Objective To evaluate the prognostic significance of right ventricular function assessed by echocardiography after start or escalation of targeted therapy in patients with pulmonary arterial hypertension.

Methods Study design: longitudinal study. Setting: tertiary referral centre for pulmonary hypertension. Patients: 81 consecutive patients with pulmonary arterial hypertension (33 naive and 48 prevalent). Interventions: right heart catheterisation and echocardiography performed prior to starting or escalating targeted therapy and repeated in 55 patients after 4–12 months of therapy. Main outcome measure: survival after follow-up examinations.

Results 11 patients died and 7 were lost to follow-up during the first year; 8 patients underwent first follow-up evaluation beyond 1 year. 55 patients were re-evaluated after therapy; during the subsequent follow-up period of 25 months, 9 patients died, 7 worsened from WHO I/II to III/IV and 15 remained in WHO III/IV despite therapy. A baseline tricuspid annular plane systolic excursion (TAPSE) ≥15 mm was associated with a lower risk of death (HR=0.32; 95% CI 0.12 to 0.83, p=0.012). Attaining a TAPSE≥15 mm after therapy was associated with a significantly lower risk of death or clinical worsening (HR=0.2; 95% CI 0.1 to 0.6, p=0.002) and a lower risk of death which approached statistical significance (HR=0.3; 95% CI 0.2 to 1.1, p=0.075). Per cent changes in TAPSE were loosely related to changes in pulmonary vascular resistances after therapy (R=0.37).

Conclusions In patients with pulmonary arterial hypertension, the evaluation of right ventricular function by TAPSE after targeted therapy is useful to predict subsequent prognosis, regardless of the haemodynamic effects of therapy.