Management of the Fontan circulation in the adult patient

The Fontan circulation poses challenges for the patient and clinician throughout the patient’s lifetime. Given the unique physiology of the Fontan circulation, even the best Fontan patient will have a degree of exercise limitation and, with advancing years, the life threatening complications of the condition become more common. Key to the preservation of life and minimising the effects of these complications isprompt recognition and management. Patients with this complex condition require lifelong follow up in specialised units, where all members of the multidisciplinary team are experienced in managing the patients. The provision of this level of care requires planning, and in all healthcare systems where these patients are, care should be taken to develop a system of adult congenital heart disease units to facilitate the management of these and other complex cardiac conditions. This review summarises the anatomy and physiology of the Fontan circulation and provides guidance on the follow up of these patients and the complications they present with

Salience and Musical Form: On the Composition of 'Objets À Réaction Poétique' for Large Chamber Ensemble

This dissertation comprises an examination of the author’s compositional processes in general, and a discussion of the practical application of these principles in the composition of Objets à réaction poétique (2015-16) for nineteen musicians. Most notably, this composition and accompanying dissertation seek to substantiate the author’s hypotheses that structural- coherence may be achieved through a process attempting to assess the degree of salience of musical materials, and based upon these assessments, to ‘optimise’ associated durations & temporal-placements of materials. The various means by which salience may be achieved & quantified are examined in detail, as is the process through which an appropriate setting for the disposition of salient events is created

Talking about diseases; developing a model of patient and public-prioritised disease phenotypes

Deep phenotyping describes the use of standardised terminologies to create comprehensive phenotypic descriptions of biomedical phenomena. These characterisations facilitate secondary analysis, evidence synthesis, and practitioner awareness, thereby guiding patient care. The vast majority of this knowledge is derived from sources that describe an academic understanding of disease, including academic literature and experimental databases. Previous work indicates a gulf between the priorities, perspectives, and perceptions held by different healthcare stakeholders. Using social media data, we develop a phenotype model that represents a public perspective on disease and compare this with a model derived from a combination of existing academic phenotype databases. We identified 52,198 positive disease-phenotype associations from social media across 311 diseases. We further identified 24,618 novel phenotype associations not shared by the biomedical and literature-derived phenotype model across 304 diseases, of which we considered 14,531 significant. Manifestations of disease affecting quality of life, and concerning endocrine, digestive, and reproductive diseases were over-represented in the social media phenotype model. An expert clinical review found that social media-derived associations were considered similarly well-established to those derived from literature, and were seen significantly more in patient clinical encounters. The phenotype model recovered from social media presents a significantly different perspective than existing resources derived from biomedical databases and literature, providing a large number of associations novel to the latter dataset. We propose that the integration and interrogation of these public perspectives on the disease can inform clinical awareness, improve secondary analysis, and bridge understanding and priorities across healthcare stakeholders

Management of Adults With Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK:Survey of Current Practice, Unmet Needs and Expert Commentary

BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD. The questions related to referral pathways, screening, therapy and palliative care. RESULTS: 821 surveys were distributed and 106 were returned. Respondents included a broad mix of specialist physicians with many patients along with general cardiologists managing only a small number of PAH-CHD patients. Although 97% of respondents have access to a specialist PH centre, patients are still being managed in non-specialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols. Palliative care services are limited and general cardiologists rarely perform 6-minute walk tests (6MWT) or quality of life assessments. People with PAH-CHD are often undertreated, with 39% of respondents reporting that fewer than 25% of these patients were receiving PAH-specific therapies. CONCLUSIONS: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects