Emergency arterial switch: Rescue therapy for life-threatening hypoxemia in infants with transposition of great arteries with intact intraventricular septum.

OBJECTIVE: A small percentage of infants with d-loop transposition of the great arteries with intact intraventricular septum have life-threatening refractory hypoxemia often due to coexistent persistent pulmonary hypertension of the newborn. In this case series we describe the outcomes of a "rescue" emergency arterial switch operation (ASO). METHODS: We undertook a retrospective medical record analysis of infants with d-loop transposition of the great arteries with intact intraventricular septum who underwent an ASO in New Zealand from January 1, 1996, to April 30, 2017. Data were compared for those who received an emergency ASO and those with a nonemergency ASO for descriptive purposes. An emergency ASO was defined as one that was undertaken for life-threatening refractory hypoxemia when the only alternative stabilization strategy was preoperative extracorporeal life support. Primary outcome measures were 30-day postoperative mortality and abnormal neurodevelopmental outcome in the survivors. Secondary outcomes were low cardiac output, arrhythmia, renal dysfunction, postoperative seizures, and length of stay. Other known risk factors for morbidity and mortality were also assessed. RESULTS: Two hundred seventy-two infants underwent an ASO with 25 (9%) who received an emergency ASO. No infants received preoperative extracorporeal life support. The emergency group had greater 30-day postoperative mortality (8.0% vs 0.4%; P = .01) with no difference in abnormal neurodevelopmental outcome among the survivors (17.4% vs 13.8%; P = .35). The emergency group had more therapies for low cardiac output syndrome, more postoperative seizures, and a longer length of stay. CONCLUSIONS: An emergency ASO is a definitive rescue therapy that can be undertaken with acceptable mortality and neurodevelopmental outcome with consideration of the preoperative clinical state.

Drug Treatment of Heart Failure in Children: Focus on Recent Recommendations from the ISHLT Guidelines for the Management of Pediatric Heart Failure.

The International Society of Heart and Lung Transplantation (ISHLT) recently updated consensus pediatric heart failure guidelines from those published in 2004 with an aim to provide a practical evidence-based resource whilst recognizing the influence of adult heart failure practice. The new guidelines were formed from published evidence for heart failure management and used parallels with adult literature where pediatric evidence was lacking. This is a summary of the pharmacological therapies discussed in the new 2014 guidelines, emphasizing changes from the previous recommendations with regards to treatment of chronic heart failure with reduced ejection fraction, chronic heart failure with preserved ejection fraction, and acute decompensated heart failure. Each recommendation is classified according to strength and level of evidence. We also discuss future perspectives in the pharmacological treatment of heart failure. The 2014 ISHLT guidelines have evolved considerably from those published in 2004 with extensive information surrounding the underlying pathophysiology, investigations and recommended treatment. The new guidelines contain a modest amount of new pediatric data on pharmacological therapies and extrapolate adult data when appropriate. It is likely that most new recommendations for pediatric heart failure will continue to be based on therapies of proven benefit in adult heart failure studies.