Published online by Cambridge University Press: 29 December 2014
Neonatal critical aortic stenosis is a rare form of CHD that often presents with cardiogenic shock. Although surgical and cardiac catheterisation-based interventions have been successful in alleviating left ventricular outflow tract obstruction, it remains associated with high morbidity and mortality. Critical aortic stenosis results in elevated left ventricular wall stress, which ultimately increases myocardial oxygen consumption and disrupts coronary artery perfusion during diastole, leading to ventricular dysfunction and cardiogenic shock. Critical care management before definitive intervention should be tailored to optimising oxygen delivery and reducing metabolic consumption of the myocardium and peripheral organ systems. This can be accomplished with prostaglandin infusion to maintain system perfusion through patency of the arterial duct, inotropic support, mechanical ventilation, and central nervous system abrogation. Management should also include a multi-specialty medical team including paediatric cardiothoracic surgeons and paediatric cardiologists with expertise in cardiac catheterisation, imaging, and transplantation.
Presented at All Children’s Hospital Johns Hopkins Medicine 14th International Symposium on Congenital Heart Disease, Saint Petersburg, Florida, 15–18 February 2014, Special Focus: Diseases of the Cardiac Valves from the Fetus to the Adult, Co-Sponsor: The American Association for Thoracic Surgery (AATS).