratio ( P < 0.001) and NYHA FC ( P < 0.01). Taken together, the VVI variables LVEI and RV/LV dimension ratio are associated with lower FC, worse hemodynamics, and higher NT-proBNP levels, thus highlighting the importance of ventricular interdependence in pediatric PH.

Pulmonary hypertension in bronchopulmonary dysplasia

“…44 Off-label use of macitentan also has been reported in BPD-PH patients. 56 Of note, bosentan lowers circulating sildenafil levels, while the other two ERA, ambrisentan and macitentan, do not. 12 Pharmacotherapy to modify the prostacyclin pathway: prostacyclin and prostacyclin analogs (PGI 2 and PGI 2 analogs; PCA).…”

A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension

“…For quantitative measure of left ventricular function, the modified Simpson formula can be used, as described elsewhere. In children, a decreased LVEF has been shown to be associated with an increased systolic PAP and PVRI (82).…”

Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

“…LV systolic function can be estimated by LVEF determination using the biplane Simpson formula. In pediatric patients with severe PH, a decreased LVEF has been shown to be associated with an increasing ratio of sPAP/sSAP and an increasing PVRi [ 138 ]. Although LVEF is one of the most widely used parameters to assess LV systolic function, it is limited in its ability to determine ventricular contractility by load dependency [ 149 ].…”

“…The RV/LV ratio, measured in the PSAX at end-systole at the LV papillary muscle level, reflects the extent of LV compression imposed by a hypertensive RV ( Figure 3 ). In pediatric and in adult PH patients, significantly higher RV/LV ratios were demonstrated when compared to control subjects [ 138 , 139 ]. An RV/LV ratio >1 was associated with adverse clinical events [ 139 , 140 ].…”

“…In children, LVesEI has been shown to be higher in patients with IPAH as compared to those with PH-CHD [ 99 ]. Pediatric PH-CHD patients with an increased LVesEI were found to have significantly smaller LA areas and an increased sPAP/SASP (pulmonary to systolic systemic arterial pressure) ratio, and an increased PVRi [ 138 ]. An LV end-diastolic EI > 1.7 has been shown to have prognostic value in adults with PH and to correlate well with invasive measurements of PAP [ 142 ].…”