Assessing the clinical benefit, safety, and patient-reported outcomes with the use of the PAHcare™ digital platform in pulmonary arterial hypertension: a pilot study

IntroductionDigital health interventions, particularly mobile health platforms, have shown promise in supporting patients with respiratory conditions, but their application in pulmonary arterial hypertension (PAH) remains limited. We aimed to assess the feasibility, acceptability, and potential clinical benefit of the novel PAHcare™ digital platform as a patient-centred intervention for PAH management through a prospective, single-arm, multicenter pilot study conducted on 53 patients diagnosed with PAH who used the platform for 6 months.MethodsThe primary objective was to assess the impact on Health-Related Quality of Life (HRQoL) through questionnaires. Secondary objectives included evaluating clinical outcomes, including disease progression, PAH signs and symptoms, the 6-min walking test, and the patient’s symptom perception. Additionally, we assessed patient satisfaction and engagement with the PAHcare™ platform, interaction with health coaches, retention, costs and healthcare resource utilisation (HCRU), and safety through monitoring device incidents.ResultsMinimal changes in HRQoL and clinical outcomes were observed over 6 months. A noteworthy 92.4% of patients actively used the platform in the first month, maintaining high usage throughout the study. Patient satisfaction was substantial, with more than half of the patients expressing excellence in service quality, willingness to reuse the platform, and fulfilment of their needs. Health coach interaction was high, with 76% of patients initiating contact within the first week. User retention rates were 70%, with prevalent ongoing usage and interaction with healthcare professionals even after the study. In terms of HCRU and costs, the study showed no significant changes in PAH-related hospital admissions, clinical visits, or tests. Finally, the low number of device-related incidents indicated platform safety.ConclusionThis pilot study provides compelling evidence supporting the feasibility and acceptability of the PAHcare™ digital platform to empower patients to manage their disease and significantly enhance their overall experience with PAH

Effect of a Pulmonary Embolism Diagnostic Strategy on Clinical Outcomes in Patients Hospitalized for COPD Exacerbation. A Randomized Clinical Trial

SLICE Trial Group.[Importance] Active search for pulmonary embolism (PE) may improve outcomes in patients hospitalized for exacerbations of chronic obstructive pulmonary disease (COPD).[Objective] To compare usual care plus an active strategy for diagnosing PE with usual care alone in patients hospitalized for COPD exacerbation.[Design, Setting, and Participants] Randomized clinical trial conducted across 18 hospitals in Spain. A total of 746 patients were randomized from September 2014 to July 2020 (final follow-up was November 2020).[Interventions] Usual care plus an active strategy for diagnosing PE (D-dimer testing and, if positive, computed tomography pulmonary angiogram) (n = 370) vs usual care (n = 367).[Main Outcomes and Measures] The primary outcome was a composite of nonfatal symptomatic venous thromboembolism (VTE), readmission for COPD, or death within 90 days after randomization. There were 4 secondary outcomes, including nonfatal new or recurrent VTE, readmission for COPD, and death from any cause within 90 days. Adverse events were also collected.[Results] Among the 746 patients who were randomized, 737 (98.8%) completed the trial (mean age, 70 years; 195 [26%] women). The primary outcome occurred in 110 patients (29.7%) in the intervention group and 107 patients (29.2%) in the control group (absolute risk difference, 0.5% [95% CI, −6.2% to 7.3%]; relative risk, 1.02 [95% CI, 0.82-1.28]; P = .86). Nonfatal new or recurrent VTE was not significantly different in the 2 groups (0.5% vs 2.5%; risk difference, −2.0% [95% CI, −4.3% to 0.1%]). By day 90, a total of 94 patients (25.4%) in the intervention group and 84 (22.9%) in the control group had been readmitted for exacerbation of COPD (risk difference, 2.5% [95% CI, −3.9% to 8.9%]). Death from any cause occurred in 23 patients (6.2%) in the intervention group and 29 (7.9%) in the control group (risk difference, −1.7% [95% CI, −5.7% to 2.3%]). Major bleeding occurred in 3 patients (0.8%) in the intervention group and 3 patients (0.8%) in the control group (risk difference, 0% [95% CI, −1.9% to 1.8%]; P = .99).[Conclusions and Relevance] Among patients hospitalized for an exacerbation of COPD, the addition of an active strategy for the diagnosis of PE to usual care, compared with usual care alone, did not significantly improve a composite health outcome. The study may not have had adequate power to assess individual components of the composite outcome.[Trial Registration] ClinicalTrials.gov Identifier: NCT02238639.Peer reviewe

Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase

Memoria de Gestión de una Unidad Multidisciplinar Autonómica de Circulación Pulmonar Integrada en el Servicio de Neumología del Hospital General Universitario de Gran Canaria “Dr Negrín”

La comunidad autónoma de Canarias es una región ultra-periférica de la Unión Europea en la que están censados 2.108.121 habitantes y que además soporta una población flotante de 10,6 millones, en un territorio geográfico fraccionado (1). Esta población precisa de una atención en temas de salud que en su mayoría cubre el Sistema Canario de Salud (parte del Sistema Nacional Español de Salud). Nuestro servicio de salud esta sujeto a numerosos retos uno de ellos es el de la atención a las enfermedades raras. Dos de estas enfermedades son la Hipertensión pulmonar (HP) del grupo I, Hipertensión Arterial Pulmonar (HAP), y 4, Hipertensión Pulmonar Tromboembolica Crónica (HPTEC) (2). El diagnostico de estos procesos se lleva a cabo con frecuencia en fases avanzadas, lo que junto con su gravedad condicionan una baja supervivencia (54% a los 5 años, según el registro español de HAP, REHAP) (3). Los procedimientos diagnósticos y terapéuticos de estas patologías son de elevada complejidad y requieren de equipos multidisciplinares con alto grado de experiencia y coordinación en su ejecución. Todo esto junto con las recomendaciones de la “European Union Committe of experts on rare diseases” (4) hacen necesario una organización específica, en centros de referencia nacional y centros de competencia regionales o autonómicos. El objetivo final de este documento es la promoción de una Unidad de Gestión Clínica Multidisciplinar de Circulación Pulmonar (UMCP) centrado en el Servicio de Neumología del Hospital General Universitario de Gran Canaria (HGUGC) “Dr Negrín” como centro de referencia autonómico para el diagnóstico y tratamiento de la hipertensión pulmonar compleja que formará parte de una red europea de centros para estas enfermedades

Guía de diagnóstico y tratamiento de la hipertensión pulmonar: resumen de recomendaciones

[EN] Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery. These guidelines describe the current recommendations for the diagnosis and treatment of the different pulmonary hypertension groups.[ES] La hipertensión pulmonar es un trastorno hemodinámico definido por el aumento anómalo de la presión arterial pulmonar, que puede presentarse en numerosas enfermedades y situaciones clínicas. Las causas de hipertensión pulmonar se clasifican en 5 grandes grupos: arterial, debida a cardiopatía izquierda, debida a enfermedad pulmonar y/o hipoxemia, tromboembólica crónica y de mecanismo no establecido y/o multifactorial. El presente documento expone de forma resumida las recomendaciones de la Guía de Diagnóstico y Tratamiento de la Hipertensión Pulmonar de la Sociedad Española de Neumología y Cirugía Torácica. En dicha guía se presentan las pautas actuales de diagnóstico y tratamiento de los distintos grupos de hipertensión pulmonar.Peer reviewe

Safety of inhaled nitric oxide withdrawal in severe chronic pulmonary hypertension

Abstract Inhaled nitric oxide (iNO) is a potent and selective pulmonary vasodilator with a safety concern due to rebound pulmonary hypertension (PH) associated with its withdrawal. We report short‐term pulsed iNO in patients with severe pulmonary arterial hypertension (PAH) and nonoperable chronic thromboembolic PH (nCTEPH). This is a retrospective analysis of 33 patients: 22 with PAH and 11 with nCTEPH. We assessed hemodynamic, echocardiographic, and other noninvasive variables to evaluate safety and efficacy of iNO. We performed an iNO withdrawal test during right heart catheterization and after 3 days of iNO treatment. iNO significantly improved all variables examined in 22 patients with PAH and 11 with nCTEPH. Two patterns of response were observed after sudden iNO withdrawal. Twenty‐nine patients (88%) showed minimal hemodynamic, oxygenation and clinical changes. Four patients (12%) had a reduction in cardiac index ≥20% and PaO2 ≥ 5%, three patients did not show clinical deterioration, and one patient developed hemodynamic collapse that needed iNO administration. This retrospective study suggests that short‐term iNO improves hemodynamics and clinical conditions in some patients with PAH an nCTPEH. However, pulsed iNO withdrawal PH rebound could be a serious concern in these patients. Given the lack of evidence, we do not recommend the use of pulsed iNO in the treatment of patients with chronic PH

Real-life experience of inhaled iloprost for patients with pulmonary arterial hypertension: Insights from the Spanish REHAP registry

REHAP investigators.[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%.We express our gratitude to Actelion, Ferrer, GlaxoSmithKline (GSK) and Merck Sharp & Dohme (MSD) for supporting the REHAP Registry with an unrestricted educational grant.Peer reviewe