PURPOSE: Oral drugs made the treatment of pulmonary hypertension (PH) feasible in non-expert center, and prostanoid therapy could be delayed over time. The aim of the study was to evaluate if late referral to a tertiary PH center could influence the clinical course of patients who need a prostanoids (Prosta).
METHODS: Population included fifty-seven consecutive patients with precapillary PH who received a Prosta in our centre. Data at the initiation of Prosta (modality of access, medical history, NYHA class, exercise capacity, echocardiographic parameters, hemodynamics) were taken in consideration for the outcome (death)
RESULTS: Overall survival at 1, 2 and 4 years was 77%, 53% and 32%, respectively. Compared to survivors (Surv), non-survivors (Non-Surv) had a worse NYHA class (ClassIII/IV: Surv 27/1, Non-Surv 17/12; p<0.01) and effort capacity (six-minute-walk-test,Surv 354+91m, Non-Surv. 254+114m;p<0.01). Non-survivors were more frequently referred from other centres on oral therapy (Surv 36%, Non-Surv 83%; p<0.01) and had a higher rate of urgent prostanoid treatment (Surv 17%,Non-Surv 69%;p<0.0001). On multivariate analysis urgent prostanoid therapy and NYHA class were indentified as the only independent prognostic factors (HR 2,0, 95%CI, 1.1-3.9; HR 3,5, 95%CI, 1.5-8.2, respectively). Survivors had a significant response to Prosta, improving NYHA class (from 2.8±0.4 to 2.3±0.5 class; p=0.002), six-minute-walk-test (from 354±91 to 426±82 m; p=0.0001) and pulmonary hemodynamics (Ppa from 56±13 to 44±18 mmHg; p<0.05; CI from 2.0±1.2 to 3.1±1.2 l/min/m2; p=0.002; PVR from 17±10 to 8±6 WU; p=0.001).
CONCLUSIONS: In our series, referral of patients on oral treatment to a tertiary PH center is late, and has a significant impact on prognosis.
CLINICAL IMPLICATIONS: A close relationship between small PH center and terziary PH center with a common follow-up program and an early referral in case of not adequate therapeutic response is mandatory for a better treatment of this severe disease.
DISCLOSURE: Carmine Dario Vizza: University grant monies: GSK, UT, Pfizer, Actelion, Lilly
The following authors have nothing to disclose: Roberto Badagliacca, Roberto Poscia, Beatrice Pezzuto, Silvia Papa, Alfred Nona, Cristina Gambardella, Francesco Fedele
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