Theme: advanced lung disease
Prognostic factors in severe pulmonary hypertension patients who need parenteral prostanoid therapy: The impact of late referral

https://doi.org/10.1016/j.healun.2011.12.011Get rights and content

Background

Oral drugs have made the treatment of pulmonary hypertension (PH) feasible in non-expert centers, which could delay patient access to prostanoid therapy.

Methods

Fifty-seven consecutive patients with precapillary PH received a prostanoid in our center. Data at prostanoid initiation included modality of center referral, medical history, New York Heart Association [NYHA] class, exercise capacity, echocardiographic parameters, and hemodynamics.

Results

Overall survival at 1, 2, and 3 years was 85%, 69%, 55%, respectively. Non-survivors had worse NYHA class III/IV (17/12) than survivors (27/1; p < 0.01) and exercise capacity on 6-minute-walk distance (254 ± 114 vs 354 ± 91 meters; p < 0.01). Non-survivors were more frequently referred on oral therapy (83% vs 36%; p < 0.01) and had a higher rate of urgent prostanoid treatment (69% vs 17%; p < 0.0001). Multivariate analysis (hazard ratio [95% confidence interval]) found the independent prognostic factors were urgent prostanoid therapy (2.0 [1.1–3.9]) and NYHA class (3.5 [1.5–8.2]). Survivors had a significant response to prostanoid, improving NYHA class from 2.8 ± 0.4 to 2.3 ± 0.5 (p = 0.002), 6-minute walk distance from 354 ± 91 to 426 ± 82 meters (p = 0.0001), and pulmonary hemodynamics (pulmonary artery pressure from 56 ± 13 to 44 ± 18 mm Hg [p < 0.05]; cardiac index from 2.0 ± 1.2 to 3.1 ± 1.2 liters/min/m2 [p = 0.002], and pulmonary vascular resistance from 17 ± 10 to 8 ± 6 WU [p = 0.001]).

Conclusions

Referral of patients on oral treatment to a tertiary PH center is delayed and significantly affects prognosis.

Section snippets

Methods

The research reported in this study was authorized by the Institutional Review Board.

Study population

Among 280 patients with PAH or CTEPH, 57 consecutive patients met the criteria for treatment with a parenteral prostanoid and were included in the analysis, comprising 36 patients with idiopathic PAH (IPAH), 10 with PAH associated to connective tissue disease (CTD-PAH), 3 with PAH associated with HIV (HIV-PAH), 6 with portopulmonary hypertension (Po-PH), and 2 with inoperable CTEPH. Follow-up was a mean duration of 755 ± 859 days (range, 2–4,249 days).

The clinical features of the overall study

Discussion

This study investigated the effect of long-term parenteral prostanoids in the oral drug era and in a real-world setting. Our cohort included 57 PH patients who represent the sickest patients with PH because they all needed a parenteral prostanoid for the severity of the disease at presentation or for clinical worsening on oral therapy. The survival rate of our population at 1 year was 85%, which is comparable with the survival rate predicted in the Registry to Evaluate Early And Long-term PAH

Disclosure statement

Carmine Dario Vizza has received fees for serving as a speaker, consultant and an advisory board member, from the following Companies: Actelion, Dompè, GSK, Italfarmaco, Lilly, Pfizer, United Therapeutics. Roberto Badagliacca has received fees for serving as a speaker and consultant from the following Companies: Dompè, Italfarmaco, Pfizer. None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts

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    Copyright © 2012 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.