Roberto Del Pozo1, Isabel Blanco2, Ignacio Hernández-González3, Manuel López-Meseguer4, Raquel López-Reyes5, María Lázaro-Salvador6, Teresa Elías-Hernández7, Pablo Álvarez-Vega8, Gregorio Miguel Pérez-Peñate9, Amaya Martínez-Meñaca10, Pedro Bedate11, Pilar Escribano-Subias12. 1. Pulmonary Hypertension Unit, Pneumology Department of Hospital Universitario 12 de Octubre, Avenida de Córdoba s/n, 28041 Madrid, Spain. 2. Pulmonary Medicine Department, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Villarroel 170, 08036 Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Avenida de Monforte de Lemos 3-5, 28029 Madrid, Spain. 3. Pulmonary Hypertension Unit, Cardiology Department of Hospital Universitario 12 de Octubre, Avenida de Córdoba s/n, 28041 Madrid, Spain. 4. Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Avenida de Monforte de Lemos 3-5, 28029 Madrid, Spain; Pneumology Department of Hospital Universitario Vall d'Hebron, Paseo de la Vall d'Hebron 119-129, 08035 Barcelona, Spain. 5. Pneumology Department of Hospital Universitari i Politècnic la Fe, Avinguda de Fernando Abril Martorell 106, 46026, Valencia, Spain. 6. Cardiology Department of Hospital Virgen de la Salud, Avenida de Barber 30, 45005 Toledo, Spain. 7. Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Avenida de Monforte de Lemos 3-5, 28029 Madrid, Spain; Medical-surgical Unit of Respiratory Diseases, Pneumology Department of Hospital Universitario Virgen del Rocío, Instituto de Biomedicina de Sevilla (IBiS), Avenida Manuel Siurot s/n, 41013 Sevilla, Spain. 8. Pneumology Department of Complejo Asistencial Universitario de Salamanca (CAUSA), Montalvos 0, 37120 Doñinos de Salamanca, Salamanca, Spain. 9. Pulmonary Circulation Unit, Pneumology Department of Hospital Universitario de Gran Canaria Dr. Negrín, Barranco de la Ballena s/n, 35010 Las Palmas de Gran Canaria, Canarias, Spain. 10. Pneumology Department of Hospital Universitario Marqués de Valdecilla, Avenida Valdecilla 25, 39008 Santander, Cantabria, Spain. 11. Pneumology Department of Hospital Universitario Central de Asturias, Avenida de Roma s/n, 33011 Oviedo, Spain. 12. Pulmonary Hypertension Unit, Cardiology Department of Hospital Universitario 12 de Octubre, Avenida de Córdoba s/n, 28041 Madrid, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Avenida de Monforte de Lemos 3-5, 28029 Madrid, Spain. Electronic address: pilar.escribano.subias@gmail.com.
Abstract
INTRODUCTION: REHAP is a voluntary, observational Spanish registry of patients with pulmonary arterial hypertension. We analyzed the experience (use and effectiveness) with inhaled iloprost (inh-ILO) in real-life conditions during a 3-year period. METHODS: Patients included were those with PAH ≥14 years recruited during 1998-2016 who had received inh-ILO. Variables were collected at the beginning of treatment (0 ± 3 months) and 12 ± 3/36 ± 6 months follow-up. Effectiveness was assessed in the intent-to-treat population as changes in functional class and/or physical performance and transplant-free survival from the beginning of treatment. Stopping inh-ILO-related survival was also assessed. Subanalyses included treatment strategy (first-line therapy -monotherapy or upfront combination- or sequential therapy) and risk of clinical worsening/death. RESULTS: Inh-ILO was the most frequently used prostanoid in Spain, rendering 267 patients eligible for analysis. Median age was 54 years; 61% were WHO FC III. Sixty (23%) patients started inh-ILO as monotherapy, 27 (10%) as upfront combination and 180 (67%) sequentially. At 3-year follow-up significant clinical improvements were observed; however, transplant-free survival rate was 54%, being poorer in patients at high risk (63% vs. 85% in low risk patients; P < 0.001) and similar in the three treatment strategies. Only 25% patients remained on inh-ILO. Three-year after stopping inh-ILO-related survival rate was 24.7%. CONCLUSION: Data from the REHAP collected during 3 years shows that inh-ILO has low effectiveness independently of the treatment strategy used, with a 3-year survival rate of 54% despite significant clinical improvements, probably due to the use in high-risk patients. Discontinuation rate was as high as 75%.
INTRODUCTION: REHAP is a voluntary, observational Spanish registry of patients with pulmonary arterial hypertension. We analyzed the experience (use and effectiveness) with inhaled iloprost (inh-ILO) in real-life conditions during a 3-year period. METHODS:Patients included were those with PAH ≥14 years recruited during 1998-2016 who had received inh-ILO. Variables were collected at the beginning of treatment (0 ± 3 months) and 12 ± 3/36 ± 6 months follow-up. Effectiveness was assessed in the intent-to-treat population as changes in functional class and/or physical performance and transplant-free survival from the beginning of treatment. Stopping inh-ILO-related survival was also assessed. Subanalyses included treatment strategy (first-line therapy -monotherapy or upfront combination- or sequential therapy) and risk of clinical worsening/death. RESULTS: Inh-ILO was the most frequently used prostanoid in Spain, rendering 267 patients eligible for analysis. Median age was 54 years; 61% were WHO FC III. Sixty (23%) patients started inh-ILO as monotherapy, 27 (10%) as upfront combination and 180 (67%) sequentially. At 3-year follow-up significant clinical improvements were observed; however, transplant-free survival rate was 54%, being poorer in patients at high risk (63% vs. 85% in low risk patients; P < 0.001) and similar in the three treatment strategies. Only 25% patients remained on inh-ILO. Three-year after stopping inh-ILO-related survival rate was 24.7%. CONCLUSION: Data from the REHAP collected during 3 years shows that inh-ILO has low effectiveness independently of the treatment strategy used, with a 3-year survival rate of 54% despite significant clinical improvements, probably due to the use in high-risk patients. Discontinuation rate was as high as 75%.
Authors: Grzegorz Kopeć; Marcin Kurzyna; Ewa Mroczek; Łukasz Chrzanowski; Tatiana Mularek-Kubzdela; Ilona Skoczylas; Beata Kuśmierczyk; Piotr Pruszczyk; Piotr Błaszczak; Ewa Lewicka; Danuta Karasek; Katarzyna Mizia-Stec; Michał Tomaszewski; Wojciech Jacheć; Katarzyna Ptaszyńska-Kopczyńska; Małgorzata Peregud-Pogorzelska; Anna Doboszyńska; Agnieszka Pawlak; Zbigniew Gąsior; Wiesława Zabłocka; Robert Ryczek; Katarzyna Widejko-Pietkiewicz; Marcin Waligóra; Szymon Darocha; Michał Furdal; Michał Ciurzyński; Jarosław D Kasprzak; Marek Grabka; Karol Kamiński; Piotr Hoffman; Piotr Podolec; Adam Torbicki Journal: J Clin Med Date: 2020-01-08 Impact factor: 4.241