BACKGROUND: The continuous administration of prostacyclin analogs (PGI2) is a proven and effective therapy in patients with group 1 pulmonary arterial hypertension (PAH). However, few studies have addressed its use in adults with PAH associated with congenital heart disease (CHD-PAH). Concerns remain regarding the theoretical risk of worsening right-to-left intracardiac shunt. In this study, we present the hemodynamic and clinical effects of long-term, continuous PGI2 administration in eight adult patients with CHD-PAH. METHODS: We retrospectively reviewed the records of patients with CHD-PAH treated with continuous prostacyclin analogs epoprostenol and treprostinil. Nine patients were identified; one patient had no documentation of an intracardiac shunt and was excluded from this study. Hemodynamic, functional, laboratory, and clinical data were included. RESULTS: Mean duration of continuous PGI2 therapy was 1 year. Compared to baseline, patients exhibited significant improvements in mean pulmonary artery (PA) pressure and PA oxygen saturation, without a significant decline in systemic blood pressure or systemic oxygen saturation. Metabolic equivalents (METs) achieved on exercise testing increased, with an improvement in oxygen desaturation. World Health Organization functional classification remained the same. CONCLUSIONS: Long-term continuous PGI2 therapy in CHD-PAH patients resulted in hemodynamic and clinical improvements similar to those with group 1 PAH. The increase in PA oxygen saturation suggests that the net effect of PGI2 therapy did not result in increased right-to-left shunting, but, instead, diminished shunt. Though larger studies are needed, PGI2 should be considered as a potential treatment modality in adult patients with severe CHD-PAH who fail conventional therapy.
BACKGROUND: The continuous administration of prostacyclin analogs (PGI2) is a proven and effective therapy in patients with group 1 pulmonary arterial hypertension (PAH). However, few studies have addressed its use in adults with PAH associated with congenital heart disease (CHD-PAH). Concerns remain regarding the theoretical risk of worsening right-to-left intracardiac shunt. In this study, we present the hemodynamic and clinical effects of long-term, continuous PGI2 administration in eight adult patients with CHD-PAH. METHODS: We retrospectively reviewed the records of patients with CHD-PAH treated with continuous prostacyclin analogs epoprostenol and treprostinil. Nine patients were identified; one patient had no documentation of an intracardiac shunt and was excluded from this study. Hemodynamic, functional, laboratory, and clinical data were included. RESULTS: Mean duration of continuous PGI2 therapy was 1 year. Compared to baseline, patients exhibited significant improvements in mean pulmonary artery (PA) pressure and PA oxygen saturation, without a significant decline in systemic blood pressure or systemic oxygen saturation. Metabolic equivalents (METs) achieved on exercise testing increased, with an improvement in oxygen desaturation. World Health Organization functional classification remained the same. CONCLUSIONS: Long-term continuous PGI2 therapy in CHD-PAH patients resulted in hemodynamic and clinical improvements similar to those with group 1 PAH. The increase in PA oxygen saturation suggests that the net effect of PGI2 therapy did not result in increased right-to-left shunting, but, instead, diminished shunt. Though larger studies are needed, PGI2 should be considered as a potential treatment modality in adult patients with severe CHD-PAH who fail conventional therapy.
Authors: Nika Skoro-Sajer; Christian Gerges; Olga Hajnalka Balint; Dora Kohalmi; Monika Kaldararova; Iveta Simkova; Johannes Jakowitsch; Harald Gabriel; Helmut Baumgartner; Mario Gerges; Roela Sadushi-Kolici; David S Celermajer; Irene Marthe Lang Journal: Heart Date: 2018-02-07 Impact factor: 5.994