Heart failure in children: Etiology, clinical manifestations, and diagnosis
- Rakesh K Singh, MD, MS
Rakesh K Singh, MD, MS
- Associate Professor of Pediatrics
- University of California, San Diego
- TP Singh, MD, MSc
TP Singh, MD, MSc
- Associate Professor of Pediatrics
- Harvard Medical School
Heart failure (HF) results from structural or functional cardiac disorders that impair the ability of the ventricle(s) to fill with and/or eject blood. The presentation of pediatric HF is diverse because of the numerous underlying cardiac etiologies (table 1) and varying clinical settings.
The etiology, clinical manifestations, and diagnostic evaluation of HF in children are reviewed here. The management of HF in children is discussed separately. (See "Heart failure in children: Management".)
In the United States, HF is estimated to affect 12,000 to 35,000 children below the age of 19 years in the United States each year [1,2]. HF-related hospitalizations account for approximately 11,000 to 14,000 hospitalizations in children per year in the United States .
PATHOPHYSIOLOGY AND ETIOLOGY
The causes of pediatric HF can be divided into pathophysiologic categories (table 1). This categorization helps in the understanding of the underlying physiology and clinical manifestations of the different causes of pediatric HF, and guides the approach to management. It is important to recognize however, that these categories may overlap in some patients (eg, volume overload and pressure overload can be associated with ventricular dysfunction). (See "Heart failure in children: Management".)
●Ventricular dysfunction − Ventricular dysfunction leads to impaired ejection of blood from the ventricle. Unless specified, ventricular dysfunction implies systolic dysfunction (reduced ventricular contractility). Ventricular diastolic dysfunction implies impaired ventricular filling and noncompliance with abnormally steep pressure-volume relationship resulting in high ventricular filling pressures. Ventricular dysfunction (systolic or diastolic) can occur in children with congenital heart disease (CHD) and in those with structurally normal hearts. Children with CHD may have ventricular dysfunction at presentation, but more commonly develop dysfunction and HF several years (or even decades) following surgical repair of their cardiac defect (ie, "burnt-out" CHD). (See 'Ventricular dysfunction' below.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Hsu DT, Pearson GD. Heart failure in children: part I: history, etiology, and pathophysiology. Circ Heart Fail 2009; 2:63.
- Rossano JW, Kim JJ, Decker JA, et al. Prevalence, morbidity, and mortality of heart failure-related hospitalizations in children in the United States: a population-based study. J Card Fail 2012; 18:459.
- Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348:1647.
- Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348:1639.
- Canter CE, Simpson KE. Diagnosis and treatment of myocarditis in children in the current era. Circulation 2014; 129:115.
- Raj S, Franco VI, Lipshultz SE. Anthracycline-induced cardiotoxicity: a review of pathophysiology, diagnosis, and treatment. Curr Treat Options Cardiovasc Med 2014; 16:315.
- Book WM. Heart failure in the adult patient with congenital heart disease. J Card Fail 2005; 11:306.
- Kirk R, Dipchand AI, Rosenthal DN, et al. The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary. [Corrected]. J Heart Lung Transplant 2014; 33:888.
- Criteria Committee of the New York Heart Association. Nomenclature and Criteria for Diagnosis of Diseases of the Heart and Great Vessels, 9th ed, Little, Brown & Co., Boston 1994.
- Ross RD, Bollinger RO, Pinsky WW. Grading the severity of congestive heart failure in infants. Pediatr Cardiol 1992; 13:72.
- Ross RD. The Ross classification for heart failure in children after 25 years: a review and an age-stratified revision. Pediatr Cardiol 2012; 33:1295.
- Ross RD, Daniels SR, Schwartz DC, et al. Plasma norepinephrine levels in infants and children with congestive heart failure. Am J Cardiol 1987; 59:911.
- Wu JR, Chang HR, Huang TY, et al. Reduction in lymphocyte beta-adrenergic receptor density in infants and children with heart failure secondary to congenital heart disease. Am J Cardiol 1996; 77:170.
- Hollander SA, Addonizio LJ, Chin C, et al. Abdominal complaints as a common first presentation of heart failure in adolescents with dilated cardiomyopathy. Am J Emerg Med 2013; 31:684.
- Hsu DT, Pearson GD. Heart failure in children: part II: diagnosis, treatment, and future directions. Circ Heart Fail 2009; 2:490.
- Satou GM, Lacro RV, Chung T, et al. Heart size on chest x-ray as a predictor of cardiac enlargement by echocardiography in children. Pediatr Cardiol 2001; 22:218.
- Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 2005; 18:1440.
- Sluysmans T, Colan SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol (1985) 2005; 99:445.
- Boston Children's Hospital Z-score calculator. The Boston Children’s Hospital z-score system is based on data gathered over 12 years on normal children. Available at: http://zscore.chboston.org/.
- Favilli S, Frenos S, Lasagni D, et al. The use of B-type natriuretic peptide in paediatric patients: a review of literature. J Cardiovasc Med (Hagerstown) 2009; 10:298.
- Neves AL, Henriques-Coelho T, Leite-Moreira A, Areias JC. The Utility of Brain Natriuretic Peptide in Pediatric Cardiology: A Review. Pediatr Crit Care Med 2016; 17:e529.
- Cantinotti M, Law Y, Vittorini S, et al. The potential and limitations of plasma BNP measurement in the diagnosis, prognosis, and management of children with heart failure due to congenital cardiac disease: an update. Heart Fail Rev 2014; 19:727.
- Eindhoven JA, van den Bosch AE, Boersma E, Roos-Hesselink JW. The usefulness of brain natriuretic peptide in simple congenital heart disease - a systematic review. Cardiol Young 2013; 23:315.
- Mir TS, Marohn S, Läer S, et al. Plasma concentrations of N-terminal pro-brain natriuretic peptide in control children from the neonatal to adolescent period and in children with congestive heart failure. Pediatrics 2002; 110:e76.
- Ko HK, Lee JH, Choi BM, et al. Utility of the rapid B-type natriuretic peptide assay for detection of cardiovascular problems in newborn infants with respiratory difficulties. Neonatology 2008; 94:16.
- Koulouri S, Acherman RJ, Wong PC, et al. Utility of B-type natriuretic peptide in differentiating congestive heart failure from lung disease in pediatric patients with respiratory distress. Pediatr Cardiol 2004; 25:341.
- Law YM, Hoyer AW, Reller MD, Silberbach M. Accuracy of plasma B-type natriuretic peptide to diagnose significant cardiovascular disease in children: the Better Not Pout Children! Study. J Am Coll Cardiol 2009; 54:1467.
- Maher KO, Reed H, Cuadrado A, et al. B-type natriuretic peptide in the emergency diagnosis of critical heart disease in children. Pediatrics 2008; 121:e1484.
- Law YM, Ettedgui J, Beerman L, et al. Comparison of plasma B-type natriuretic peptide levels in single ventricle patients with systemic ventricle heart failure versus isolated cavopulmonary failure. Am J Cardiol 2006; 98:520.
- Lowenthal A, Camacho BV, Lowenthal S, et al. Usefulness of B-type natriuretic peptide and N-terminal pro-B-type natriuretic peptide as biomarkers for heart failure in young children with single ventricle congenital heart disease. Am J Cardiol 2012; 109:866.
- Hayakawa H, Komada Y, Hirayama M, et al. Plasma levels of natriuretic peptides in relation to doxorubicin-induced cardiotoxicity and cardiac function in children with cancer. Med Pediatr Oncol 2001; 37:4.
- Pinarli FG, Oğuz A, Tunaoğlu FS, et al. Late cardiac evaluation of children with solid tumors after anthracycline chemotherapy. Pediatr Blood Cancer 2005; 44:370.
- Aggarwal S, Pettersen MD, Bhambhani K, et al. B-type natriuretic peptide as a marker for cardiac dysfunction in anthracycline-treated children. Pediatr Blood Cancer 2007; 49:812.
- Holmström H, Hall C, Thaulow E. Plasma levels of natriuretic peptides and hemodynamic assessment of patent ductus arteriosus in preterm infants. Acta Paediatr 2001; 90:184.
- Sugimoto M, Manabe H, Nakau K, et al. The role of N-terminal pro-B-type natriuretic peptide in the diagnosis of congestive heart failure in children. - Correlation with the heart failure score and comparison with B-type natriuretic peptide -. Circ J 2010; 74:998.
- Mangat J, Carter C, Riley G, et al. The clinical utility of brain natriuretic peptide in paediatric left ventricular failure. Eur J Heart Fail 2009; 11:48.
- Price JF, Thomas AK, Grenier M, et al. B-type natriuretic peptide predicts adverse cardiovascular events in pediatric outpatients with chronic left ventricular systolic dysfunction. Circulation 2006; 114:1063.
- Nasser N, Perles Z, Rein AJJT, Nir A. NT-proBNP as a marker for persistent cardiac disease in children with history of dilated cardiomyopathy and myocarditis. Pediatr Cardiol 2006; 27:87.
- Auerbach SR, Richmond ME, Lamour JM, et al. BNP levels predict outcome in pediatric heart failure patients: post hoc analysis of the Pediatric Carvedilol Trial. Circ Heart Fail 2010; 3:606.
- Sachdeva S, Song X, Dham N, et al. Analysis of clinical parameters and cardiac magnetic resonance imaging as predictors of outcome in pediatric myocarditis. Am J Cardiol 2015; 115:499.
- Soongswang J, Durongpisitkul K, Nana A, et al. Cardiac troponin T: a marker in the diagnosis of acute myocarditis in children. Pediatr Cardiol 2005; 26:45.
- Goldberg JF, Shah MD, Kantor PF, et al. Prevalence and Severity of Anemia in Children Hospitalized with Acute Heart Failure. Congenit Heart Dis 2016; 11:622.
- Price JF, Kantor PF, Shaddy RE, et al. Incidence, Severity, and Association With Adverse Outcome of Hyponatremia in Children Hospitalized With Heart Failure. Am J Cardiol 2016; 118:1006.
- Attili AK, Parish V, Valverde I, et al. Cardiovascular MRI in childhood. Arch Dis Child 2011; 96:1147.
- PATHOPHYSIOLOGY AND ETIOLOGY
- Ventricular dysfunction
- - Structurally normal heart
- - Congenital heart disease
- Volume overload with preserved ventricular contractility
- Pressure overload with preserved ventricular contractility
- STAGING AND SEVERITY
- CLINICAL MANIFESTATIONS
- Physical examination
- DIAGNOSTIC EVALUATION
- Unstable patients
- Chest radiography
- Laboratory tests
- Additional evaluation
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS