Exertional dyspnoea in pulmonary arterial hypertension.

Dyspnoea is a principal presenting symptom in pulmonary arterial hypertension (PAH), and often the most distressing. The pathophysiology of PAH is relatively well understood, with the primary abnormality of pulmonary vascular disease resulting in a combination of impaired cardiac output on exercise and abnormal gas exchange, both contributing to increased ventilatory drive. However, increased ventilatory drive is not the sole explanation for the complex neurophysiological and neuropsychological symptom of dyspnoea, with other significant contributions from skeletal muscle reflexes, respiratory muscle function, and psychological and emotional status. In this review, we explore the physiological aspects of dyspnoea in PAH, both in terms of the central cardiopulmonary abnormalities of PAH and the wider, systemic impact of PAH, and how these interact with common comorbidities. Finally, we discuss its relationship with disease severity

Pulmonary arterial hypertension registries: past, present and into the future

Registries have greatly contributed to knowledge about PAH epidemiology, risk factors, prognosis and treatment. Future registries face unique challenges but may benefit from integration of multiple data sources and capitalising on “Big Data” opportunitiesConflict of interest: J. Weatherald reports grants, personal fees and non-financial support from Janssen Inc. and Actelion, personal fees and non-financial support from Bayer, personal fees from Novartis, and grants from Alberta Lung Association, Canadian Vascular Network, European Respiratory Society and Canadian Thoracic Society, outside the submitted work. Conflict of interest: A. Reis reports grants, personal fees and non-financial support from Janssen Inc. and Actelion, outside the submitted work. Conflict of interest: O. Sitbon reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer and Merck, grants from GlaxoSmithKline, grants, and personal fees from United Therapeutics and Gossamer Bio, outside the submitted work. Conflict of interest: M. Humbert reports personal fees from Actelion and Merck, and grants and personal fees from Bayer, GSK and United Therapeutics, outside the submitted work.info:eu-repo/semantics/publishedVersio

Exploring engagement at ArtPlay: What factors influence the engagement of children and families in an artist-led community-based workshop?

THE IMPORTANCE OF THE ARTS to the creative, cultural and social development of children is increasingly acknowledged. In recognition of this, ArtPlay was established in 2005 by the City of Melbourne. Open to children aged three to 12 years, the facility provides a wide range of artist-led programs that serve a broad community, including parents and teachers. While institutions such as ArtPlay are emerging in response to a growing community demand, there has yet to be significant and sustained research into processes and outcomes of such organisations. This paper reports on a three-year Australia Research Council-funded research project (2007–2009) that has been designed to identify, map and evidence the practices of ArtPlay in relation to engagement, learning and cultural citizenship. Through reference to the responses of children, families and artists to one key ArtPlay program, Pocketfool for preschoolers, this paper explores the question: What factors influence the engagement of children and families in an artist-led community-based workshop?12

Klippel-Trenaunay syndrome as a rare cause of chronic thromboemboembolic pulmonary hypertension

International audienceKlippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous capillary malformations, soft tissue and bone hypertrophy, and multiple capillary, venous or lymphatic malformations. KTS is associated with recurrent thromboembolic events. We reported herein five cases of chronic thromboembolic pulmonary hypertension (CTEPH) associated with KTS (age minimum-maximum 26-50 years old, 3 males/2 females). Hemodynamics showed severe pulmonary hypertension (PH) with pulmonary vascular resistance ranging from 5.6 to 18.3 Wood units (WU), associated with marked clinical impairment (NYHA functional class III or IV in 4 patients). Computed tomography (CT) of the chest and pulmonary angiography confirmed proximal CTEPH accessible to surgical intervention in one patient and distal forms of CTEPH in 4 patients. Evolution after pulmonary endarterectomy showed hemodynamic normalization, while the patients with distal CTEPH had severe outcomes with 2 early deaths after PH diagnosis (44 and 35 months respectively). One patient with distal CTEPH was still alive 16 years after diagnosis on specific PH therapy and one was transplanted after 15 years because of right heart failure (death after 12 months). Histological analysis of the lung explants showed typical chronic thromboembolic material specific for CTEPH. In conclusion, KTS may be complicated by severe CTEPH requiring careful anticoagulation and multidisciplinary follow-up in expert centers to screen for disease potentially accessible to endarterectomy. In the modern management era of CTEPH, balloon pulmonary angioplasty will certainly be an interesting option in patients with inoperable disease

Awake Proning in Patients with COVID-19-related hypoxemic acute respiratory failure: a Rapid Practice Guideline.

This Rapid Practice Guideline provides evidence-based recommendations for the use of awake proning in adult patients with acute hypoxemic respiratory failure due to COVID-19. The panel included 20 experts from 12 countries, including one patient representative, and used a strict conflict of interest policy for potential financial and intellectual conflicts of interest. Methodological support was provided by the Guidelines in Intensive Care, Development, and Evaluation (GUIDE) group. Based on an updated systematic review, and the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) method we evaluated the certainty of evidence and developed recommendations using the Evidence-to-Decision framework. We conducted an electronic vote, requiring >80% agreement amongst the panel for a recommendation to be adopted. The panel made a strong recommendation for a trial of awake proning in adult patients with COVID-19 related hypoxemic acute respiratory failure who are not invasively ventilated. Awake proning appears to reduce the risk of tracheal intubation, although it may not reduce mortality. The panel judged that most patients would want a trial of awake proning, although this may not be feasible in some patients and some patients may not tolerate it. However, given the high risk of clinical deterioration amongst these patients, awake proning should be conducted in an area where patients can be monitored by staff experienced in rapidly detecting and managing clinical deterioration. This RPG panel recommends a trial of awake prone positioning in patients with acute hypoxemic respiratory failure due to COVID-19. This article is protected by copyright. All rights reserved