Management of pulmonary hypertension in Down syndrome.

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.

Surgical versus balloon valvotomy in neonates and infants: results from the UK National Audit.

Objective: There are conflicting data on choosing balloon aortic valvoplasty (BAV) or surgical aortic valvotomy (SAV) in neonates and infants requiring intervention for aortic valve stenosis. We aim to report the outcome of both techniques based on results from the UK national registry. Methods: This is a retrospective study, including all patients under 1 year undergoing BAV/SAV between 2000 and 2012. A modulated renewal approach was used to examine the effect of reinterventions on outcomes. Results: A total of 647 patients (488 BAV, 159 SAV, 292 neonates) undergoing 888 aortic valve procedures were included, with a median age of 40 days. Unadjusted survival at 10 years was 90.6% after initial BAV and 84.9% after initial SAV. Unadjusted aortic valve replacement (AVR) rate at 10 years was 78% after initial BAV and 80.3% after initial SAV. Initial BAV and SAV had comparable outcomes at 10 years when adjusted by covariates (p>0.4). AVR rates were higher after BAV and SAV reinterventions compared with initial valvoplasty without reinterventions (reference BAV, HR=3 and 3.8, respectively, p<0.001). Neonates accounted for 29/35 of early deaths after the initial procedure, without significant differences between BAV and SAV, with all late outcomes being worse compared with infants (p<0.005). Conclusions: In a group of consecutive neonates and infants, BAV and SAV had comparable survival and freedom from reintervention as initial procedures and when performed as reinterventions. These findings support a treatment choice based on patient characteristics and centre expertise, and further research into the best patient profile for each choice.