Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient’s age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry.

Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg).

At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy.

There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.

Introduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. Results: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months–11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries – two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. Conclusions: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.

The following document provides a summary of the guidelines and recommendations for paediatric cardiac intensive care training as a requirement for recognition as a European paediatric cardiologist. It is therefore primarily targeting paediatric cardiology trainees in Europe, including those doctors who might wish to become experts in cardiac intensive care. These recommendations represent a frame for consistency, will evolve, and may be adapted to specific institutional requirements. They will be complemented by a learning module to be provided by our Association in the near future.

Cardiopulmonary bypass induces a generalized inflammatory response, with fever and leukocytes, which is difficult to differentiate from an infection. Recently, procalcitonin has been proposed as an early and specific marker of bacterial infection. The influence of cardiopulmonary bypass on production of procalcitonin, therefore, must be assessed before considering this molecule as a valuable marker of infection after cardiac surgery in children. With this in mind, we measured levels of procalcitonin, interleukin 6, and C-reactive protein before and 6 h, 1, 3 and 5 days after cardiopulmonary bypass, in 25 children undergoing cardiac surgery. Cardiopulmonary-bypass induced a transient increase in procalcitonin, with a peak at 24 h, with a median of 1.13 μg/l, a 25th and 75th interquartile of 0.68–2.25, and a p value of less than 0.001. The value had returned to normal in the majority of the children by the third day after surgery. Peak values correlated with the duration of cardiopulmonary-bypass, with a r-value of 0.58 and a p value of 0.003; cross-clamp time, with a r-value of 0.62 and a p value of 0.001; days of mechanical ventilation, with a r-value of 0.62 and a p value of 0.001; and days of stay in intensive care, with a r-value of 0.68, and a p value of 0.0003. The value returned to normal after 3 days in 83% of the patients. Levels of interleukin 6 and C-reactive protein also increased significantly after surgery, and remained elevated for up to 5 days.

Thus, in contrast to other markers, levels of procalcitonin in the serum are only slightly and transiently influenced by cardiopulmonary bypass, and may prove to be useful in the early recognition of an infection subsequent to cardiopulmonary bypass.

Sudden death and ventricular tachycardia are known to occur late after correction of tetralogy of Fallot. Abnormal dispersion of the QT interval, ventricular late potentials, and prolongation of the QRS complex, alone or in combination, are useful markers of the risk for such complications. Our present prospective study investigates the time course of appearance of two markers, dispersion of QT and JT, and ventricular late potentials, before and after corrective surgery. Dispersion of QT and JT, and signal averaged electrocardiographic parameters, were determined sequentially in 20 patients before, a mean of 9 ± 3 days after, and again 35 ± 11 days post-operatively. Dispersion of QT was already abnormal before surgery in two-fifths of the patients, but increased markedly in the later post-operative period. Ventricular late potentials were absent before surgery and in the immediate post-operative period, but were found in one-fifth of patients 1 month later. We conclude that abnormal dispersion of QT is, to some extent, a fact of the natural history of tetralogy of Fallot, but is significantly amplified by surgery. Ventricular late potentials, on the other hand, are absent before surgery, but appear with some delay after the operation, probably as a result of scarring rather than the surgical incision itself. Long-term follow-up is needed to assess the significance of these findings.

Aortic coarctation can now be repaired surgically with excellent results. Even though rare, injury to the spinal cord resulting in paraplegia remains a major concern. Preoperative evaluation showing the absence of collateral circulation is valuable in order to introduce protective actions. This report describes our experience using a temporary bypass from the ascending to the descending aorta bypass in children undergoing surgical correction of aortic coarctation in the setting of poorly developed collateral circulation.

Between 1990 and 2002, we undertook direct surgical repair in 56 patients with isolated aortic coarctation, 20 as neonates, 11 as infants, and 25 during childhood. From 1998 onwards, we introduced preoperative evaluation of the collateral circulation with magnetic resonance imaging. From that time, we placed a temporary bypass from the ascending to the descending aorta, using a polytetrafluoroethylene tube of 4 to 8 mm diameter, whenever distal pressures were shown to be 25 mmHg or less after test clamping, or when magnetic resonance imaging revealed absence of collateral circulation.

We found excellent correlations between the direct intra-operative measurements of distal pressure and the findings at magnetic resonance imaging. Following introduction of the temporary bypass, we observed no neurological complications, nor were there any complications related to bypass. Freedom from restenosis was 96%.

Preoperative magnetic resonance imaging, therefore, can accurately visualize poor collateral circulation in children with aortic coarctation. The use of a temporary bypass can possibly eliminate the risk of neurological sequels following direct repair of coarctation in children with poorly developed collateral circulation. The temporary bypass is both easy to apply and safe.