P1003Pulmonary artery dilatation in Eisenmenger syndrome, when and why?

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Background: Due to pulmonary artery (PA) dilatation it may come to thrombosis and in extreme forms to PA rupture and death. Eisenmenger syndrome (ES) hemodynamics represents volume overload resulting from congenital shunt combined with pressure overload from secondary pulmonary arterial hypertension. The aim of the study was to evaluate the degree of PA dilatation and possible influencing factors in ES patients.Patients and methods: 37 ES patients (pts) (28F/9M) with mean age 36±18.8 years [30 (81.1%) with post-tricuspid (post-TD); 7 (18.9%) with pre-tricuspid (pre-TD) defects] were analyzed and compared to healthy matched controls. Anatomic and hemodynamic parameters were established by echocardiography and in ES by right heart catheterization. PA diameter was indexed to body height (mm/m). PA dilatation was considered >15mm/m (mild 15-20mm/m, severe >20mm/m).Results: 1. PA was significantly greater in ES pts compared to controls: median 22.7mm/m (14.4-34.1) vs 11mm/m (8.9-16), p<0.0001. 2. PA was not dilated in controls, but significantly (p<0.0001) dilated in all ES pts (severe vs mild PA dilatation: in pre-TD 90.9% vs 9.1% and in post-TD 53.8% vs 46.2%). 3. PA dilatation was more severe in right ventricular dilatation (>30mm) (22.7 vs 18.9mm/m, p=0.01). 4. a more significant PA dilatation was found in pts with pulmonary-to-systemic flow ratio Qp/Qs ≥1 compared to Qp/Qs<1 (22.4 vs 19.3mm/m, p=0.04); in Qp/Qs≥1 severe PA dilatation(>20mm/m) was found in 75% of pts in comparison to Qp/Qs<1, where this was present only in 50% of pts, p=0.04. 5. PA diameter correlated with age (p=0.0003) but not with sex. 6. No correlation between PA dilatation and PA pressures in ES pts was found, though in pts with extreme PA dilatation (>25mm/m) there was a trend to lower PA pressures (mean PA pressure: 52 vs 70.5mmHg, p=0.21; systolic PA pressure: 75 vs 103mmHg, p=0.2).Conclusions: Compared to healthy individuals PA is in ES significantly dilated, already in children and progressive with age. Volume and pressure overload certainly influence PA diameter but the mechanism may be more complex. Patients with pre-TD (compared to post-TD) tend to more severe dilatation despite lower PA pressures. This may be explained by a less effective off-load at the atrial level, which is already known for the right ventricle but seems to be true also for the pulmonary artery. Therefore patients with a pre-TD have the highest risk for complications resulting from PA dilatation.

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