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Pulmonary hypertension: Real-world data from a Portuguese expert referral centre

Published on Jul 1, 2018in Pulmonology
· DOI :10.1016/j.pulmoe.2018.02.003
Ana P. Gomes21
Estimated H-index: 21
,
Célia F. Cruz5
Estimated H-index: 5
+ 7 AuthorsAbílio Reis4
Estimated H-index: 4
Abstract
Abstract Background Pulmonary hypertension (PH) is a heterogeneous, debilitating condition with highly relevant impact on functional capacity, quality of life, and life-expectancy. Objectives This study aims to provide long-term data on the Portuguese PH population, by characterising the clinical presentation, evolution, and outcomes of PH patients in a specialised referral centre. Methods Retrospective analysis of a cohort of 101 patients with pre-capillary PH (pcPH) referenced to an expert tertiary care referral centre in northern Portugal from 2002 to 2013. Diagnosis was confirmed by right heart catheterisation (RHC). PH classification followed consensus criteria from the 5th World Symposium in Nice, 2013. Results The most frequent causes of pcPH were Group 1 PH – pulmonary arterial hypertension (PAH) (54.4%) and Group 4 PH – Chronic thromboembolic pulmonary hypertension (CTEPH) (25.7%); importantly, 17.8% of patients presented PH associated with multiple aetiologies. Targeted therapy was used in 91.1% of patients (48.5% combination therapy). 1-, 3-, and 5-year survival was estimated at 86.6%, 76.7%, and 64.1%, respectively. Survival was significantly better for those ≤40 years old (10.5 vs. 6.4 years; P = 0.003) and for women with I/HPAH (9.3 vs. 4.5 years; P = 0.039). Conclusions This study provides long-term, real-world data for the management of PAH and CTEPH in Portugal and demonstrates the importance of dedicated electronic medical records and well defined clinical management protocols for better patient outcomes. Patients presented mostly with intermediate or high risk of mortality, which suggests delayed diagnosis and highlights the need to increase awareness among clinicians.
  • References (50)
  • Citations (1)
References50
Newest
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Last. Graça CastroH-Index: 6
view all 5 authors...
Abstract Background Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. Methods Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7–5.4] years. Kaplan–Meier survival a...
3 CitationsSource
Background and Aims The aim of our study was to describe the characteristics and clinical outcomes of pulmonary hypertension (PH) patients who underwent their follow-up at a single centre. Methods We included patients who fulfilled the criteria for the diagnosis of PH by right heart catheterisation. A detailed clinical history and complete physical examination were recorded for each patient. Demographic data, laboratory testing, haemodynamic values and treatment records were also collected. A 6-...
3 CitationsSource
#1R. Awdish (HFHS: Henry Ford Health System)H-Index: 1
#2H. Cajigas (HFHS: Henry Ford Health System)H-Index: 1
Pulmonary arterial hypertension (PAH) is a subcategory of pulmonary hypertension (PH) that comprises a group of disorders with similar pulmonary vascular pathology. Though PH is common, the estimated incidence of IPAH is 1–3 cases per million, making it a rare disease. The hemodynamic definition of PAH is a mean pulmonary artery pressure at rest >OR = 25 mm Hg in the presence of a pulmonary capillary wedge pressure
8 CitationsSource
#2R. del PozoH-Index: 1
Last. J.A. Barberà-Mir (University of Barcelona)H-Index: 54
view all 10 authors...
Abstract Background The Spanish "Registry of Pulmonary Arterial Hypertension" (REHAP), started in 2007, includes chronic thromboembolic hypertension (CTEPH) patients. Based on data provided by this registry and retrospective data from patients diagnosed during 2006 (≤12months since the registry was created), clinical management and long-term outcomes of CTEPH patients are analyzed nationwide for the first time in a scenario of a decentralized organization model of CTEPH management. Methods and r...
25 CitationsSource
#1Nazzareno GalièH-Index: 80
#2Marc HumbertH-Index: 119
Last. Marius M. HoeperH-Index: 79
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ALAT : alanine aminotransferase ASAT : aspartate aminotransferase APAH : associated pulmonary arterial hypertension BAS : balloon atrial septostomy BMPR2 : bone morphogenetic protein receptor 2 BNP : brain natriuretic peptide BPA : balloon pulmonary angioplasty BREATHE : Bosentan
1,884 CitationsSource
#1Kasper Korsholm (Aarhus University Hospital)H-Index: 3
#2Asger Andersen (Aarhus University Hospital)H-Index: 10
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AbstractWe aimed to characterize and estimate survival rates in patients diagnosed with pulmonary arterial hypertension (PAH) in western Denmark in the modern management era. All incident cases of PAH were consecutively enrolled in our single-center prospective cohort study between January 2000 and March 2012. A total of 134 patients fulfilling the inclusion criteria were followed up from first diagnostic right heart catheterization to either death or the end of the study. Kaplan-Meier survival ...
16 CitationsSource
#1Nazzareno Galiè (UNIBO: University of Bologna)H-Index: 80
#2J.A. Barberà-Mir (University of Barcelona)H-Index: 54
Last. Lewis J. Rubin (UCSD: University of California, San Diego)H-Index: 97
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The primary analysis included 500 participants; 253 were assigned to the combination-therapy group, 126 to the ambrisentan-monotherapy group, and 121 to the tadalafil-monotherapy group. A primary end-point event occurred in 18%, 34%, and 28% of the participants in these groups, respectively, and in 31% of the pooledmonotherapy group (the two monotherapy groups combined). The hazard ratio for the primary end point in the combination-therapy group versus the pooled-monotherapy group was 0.50 (95% ...
354 CitationsSource
#1Nazzareno Galiè (UNIBO: University of Bologna)H-Index: 80
#2Katharina Müller (Bayer AG: Bayer HealthCare Pharmaceuticals)H-Index: 3
Last. Ekkehard Grünig (University Hospital Heidelberg)H-Index: 25
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PATENT PLUS evaluated the safety and efficacy of riociguat in combination with sildenafil in pulmonary arterial hypertension patients. Patients receiving sildenafil (20 mg three times daily) were randomised to placebo or riociguat (up to 2.5 mg three times daily) for 12 weeks. The primary outcome was maximum change in supine systolic blood pressure (SBP) from baseline within 4 h of dosing. Secondary objectives comprised additional blood pressure, heart rate and exploratory efficacy variables, an...
82 CitationsSource
#1Lewis J. Rubin (UCSD: University of California, San Diego)H-Index: 97
#2Nazzareno Galiè (UNIBO: University of Bologna)H-Index: 80
Last. Hossein Ardeschir Ghofrani (Imperial College London)H-Index: 71
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Riociguat is a soluble, guanylate cyclase stimulator, approved for pulmonary arterial hypertension. In the 12-week PATENT-1 study, riociguat was well tolerated and improved several clinically relevant end-points in patients with pulmonary arterial hypertension who were treatment naive or had been pretreated with endothelin-receptor antagonists or prostanoids. The PATENT-2 open-label extension evaluated the long-term safety and efficacy of riociguat. Eligible patients from the PATENT-1 study rece...
73 CitationsSource
#1Vallerie V. McLaughlin (UM: University of Michigan)H-Index: 44
#2Sanjiv J. Shah (NU: Northwestern University)H-Index: 51
Last. Marc Humbert (University of Paris-Sud)H-Index: 119
view all 4 authors...
Abstract Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, progn...
145 CitationsSource
Cited By1
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#1Alexandra Arvanitaki (A.U.Th.: Aristotle University of Thessaloniki)H-Index: 1
#2Maria BoutsikouH-Index: 4
Last. Athanasios ManginasH-Index: 7
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Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemio...
1 CitationsSource