P451Echocardiographic evaluation of the right heart in Eisenmenger patients: a comparison study with other causes of PAH.


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Abstract

Patients with Eisenmenger's syndrome have a survival advantage over those with iPAH or pulmonary PAH. Improved survival may result possibly from preservation of RV function as the RV does not undergo remodelling at birth and remains hypertrophied.In this study, we aimed to compare the right heartechocardiographic parameters in 2 different populations: adult patients with Eisenmenger syndrome and patients with idiopathic and other causes of PAH.We studied 62 patients with Eisenmenger syndrome (10 ASD, 1 aorto-pulmonarywindow, 8 PDA, 22 VSD, 3 VSD + PDA, 18 complete AVSD) and 24 patients with PAH(9 iPAH, 3 pulmonary fibrosis with inappropriate PAH, 8 CTPH, 1 porto-pulmonaryhypertension, 3 PAH related to HIV and 1 Von-Recklinghausen's syndrome).Patients with Eisenmenger syndrome were younger (39 ± 15 years vs 67y± 13, p<0,0001) than others and the peak systolic tricuspid pressure gradient was higher (+23mmHg, p<0,001). The RA area and the EDRV area were higher than normal for both patients but significantly lower in Eisenmenger patients (20cm2 vs 26 cm2, p = 0,01 and 26cm2 vs 30cm2, p= 0,02 respectively for RA area and EDRV area). Concerning the right ventricular systolic function, the TAPSE is similar in the 2 groups (19,1± 5 mm vs 20,5 ± 4,2 mm p = 0,1), whereas the peak of the TDI S wave at the tricuspid annulus is significantly lower in Eisenmenger patients (10 ± 2,3 vs12,6 ± 3,1 cm/s p=0,002). Despite the impaired long-axis function, the FAC is better in Eisenmenger patients (38,8 ± 10,9% vs 31 ± 8,7% p = 0,04).Due to the improve survival in Eisenmenger patients, we expected better RV parameters. On the contrary, we found that the "standard" long-axis RV evaluation is not different in Eisenmenger compared to other causes of PAH, whereas the FAC is higher. That data is important, and might be explained by a different pattern of RV contraction in Eisenmenger patients with a major role for the radial contraction. The RV in Eisenmenger syndrome would contract more like a LV.

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