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Time to extubation, maximum vasoactive-inotropic score, and length of hospital stay were selected as the secondary outcome variables.Compared with patients in whom the PDA was left open, patients with a surgically closed arterial duct had a higher incidence of resuscitation events (29.0% vs 0%, P = 0.0012), reinterventions (35.5% vs 3.2%, P = 0.0013), and higher early hospital mortality (9.7% vs 0%, P = 0.038). Time to extubation and length of hospital stay did not differ between the 2 groups (P = 0.16 and 0.73, respectively). A trend towards a higher maximum vasoactive-inotropic score in the group with a closed duct was observed (median 13.5 vs 10, P = 0.10). Authors concluded that in newborns with pulmonary atresia, ductal closure during modified BT shunt procedure is associated with increased incidence of resuscitation events, need for reintervention, and increased mortality during the early postoperative period.

BackgroundPatients with pulmonary arterial hypertension (PAH) are managed according to evidence-based treatment guidelines.Methods and resultsIn this single-centre retrospective analysis, we examined outcomes of patients with PAH caused by congenital heart disease (PAH-CHD) with respect to exercise capacity and survival of adults treated with either bosentan or sildenafil monotherapy or bosentan-sildenafil dual therapy between January 2007 and January 2014. Of the 82 patients analysed, 29 had Down syndrome; 54 (65.8%) received bosentan monotherapy, 16 (19.5%) sildenafil monotherapy and 12 (14.6%) dual therapy. Mean treatment duration was 2.5 years for all patients and 4.1 years for 38 patients treated for ≥2 years. Pooled patient and treatment data showed initial improvement followed by stabilisation in mean 6 min walk distance (6MWD). For Down and non-Down patients, mean 6MWD increased and then stabilised on bosentan monotherapy. Mean 6MWD of patients on dual therapy at the time of analysis was 246.3 m before PAH-specific therapy initiation, 211.9 m immediately prior to addition of a second therapy and 214.4 m at last visit while on dual therapy. 1, 2 and 3-year survival rates for all patients from time of treatment initiation were 96%, 87% and 80%, respectively.ConclusionsFor the majority of patients, monotherapy with a PAH-specific medication provided improved and sustained exercise benefits. For the small percentage of patients who required it, add-on therapy appeared to prevent further deterioration in exercise capacity but did not improve 6MWD.

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Removal of chest drains is a painful procedure and distresses patients, parents and nurses. To identify problems and improve management we developed a concerted approach to both assessment and treatment using audit. An initial prospective audit of post-op cardiac children undergoing chest drain +/- pacing wire removal was undertaken. A clinical tool was developed, to assess children before procedures and provide a treatment plan aimed at reducing pain and distress. The tool incorporated the intensity of the procedure, the child's usual response to procedures and previous experience. The treatment protocol included drug and non-drug therapies. The initial audit of 91 children confirmed that pain and distress were problems. It highlighted inconsistencies in management as a common feature. Following introduction of the LAPPS tool and treatment protocol, a second audit of 72 children was undertaken. Although only 46 per cent of pathways were satisfactorily completed, the percentage of patients described as 'inconsolable, screaming and withdrawn' reduced from 29 per cent to 9 per cent. Introduction of the LAPPS assessment tool and treatment protocol reduced pain and distress during removal of chest drains. Research is needed to test the psychometrics of the tool and the effectiveness of the treatment protocol prior to wider use.