Acute decompensated heart failure

The main underlying diagnoses of children with end-stage heart failure are congenital heart disease and cardiomyopathy. These children require close clinical monitoring by a cardiac failure specialist, initially as outpatients treated with oral anti congestive heart failure medication, and eventually as inpatients treated with inotropes. In fact, the majority of patients followed by the Pediatric Heart Transplant Study Group are United Network of Organ Sharing (UNOS) status 1 (heart failure requiring intravenous inotropes). Because of the limited availability of organs, waiting time for heart transplantation is long and as a result, cardiac function continues to deteriorate prior to transplantation, eventually leading to inadequate systemic oxygen delivery and end-organ failure. If the latter develops, the patient is no longer a candidate for heart transplantation. In order to avoid end organ failure, close monitoring of oxygen delivery or surrogate measures of oxygen delivery and step-wise escalation of cardiopulmonary support are required. Other than standard monitoring measures, advanced techniques should be utilized to increase the safety margin and allow timely escalation of support. These advanced monitoring techniques include pulmonary and transpulmonary thermodilution, pulse contour analysis, tissue and regional near infrared spectroscopy, and biochemical markers of perfusion and myocardial strain. Inadequate oxygen delivery in a patient treated with one inotrope warrants admission to the intensive care unit and initiation of a second vasoactive medication. The next step is intubation and mechanical ventilation. Since peritransplantation morbidity and mortality are higher in mechanically ventilated children in comparison to children supported with long-term mechanical assist devices, serious consideration should be given to implantation of a ventricular assist device shortly after intubation.