Aims The aim of this study was to evaluate long-term outcome of pacemakers (PMs) in paediatric patients. Methods and results Patients' data were retrospectively reviewed. We recorded the techniques and systems used, any complication, and outcome. Endocardial leads were inserted by transcutaneous puncture of subclavian vein and fixed with a non-absorbable ligature, and epicardial leads by standard surgical technique. Lead survival was calculated and plotted with the product limit method of Kaplan–Meier. Between 1982 and 2002, 292 patients, aged 8±7 years (range 1 day–18 years), underwent PM implantation: the first PM had endocardial leads in 165 patients and epicardial in 127 patients. Structural heart disease (HD) was present in 239 patients. Follow-up was 5±4 (range 0.1–18) years. There were no pacing-related deaths. In total, 211 endocardial implantation procedures with 90 atrial and 165 ventricular leads and 145 epicardial procedures with 103 atrial and 123 ventricular leads were performed. Early (<3 months) complications: haemothorax occurred in 3.5% of endocardial leads and dislodgement was not significantly different for atrial and ventricular endocardial leads. Late complications: 63 leads failed (48 epicardial), with the worst outcome for conventional epicardial leads (31 vs. 9% endocardial, P<0.05; steroid eluting 8% epicardial vs. 5% endocardial, P=NS). Endocardial atrial leads failed (7%) in operated HD and ventricular leads failed (6%) after body growth, without difference in estimated mean survival time (11 years). Early and late PM infection/erosion was ∼2% in all patients. Conclusion Pacing in children shows good results, but complications are frequent and related to leads. Endocardial pacing showed better long-term outcome.

Duchenne muscular dystrophy (DMD) cardiomyopathy (DCM) is characterized by a hypokinetic, dilated phenotype progressively increasing with age. Regular cardiac care is crucial in DMD care. Early recognition and prophylactic use of angiotensin converting enzyme inhibitors (ACEi) are the main stay therapeutic strategy to delay incidence of DMD-DCM. Pharmacological treatment to improve symptoms and left ventricle (LV) systolic function, have been widely implemented in the past years. Because of lack of DMD specific drugs, actual indications for established DCM include current treatment for heart failure (HF). This review focuses on current HF strategies to identify, characterize, and treat DMD-DCM.

Cardiac pacing is a difficult technique in children, particularly in patients with congenital heart defects (CHDs). Few studies to date have addressed this topic. We performed a retrospective analysis of the results of a single centre. Between 1982 and 2008, 287 patients with CHD, median age of 5 years (25–75%, 1–11) underwent cardiac pacing for sinus node dysfunction (SND) and atrioventricular block (AVB); 97% of patients underwent at least one heart surgery. Endocardial systems (Endo) were implanted in 117 patients, epicardial systems (Epi) in 170, with 595 leads (228 Endo, 367 Epi). Endocardial systems showed a significantly older age group with more frequent SND; Epi a younger age group, with more frequent AVB, greater number of surgical interventions. Perioperative complications were mortality 0.6% (Epi), pericardial effusion 0.6% (Epi), and haemothorax 3.4% (Endo). The median follow-up is 5 (2–10) years: the pacing system failed in 29% of patients, 13% Endo, and 40% Epi (P < 0.0001). Multivariate analysis showed a significantly higher risk of failure for Epi, a lower implant age, greater the number of leads implanted. The risk of malfunction of the leads increases significantly for Epi and the younger age when implanted. The steroid-eluting leads have a lower risk of malfunction (P = 0.05), steroid-eluting Endo leads provide significantly better outcomes than Epi. Cardiac pacing in paediatric patients with CHD shows satisfactory results in the long term. Endocardial systems show significantly better results than Epi systems. A younger age when implanted is a risk factor for complications at follow-up.