Usefulness of cardiac catheterization in Eisenmenger syndrome is controversial. We investigated the prognostic value of invasive hemodynamic parameters.

69 consecutive patients with congenital heart disease and pulmonary vascular resistance (PVR)>8UW.m² (Eisenmenger syndrome, n=63; non-correctable left-to-right shunt, n=6), with at least 1 catheterization after 1994, were included. Pulmonary artery pressures (PAP) and oxygen (O²) consumption were measured using the same standardized method. PVR were calculated using the Fick principle. Outcome was assessed in 2015 and survival analysis was performed.

Mean age at first catheterization with this protocol was 38.4±13.3 y.o. Pre-tricuspid, post-tricuspid and combined shunts were observed in 44 (63.8%), 23 (33.3%) and 2 (2.9%) cases. Patients were free of any pulmonary anti-hypertensive drugs in 54 (78.3%) cases. A Who status 3 or 4 was observed in 37 patients (53.6%). Median PVR and diastolic PAP were 24.6 [19.2-37.6] UW.m² and 40.0mmHg [34.5-50]. There was no complication. During a median follow-up of 7.2 y. [5.2-11.6], 23 (33.3%) patients reached a composite outcome criteria (death n=12; heart-lung transplantation n=8; transplantation list registration n=7). Outcome was associated with pulmonary O² sat. <70% (p=0.01), aortic O² sat. ≤88% (p=0.02), mixed venous blood O² sat. ≤65% (p=0.01), PVR≥30UW.m² (p=0.02), diastolic PAP≥45mmHg (p=0.01) and who 3-4 (p=0.01). After adjustment for the position of the shunt and the number of anti-hypertensive drugs, diastolic PAP≥45mmHg and Who 3-4 remained associated with outcome in Cox regression analysis (HR 5.6, p=0.006; HR=5.3, p=0.008). There were trends that did not reach significance for the other hemodynamic parameters.

In addition to functional status, first catheterization provides prognostic information in patients with Eisenmenger syndrome. If these information could improve the therapeutic algorithm remains to be demonstrated (figure next page).