Survival in pulmonary arterial hypertension (PAH) relates to right ventricular (RV) function. Whereas prognosis differs widely between PAH associated with congenital heart disease (CHD) and other causes of PAH, only little is known about differences in RV function.

We aimed at comparing RV function assessed by 3D-speckle-tracking in patients with CHD-PAH, other PAH aetiologies and healthy controls; and assess the relationship between ventricular function and prognostic parameters.

We performed a prospective multi-centric study between June 2015 and June 2017 recruiting 27 patients with CHD-PAH (3 had closed shunts, 24 had Eisenmenger syndrome; among these, 11 had a pre-tricuspid shunt, 13 had a post-tricuspid shunt), to compare with 27 group 1 non-CHD related-PAH patients (nPAH) and 27 controls matched on age and sex with the CHD-PAH group. Patients with complex CHD were excluded. All patients underwent 2D and 3D transthoracic echocardiography at baseline. 3D RV echocardiographic sequences were analysed by a commercial RV-specific software and output meshes were post-processed to extract deformation data.

There was no significant age difference between the subgroups. In CHD-PAH patients, RV global area and longitudinal strain did not significantly differ as compared to nPAH but RV global circumferential strain was significantly better (P=0.006). All strain components were impaired as compared to controls (P<0.0001). In the whole patient population, over a mean follow-up of 27.6±13.3 months, 10 patients (17.5%) died from PAH or were transplanted (including 2 patients with CHD-PAH). Global RV circumferential strain was significantly associated to death or transplant (P=0.004, AUC 0.823; HR 1.41[1.09–1.81]) (Fig. 1).

RV remodelling differs between adults with CHD-PAH and PAH from other aetiologies: 3D RV global circumferential strain is better in CHD-PAH patients and associated with survival free from transplant.