No abstract

Acute pulmonary hypertension (PH) complicates the course of several cardiovascular, pulmonary and other systemic diseases in children. An acute rise of RV afterload, either as exacerbating chronic PH of different aetiologies (eg, idiopathic pulmonary arterial hypertension (PAH), chronic lung or congenital heart disease), or pulmonary hypertensive crisis after corrective surgery for congenital heart disease, may lead to severe circulatory compromise. Only few clinical studies provide evidence on how to best treat children with acute severe PH and decompensated RV function, that is, acute RV failure. The specific treatment in the intensive care unit should be based on the underlying pathophysiology and not only be focused on so-called 'specific' or 'tailored' drug therapy to lower RV afterload. In addition therapeutic efforts should aim to optimise RV preload, and to achieve adequate myocardial perfusion, and cardiac output. Early recognition of patients at high risk and timely initiation of appropriate therapeutic measures may prevent the development of severe cardiac dysfunction and low cardiac output. In patients not responding adequately to pharmacotherapy, (1) novel surgical and interventional techniques, temporary mechanical circulatory support with extracorporeal membrane oxygenation, (2) pumpless lung assist devices (3) and/or lung or heart-lung transplantation should be timely considered. The invasive therapeutic measures can be applied in a bridge-to-recovery or bridge-to-lung transplant strategy. This consensus statement focuses on the management of acute severe PH in the paediatric intensive care unit and provides an according treatment algorithm for clinical practice.

No abstract

Despite alterations in lymphocyte subpopulations, individuals with Down syndrome can mount effector T-cell responses with similar phenotype and functionality as controls but may require higher effector T-cell frequencies to ensure pathogen control.

Magnetic resonance angiography (MRA) is an established noninvasive imaging modality for detection and evaluation of vascular pathologies in children with congenital heart disease. Standard first-pass (FP)-MRA uses a 3-dimensional MRA sequence with an extracellular contrast agent, in which spatial resolution is limited by breath-hold duration, and image quality (IQ) is limited by motion artifacts. The purpose of this study was to compare the diagnostic confidence, IQ, and image artifacts of standard FP-MRA to a high-resolution, motion compensated steady-state (SS)-MRA of the thoracic vasculature in children and adolescents with congenital heart disease using a blood-pool contrast agent (gadofosveset trisodium). SS-MRA of the thoracic vasculature (technically successful in 90% of patients) offers superior diagnostic confidence and IQ compared with FP-MRA and shows fewer motion-related image artifacts. In addition, SS-MRA revealed findings missed by FP-MRA. Therefore, SS-MRA may prove specifically beneficial for imaging of thoracic vessels that are small and/or subject to motion.