New multidimensional echocardiographic markers have been developed to assess right ventricle remodeling and function in patients with pulmonary arterial hypertension (PAH). Their prognostic value in patients with congenital heart diseases (CHD) remains little known.

We assess the prognostic value of right ventricle end-systolic remodeling index (RVESRI), RV global longitudinal strain (GLS), RV fractional area change (FAC) in 34 patients (47.7±12.6-year-old) with PAH and pre-tricuspid shunt. Associations between baseline echocardiographic values and major adverse cardiovascular events (MACE) were assessed using a univariate Cox regression analysis.

Eisenmenger syndrome was observed in 15 patients (44.1%). Mean pulmonary artery pressure was 48.7±12.7mmHg. Median pulmonary vascular resistance was 9.4 wood-unit [5.1–15]. Mean RV GLS was -15.1±5.0%. Mean TAPSE was 18.9±5.8. Mean RV FAC was 30.0±8.6%. Mean RVESRI was 1.6±0.3. Mean right atrium area was 21.8±6.4mm². Mean right to left atrium area ratio was 1.5±0.5. Pericardial effusion was noted in 7 patients (20.6%). Mean systole to diastole duration ratio (S/D) was 1.3±0.5.

After a mean follow-up of 16.6±8.4months, a major adverse cardiovascular event (MACE) was reported in 8 patients (23.5%) including 1 death, 3 transplantation and 4 waitlisting. Echocardiographic data associated with outcome were RV ESRI (HR=25.2, P=0.02); right atrium area (HR=1.3, P=0.002); RV GLS (HR=1.8, P=0.03); right to left atrium area ratio (HR=6.6, P=0.02) and S/D (HR=7.3, P=0.004). A non-significant trend towards an association with MACE was observed for pericardial effusion (HR=3.4, P=0.1); RV FAC (HR=0.9, P=0.1); TAPSE (HR=0.9, P=0.1) and RV systolic area (HR=1.1, P=0.07).

RVESRI and RV GLS carries strong relationships with outcome in addition to conventional echocardiographic parameters of RV function in patients with PAH and CHD.