Background .– Despite comparable morbidity, survival prospects for Eisenmenger pts are far superior compared with pts with idiopathic pulmonary arterial hypertension (PAH). This improved survival is likely the result of the preservation of RV function, since it does not remodel at birth and remains hypertrophied and adapted to pressure overload.

Aim of study .– We compared the echocardiographic parameters of right heart function between pts with Eisenmenger syndrome and pts treated for idiopathic PAH, with an equal level of systolic PAP (PAPs).

Patients .– We studied 15 pts with Eisenmenger (group 1: three CAVC, six ASD, two VSD, two ductus arteriosus, one TOF, one agenesis of pulmonary artery) and 15 pts with PAH (gr2). All subjects underwent a right heart catheterization and echocardiography within the same week. Each gr2 pt was matched to a gr1 pt according to age, sex and level of PAPs (± 5 mmHg). The following indices of RV function were obtained: TAPSE (mm), RV fractional area change (RVFAC, %), myocardial performance index (Tei) and maximal velocity of systolic wave (S max, cm/s) of the lateral tricuspid annulus. We measured maximal longitudinal strain (ε, %) in the three segments of the 3 RV walls (septal (sep), lateral (lat) from 4Cview and inferior (inf) from RV2Cview) by 2D speckle imaging. We calculated a lat ε (mean of 3 lat segments), an inf ε (mean of 3 inf segments) and an inferolat ε (mean of these two walls).

Results .– The surfaces of the RA and RV were not significantly different while the ratio RV/LV was significantly higher in the PAH group ( P = 0.03). There was no difference in any of the RV function parameters between both groups although there was a non-significant trend toward lower values of strain in gr2.

Gr1, Eisenmenger Gr2, PAH P value.

Age (yrs) 50.2 ± 15.8, 54.1 ± 17.8, 0.06.

IT gd max (mmHg) 78.3 ± 25.8, 77.9 ± 23.4, 0.92.

Cardiac index (l mn/m ² ) 2.9 ± 0.9, 2.5 ± 0.7, 0.29.

RV/LV 1.3 ± 0.5 1.7 ± 0.5, 0.01.

Smax (cm/s) 10.8 ± 3.5, 10.6 ± 1.8, 0.51.

TAPSE (mm) 19.0 ± 8.6, 15.9 ± 3.9, 0.43.

RV FAC (%) 0.36 ± 0.14, 0.31 ± 0.07, 0.25.

RV Tei 0.52 ± 0.22 0.53 ± 0.19, 0.86.

RV lateral strain (%) −18.0 ± 4.4, −14.7 ± 5.7, 0.08.

RV septal strain (%) −13.5 ± 6.9, −14.7 ± 3.1, 0.73.

RV inferior strain (%) −17.4 ± 4.6, −13.2 ± 5.0, 0.11.

RV inferolat strain (%) −16.3 ± 4.7, −13.9 ± 3.9, 0.13.

Conclusion .– In this population of Eisenmenger pts with severe PAH, conventional parameters of RV function are not different from those of pts with idiopathic PAH at the same level of systolic PAP. However, there is a tendency, although not significant, towards a lower alteration of RV longitudinal deformation parameters in pts with Eisenmenger, suggesting a relative preservation of RV function. The lack of significant difference between the two groups may be explained by the small sample size of this population with a significant number of pts with atrial shunt. However, the effect of sustained high level of PAP may perhaps also be almost the same on the RV after a long time of evolution whatever its etiology.