Hemodynamic assessment of pulmonary hypertension in corrected versus Non-corrected grown-up congenital heart disease

Purpose: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) is thought to result from persistent exposure of the pulmonary vasculature to increased blood flow of systemic-to-pulmonary shunts, and comprises a typical pulmonary arteriopathy in association with biventricular heart failure. Signs of endothelial dysfunction have been demonstrated in individuals with congenital heart disease both before and after surgery. Methods: 3107 right and left heart catheterizations were analyzed. 241 data sets were from patients with CHD. Results: Our database showed pre-tricuspid defects in 162 patients, post-tricuspid defects in 36 patients and complex lesions in 43 patients. 44 patients with CHD were corrected and 197 patients did not undergo any correction of their defect(s). PH was observed in 27 patients with a correction status and in 22 patients with non-corrected CHD. Mean survival of "corrected" patients with PH (7.3 years) was worse than in "non-corrected" patients PH (11.4 years; p=0.009). There was a significant difference in mixed venous saturation (SvO2) (p=0.01), diastolic pulmonary artery pressure (dPAP) (p=0.018) and mean pulmonary capillary wedge pressure (mPCWP) (p=0.006) between corrected and non-corrected patients with PH (Table 1). Table 1 Conclusion: The data demonstrate that grown-up patients with PH due to corrected CHD show a worse survival than patients with PH due to non-corrected CHD.