I want to thank the Organizing Committee of the 53rd Congress for inviting me to speak on optimizing the medical management of acute decompensated heart failure. I have nothing to disclose. The standard objectives are to define acute decompensated heart failure, review the causes, pathophysiology, classification, and management. Pediatric heart failure is a complex clinical and pathophysiologic syndrome that encompasses a diverse population of patients with congenital heart disease, cardiomyopathy, infectious inflammatory diseases, oncological processes, metabolic syndromes, renal failure, and malnutrition. On the other hand, acute decompensated heart failure is the final common pathway for children with congenital and acquired heart disease resulting in admissions to CICU. Let's define heart failure. It is a clinical syndrome characterized by impaired ejection of ventricular filling leading to cardinal symptoms of shortness of breath, feeding intolerance, and fatigue. Acute decompensated heart failure is heart failure severe enough to require hospitalization. Advanced heart failure is heart failure unresponsive to medical management, thus requiring mechanical circulatory support, ventricular assist device, and heart transplantation. The commonest cause of heart failure in younger age group is congenital heart disease, which may be due to a volume load. An example is a ventricular septal defect, patent ductus arteriosus, and valvular regurgitation. It could be also due to a pressure load seen with coarctation, aortic stenosis, pulmonary stenosis, or pulmonary arterial hypertension. Could also be secondary to single ventricle lesions. In older kids, cardiomyopathy is the commonest cause of heart failure in that population. Could be primary related to dilated cardiomyopathy, hypertrophic cardiomyopathy, or restrictive cardiomyopathy, or secondary to ischemia, toxins, infiltrative, or infectious processes. It could also be due to a rhythm disturbance. If the rhythm is too fast, an example in ectopic atrial tachycardia or ventricular tachycardia, or too slow, seen with congenital heart block or medications such as beta blockers or calcium channel blockers, or it could be pacing related as seen with a pacing induced cardiomyopathy. Acute decompensated heart failure continues to be an entity with incompletely understood pathophysiology and unfortunately limited therapeutic options. The pathophysiology is dependent on a number of factors such as the degree of systolic and diastolic cardiac function, involvement of the right and or left ventricle, arterial and venous vascular tone, neurohormonal and inflammatory activation states, comorbid contributing factors. Javier Laza et al. and the Pediatric Cardiac Critical Care Consortium PC4 published an article on the epidemiology and outcomes of acute decompensated heart failure in children in circulatory heart failure in 2020. This was a contemporary cohort of 1,494 children less than 18 years of age treated for acute decompensated heart failure in 23 PC4 centers. Acute decompensated heart failure was defined as a systolic or diastolic dysfunction requiring continuous vasoactive or diuretic infusion, respiratory support, or mechanical circulatory support. There were 26,294 CICU admissions during the time frame 8-1-2014 to 4-4-2017. 1,494 met diagnostic criteria for acute decompensated heart failure. 852 or 57% had congenital heart disease. 642-43% did not have congenital heart disease. The results were as follows. The CICU mortality rate for overall acute decompensated heart failure was 15% with an N of 231. There were a higher mortality rate in patients with congenital heart disease 19% 159 compared to non-congenital heart disease 11% or 72 patients. Acute decompensated heart failure occurred in 6% of CICU admissions. 1 in 5 acute decompensated heart failure related hospitalizations resulted in death. Congenital heart disease was present in over half of those hospitalized with acute decompensated heart failure and when compared to non-congenital heart disease was associated with a higher length of stay, greater use resource utilization, and poor survivor. The initial approach to a patient presenting an acute decompensated heart failure is to classify them based on one of four distinct hemodynamic profiles based on the assessment of the patient's volume or perfusion status. In terms of the volume status, are they wet versus dry? Perfusion status, are they cold versus warm? Cantor et al published in the European Journal of Pediatrics in 2010 their initial treatment of acute decompensated heart failure based on a two-minute assessment. They asked two questions with a yes-no answer. Is there congestion? Yes, if there were signs of hepatomegaly, tachypnea, orthopnea, edema, or ascites. The second question, is there poor perfusion? Yes, if there were signs of tachycardia, a narrow pulse pressure, cold extremities, irritability, and a decreased level of consciousness. They further classified them into four quadrants. A, warm and dry. B, warm and wet. C, cold and dry. D, cold and wet. For the warm and wet patient, based on the ISHLT published guidelines of management of pediatric heart failure in heart lung transplant in 2014, there is a class one indication, level C evidence, for initiation of diuretics in patients with fluid retention associated with ventricular dysfunction. Diuretics were the main line of therapy to decrease preload and achieve uvulemia. Loop diuretics are the drug of choice in these patients. The diuresis can also be enhanced when used in combination with thiazide diuretics. In the cold and wet patient, there is a class one indication for use of temporary intravenous inotropic support in patients presenting in cardiogenic shock. And class two indication in patients with evidence of low cardiac output syndrome and compromised end organ perfusion. Inotropes, vasodilators, and diuretics are indicated in this group of patients. Pure vasodilators such as nipride, nitroglycerin, and inodilators, milrinone, are considered first line therapy to augment cardiac output while reducing myocardial work. Positive inotropes such as epinephrine, dopamine, and dobutamine are considered second line therapy because they drive up myocardial work and may precipitate arrhythmias. They are reserved for hypotension or worsening renal dysfunction. There is evidence of class three harm in long-term use of inotropes, so it is not recommended in heart failure. In summary, acute decompensated heart failure demands early recognition and effective treatment. Early intervention with diuretics and inodilator support has been proven effective in stabilizing majority of patients, allowing for a detailed diagnostic assessment of the underlying disease. With that, I would like to thank you for your attention. I would like to acknowledge Dr. Himera Ahmed, a heart failure cardiologist at Seattle Children's Hospital, and Krista Kirk, a Heart Center pharmacist, for their support of my talk with slides and data. Thank you for your attention.

acute decompensated heart failure

children

congenital heart disease

management strategies

early intervention