Initial oral combination therapy in PAH decreased PVR by an average of 35%, but a low-risk status at 6 months was achieved in only 34.8% to 43.1% of patients.
Cohort (n=181)
Does initial combination of oral endothelin receptor antagonist and phosphodiesterase-5 inhibitor drugs improve pulmonary vascular resistance and risk status in treatment-naive patients with pulmonary arterial hypertension?
Despite initial combination therapy reducing pulmonary vascular resistance by an average of 35%, less than half of treatment-naive PAH patients achieved a low-risk status at 6 months.
Abstract Rationale An initial oral combination of drugs is being recommended in pulmonary arterial hypertension (PAH), but the effects of this approach on risk reduction and pulmonary vascular resistance (PVR) are not known. Objectives To test the hypothesis that a low-risk status would be determined by the reduction of PVR in patients with PAH treated upfront with a combination of oral drugs. Methods The study enrolled 181 treatment-naive patients with PAH (81% idiopathic) with a follow-up right heart catheterization at 6 months (interquartile range, 144–363 d) after the initial combination of endothelin receptor antagonist + phosphodiesterase-5 inhibitor drugs and clinical evaluation and risk assessments by European guidelines and Registry to Evaluate Early and Long-Term PAH Disease Management scores. Measurements and Main Results Initial combination therapy improved functional class and 6-minute-walk distance and decreased PVR by an average of 35% (median, 40%). One-third of the patients had a decrease in PVR 25%. This poor hemodynamic response was independently predicted by age, male sex, pulmonary artery pressure and cardiac index, and at echocardiography, a right/left ventricular surface area ratio of greater than 1 associated with low tricuspid annular plane systolic excursion of less than 18 mm. A low-risk status at 6 months was achieved or maintained in only 34.8% (Registry to Evaluate Early and Long-Term PAH Disease Management score) to 43.1% (European score) of the patients. Adding criteria of poor hemodynamic response improved prediction of a low-risk status. Conclusions A majority of patients with PAH still insufficiently improved after 6 months of initial combinations of oral drugs is identifiable at initial evaluation by hemodynamic response criteria added to risk scores.
Badagliacca et al. (Fri,) conducted a cohort in Pulmonary arterial hypertension (n=181). Initial oral combination of endothelin receptor antagonist and phosphodiesterase-5 inhibitor was evaluated on Reduction of pulmonary vascular resistance and achievement of low-risk status. Initial oral combination therapy in PAH decreased PVR by an average of 35%, but a low-risk status at 6 months was achieved in only 34.8% to 43.1% of patients.
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