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We examine the incidence and prevalence of domiciliary ventilation in the South West region of the UK, assess trends over 15 years, and describe patient outcome. We conducted a retrospective review of all patients below 18 years receiving domiciliary ventilation in the South West region of the UK between January 1994 and August 2009. Children who received long-term ventilation solely in hospital were excluded from the study. Information was obtained from a locally held database, medical notes, and hospital administration systems. One hundred-six patients were identified. Prevalence has increased since 1994 from 0.2 to 6.7 per 100,000 children. The incidence of both invasive and non-invasive ventilations has increased with a trend towards more non-invasive therapy. The commonest underlying disorders were airway pathology (37 patients), neuromuscular disease (34 patients), and central congenital hypoventilation disorder (17 patients). Sixty-seven patients had significant co-morbidities. Of 38 non-current patients, 19 were transferred to adult ventilation services, 11 died, and 6 were successfully weaned from ventilatory support. In conclusion, there has been a 30-fold increase in the prevalence of paediatric domiciliary ventilation, in the South West region of the UK, since 1994. Co-morbidities are common. Very few children discontinue long-term ventilation, and increasing numbers of ventilated children are transferred to adult services.

Unlabelled: Children with Down syndrome (DS) are at greater risk of pulmonary arterial hypertension (PAH) than the general population, partly due to upper airway obstruction and congenital heart disease. We wished to review our management of PAH and suggest a protocol for the systematic management of these children. Children with DS and PAH were included as referred for assessment from March 2005 to May 2010. Twenty-five patients (13 boys) met inclusion criteria. The median age was 385 days (range, 106 to 5,734); mean tricuspid regurgitation jet was 3.5 (range, 2.7-4.8) m/s. At cardiac catheterisation, mean pulmonary artery mean pressure was 26 mmHg (range, 12 to 46), and mean pulmonary vascular resistance (PVR) was 4.14 U.m² (range, 1.20 to 12.43) at baseline. PVR fell to a mean of 2.68 U.m² (range, 0.38 to 10.69) with 20 ppm inhaled nitric oxide and 100% oxygen. Respiratory assessment included polysomnography (18), bronchoscopy (16), showing malacia (eight), adenotonsillar hypertrophy (eight) and floppy aryepiglottic folds (four). One lung biopsy showed plexogenic arteriopathy, and one was diagnosed with tracheo-oesophageal fistula., Conclusion: In order to manage this complex group of patients, a combined cardiological, respiratory and surgical approach was required. A protocol with cardiac catheterisation, blood tests and respiratory assessment is suggested for the management of pulmonary hypertension in these children.