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

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

Long-term outcomes of cardiac resynchronization therapy in adult congenital heart disease

Background and Aims: Randomized, controlled trials of cardiac resynchronization therapy (CRT) excluded patients with adult congenital heart disease (ACHD). We sought to explore long-term clinical outcomes. Methods and Results: In this single-center, observational study, events were collected from hospital records on patients with structural ACHD (sACHD) and adults with ischemic (ICM) or nonischemic (NICM) cardiomyopathy undergoing CRT. Patients with sACHD (n = 23, age: 41.6 ± 13.5 years [mean ± standard deviation]) and adults with ICM (n = 533) or NICM (n = 458) were followed-up for 4.1 years (median; interquartile range: 2.2-6.1). Total mortality was 5/23 (21.7%; 4.4 per 100 person-years) in sACHD, 221/533 (41.5%; 11.8 per 100 person-years) in ICM, and 154/458 (33.6%; 9.7 per 100 person-years) in NICM. In univariate analyses, total mortality in sACHD was lower than in ICM (hazard ratio [HR]: 0.38; 95% confidence interval [CI] 0.15-0.91), but similar to NICM (HR: 0.48, 95% CI 0.20-1.16). Cardiac mortality in sACHD was similar to ICM (HR: 0.78, 95% CI 0.32-1.92) and NICM (HR: 1.12, 95% CI 0.45-2.78). Heart failure (HF) hospitalization rates were similar to ICM (HR: 0.44, 95% CI 0.11-1.77) and NICM (HR: 0.75, 95% CI 0.18-3.08). In multivariate analyses, no differences emerged in total mortality, cardiac mortality, or HF hospitalization between sACHD and NICM or ICM, after adjustment for age, sex, New York Heart Association class, diabetes, atrial rhythm, QRS duration, QRS morphology, systemic ventricular ejection fraction, and medical therapy. Conclusion: Total mortality, cardiac mortality, and HF hospitalization after CRT in patients with sACHD was similar to adults with ICM or NICM