Carlos Andrés Quezada Loaiza1, María Teresa Velázquez Martín2, Carmen Jiménez López-Guarch3, María José Ruiz Cano4, Paula Navas Tejedor5, Patricia Esmeralda Carreira6, Ángela Flox Camacho2, Alicia de Pablo Gafas7, Juan Francisco Delgado Jiménez3, Miguel Ángel Gómez Sánchez8, Pilar Escribano Subías9. 1. Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Neumología, Hospital Universitario 12 de Octubre, Madrid, Spain. 2. Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, Spain. 3. Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, Spain; CIBER de Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain. 4. Herz-und Diabetes Zentrum NRW, Ruhr-Universität Bochum, Bad Oeynhausen, Germany. 5. Servicio de Cardiología, Hospital Universitario Gregorio Marañón, Madrid, Spain. 6. Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, Spain. 7. Unidad de Trasplante Pulmonar, Servicio de Neumología, Hospital Universitario 12 de Octubre, Madrid, Spain. 8. Servicio de Cardiología, Hospital Nuestra Señora del Prado, Talavera de la Reina, Toledo, Spain; CIBER de Enfermedades Respiratorias, Instituto de Salud Carlos III, Madrid, Spain. 9. Unidad Multidisciplinar de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, Spain; CIBER de Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain. Electronic address: pilar.escribano.subias@gmail.com.
Abstract
INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. RESULTS: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.
INTRODUCTION AND OBJECTIVES:Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. RESULTS: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.
Authors: Sophia Emmons-Bell; Catherine Johnson; Alexandra Boon-Dooley; Paul A Corris; Peter J Leary; Stuart Rich; Magdi Yacoub; Gregory A Roth Journal: Pulm Circ Date: 2022-01-18 Impact factor: 2.886
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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