Pediatric Heart Failure

Definition

The International Society for Heart and Lung Transplantation defines pediatric heart failure (HF) as “a clinical and pathophysiologic syndrome that results from ventricular dysfunction, volume, or pressure overload, alone or in combination. In children, it leads to characteristic signs and symptoms, such as poor growth, feeding difficulties, respiratory distress, exercise intolerance, and fatigue, and is associated with circulatory, neurohormonal, and molecular abnormalities” (Kirk 2014).

Demographics

Pediatric heart failure-related hospitalizations occur in 11,000-14,000 children annually in the United States, with an overall mortality of 7%. Infants account for the majority (64%) of pediatric heart failure admissions (Rossano 2012). HF in children is most commonly attributable to coexistent congenital heart disease (CHD), with different risks depending on the specific type of malformation (Hinton 2017).  HF occurs in approximately 20% of all patients with CHD (Jayaprasad 2016).

Etiology

Ventricular dysfunction leading to HF can result from congenital or acquired disease processes, including CHD, cardiomyopathy, infectious disease, renal failure, oncological processes, metabolic syndromes, malnutrition, etc.(Ahmed 2021). Two mechanisms, congenital heart disease or cardiomyopathy, characterized most cases. According to Hinton and Ware, about twenty percent (20%) of CHD cases will involve ventricular dysfunction, while 100% of cardiomyopathy cases will result in ventricular dysfunction. In many cases of HF, the two mechanisms overlap.

Diagnostics

HF is characterized as "Any structural or functional cardiac disorder that impairs the ability of the ventricle to fill or eject blood" (Hunt 2009). Echocardiography provides diagnostic information on the cardiac structure and the degree of myocardial dysfunction. Classifying pediatric HF in children is complicated by age and development:

• Fetal HF is characterized by, decreased fetal movement, pericardial effusion, and ascites (Tworetsky 1999).
• Preterm low birth weight newborn, HF is characterized by acidemia, anemia, and hypoxemia.
• Term newborn HF is characterized by tachypnea, fatigue with feedings, and decreased urine output (Hinton 2017)   

In children, less than 18 years of age, the primary cardiac diagnosis at the time of admission is CHD (69%), followed by arrhythmias (12–15%), cardiomyopathy (13–14%), and myocarditis (~2%) (Ahmed 2021).

Signs and symptoms of CHF in infants & young children:

• Tachypnea: a fast respiratory rate, usually greater than 50 breaths per minute.
• Retractions: labored breathing, with ribs looking more prominent when the child inhales.
• Diaphoresis: profuse sweating
• Edema: swelling appearing first around the face and eyes, but also in the hands and feet
• Failure to gain weight.
• Difficulty feeding: due to fatigue and shortness of breath; infant may suck vigorously at first and then tire quickly.
• Hepatomegaly: liver enlargement due to increased venous pressure.
• Cyanosis: bluish coloration of skin and lips, especially while crying.

Assess for signs of low perfusion

• hypotension or tachycardia with narrow pulse pressure
• extremities cool to touch
• irritable or decreased consciousnes

Assess for pulmonary congestion/cardiac failure

• tachypnea
• orthopnea
• pulmonary edema
• dependent edema
• ascities
• persistent cough
• weight gain
• hepatomegaly

Acute HF treatment goals - Improve hemodynamics and prevent progression

• Inotropes/vasopressors/diuretics/spironolactone/vasodialators
• Mechanical ventilation
• Arrhythmia treatment
• Mechanical circulatory support indicated for use children with decompensated HF who cannot be stabilized with medical therapy alone to unload the failing ventricle and maintain end-organ perfusion.
  • VAD - ventricular assist device
  • ECMO - extracorporeal membrane oxygenation

Chronic HF treatment goals - maintain stability, provide support, enabling growth and development (Das 2018).

Medical treatment

• angiotensin-converting enzyme inhibitors (ACEi) are first-line (decrease afterload by antagonizing the renin-angiotensin aldosterone system)
• angiotensin receptor blockers (ARBs)
• β-receptor antagonists are second-line therapies in children (β-blockers antagonize the deleterious effects of chronic sympathetic myocardial activation and can reverse LV remodeling)
• Spironolactone - Aldosterone antagonist, potassium sparing diuretic. Aldosterone is secreted by the adrenals during physiologic stress, hypovolemia and hyponatremia
• Digoxin recommendations against use in children with asymptomatic left ventricular dysfunction (LVD), Low dosage for symptomatic LVD and with caution in patients with renal dysfunction. Antidote for the treatment of patients with life-threatening or potentially life-threatening digoxin toxicity or overdose, DIGOXIN IMMUNE FAB
• Cardiac resynchronization therapy for children with severely-reduced LV systolic dysfunction (LV EF < 35%) with prolonged QRS duration, and with or without complete left bundle branch block.
• Permantent pacemaker

Surgical correction or transplant

Reference

Ahmed, H., & VanderPluym, C. (2021). Medical management of pediatric heart failure. Cardiovascular diagnosis and therapy, 11(1), 323–335. https://doi.org/10.21037/cdt-20-358

Das B. B. (2018). Current State of Pediatric Heart Failure. Children (Basel, Switzerland), 5(7), 88. https://doi.org/10.3390/children5070088

Hinton, R. B., & Ware, S. M. (2017). Heart Failure in Pediatric Patients With Congenital Heart Disease. Circulation research, 120(6), 978–994. https://doi.org/10.1161/CIRCRESAHA.116.308996

Hunt SA, Abraham WT, Chin MH, Feldman AM, Francis GS, Ganiats TG, Jessup M, Konstam MA, Mancini DM, Michl K, Oates JA, Rahko PS, Silver MA, Stevenson LW, Yancy CW; American College of Cardiology Foundation; American Heart Association. 2009 Focused update incorporated into the ACC/AHA 2005 Guidelines for the Diagnosis and Management of Heart Failure in Adults A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the International Society for Heart and Lung Transplantation.J Am Coll Cardiol. 2009; 53:e1–e90. doi:

Jayaprasad N. (2016). Heart Failure in Children. Heart views : the official journal of the Gulf Heart Association, 17(3), 92–99. https://doi.org/10.4103/1995-705X.192556

Kirk, R., Dipchand, A. I., Rosenthal, D. N., Addonizio, L., Burch, M., Chrisant, M., Dubin, A., Everitt, M., Gajarski, R., Mertens, L., Miyamoto, S., Morales, D., Pahl, E., Shaddy, R., Towbin, J., & Weintraub, R. (2014). The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary. [Corrected]. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, 33(9), 888–909. https://doi.org/10.1016/j.healun.2014.06.002

Rossano, J. W., Kim, J. J., Decker, J. A., Price, J. F., Zafar, F., Graves, D. E., Morales, D. L., Heinle, J. S., Bozkurt, B., Towbin, J. A., Denfield, S. W., Dreyer, W. J., & Jefferies, J. L. (2012). Prevalence, morbidity, and mortality of heart failure-related hospitalizations in children in the United States: a population-based study. Journal of cardiac failure, 18(6), 459–470. https://doi.org/10.1016/j.cardfail.2012.03.001

Tahlawi, M. E. (2020). Heart failure in pediatric patients. Bentham Science Publishers.

Tworetzky W, McElhinney DB, Brook MM, Reddy VM, Hanley FL, Silverman NH. Echocardiographic diagnosis alone for the complete repair of major congenital heart defects.J Am Coll Cardiol. 1999; 33:228–233