Arteriovenous malformations may present with significant haemodynamic compromise in the neonatal period, typically with high output cardiac failure that may be accompanied by hypoxia and right ventricular dysfunction. Targeted neonatal echocardiography performed by trained neonatologists provides an enhanced physiology-based approach that can guide treatment and minimise complications. We present a case of a large hepatic vascular malformation whose therapy was guided by targeted neonatal echocardiography to prevent paradoxical embolisation of procedural glue to the systemic circulation.

Hypoxemic patients often desaturate further with movement and transport. While inhaled epoprostenol does not improve mortality, improving oxygenation allows for transport of severely hypoxemic patients to tertiary care centers with a related improvement in mortality rates. Extracorporeal membrane oxygenation (ECMO) use is increasing in frequency for patients with refractory hypoxemia, and with increasing regionalization of care, safe transport of hypoxemic patients only becomes more important. In this series, four cases are presented of young patients with severe hypoxemic respiratory failure from Legionnaires’ disease transported on inhaled epoprostenol to ECMO centers for consideration of cannulation. With continued climate changes, Legionella and other pathogens are likely to be a continued threat. As such, optimizing oxygenation to allow for transport should continue to be a priority for critical care transport (CCT) services.

Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome.

A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009.

Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.