Heart failure in children. Part I: clinical evaluation, diagnostic testing, and initial medical management

Abstract Current evidence suggests that almost half of allchildren with cardiomyopathy and symptomatic heartfailure will die or require a cardiac transplant within5 years of diagnosis. The recognition, diagnostic assess-ment, and treatment of heart failure in children aretherefore challenging undertakings, to say the least. Itinvolves an assessment ofcardiac appearance and function,adaptation of the child as a whole, and a diagnosticapproach that evaluates many possible root causes. Thisreview is intended to assist the practicing pediatrician andcardiologist by providing a framework for this diagnosticassessment and to give an overview of the treatmentoptions available for children with heart failure. In this firstpart, we will focus on clinical evaluation, diagnostictesting, and initial medical management. In the secondpart of this series, the maintenance treatment and treatmentoptions applicable when medical treatment is insufficientwill be addressed.Keywords Heartfailure.Cardiomyopathy.Treatment.ChildrenIntroductionHeart failure occurs in children as a consequence ofcongenital or acquired disorders, either systemic or involv-ing only the cardiovascular system. Herein, we review anapproach to the diagnosis and assessment of heart failure asa clinical syndrome in children. Acute-phase stabilizationand medical treatment are discussed. Later, in part 2 of thisseries, we will review the maintenance treatment andintervention options for advanced heart failure, such asdevice therapy and heart transplantation. We will deal withheart failure arising both in infancy and in childhood due toeither structural congenital or primarily cardiomyopathicdisease.Heart failure due to congenital structural heart diseasetypically presents early in life [3], resulting from abnormalcardiac chamber morphology, valvular function, or circula-tory connections. Genetically determined diseases of themyocardium (cardiomyopathies, CM) may occasionally beapparent at birth but more frequently manifest later ininfancy, childhood, or indeed during adult life. In CM, thebasis for heart failure is usually reduced systolic function ofthe left ventricle (LV), although associated diastolicdysfunction is increasingly recognized as an importantcontributing factor in the pathophysiology of heart failurein children [38]. The function of the right ventricle (RV),previously overlooked, has also come under scrutiny, as apotential predictor of adverse outcome in patients with heartfailure [13, 38].Disorders affecting the myocardium are diverse and mayarise from genetic abnormalities often involving sarcomericand structural proteins or can be secondary to an acquireddisease (like myocarditis) or toxic exposure (anthracyclinetoxicity). It is important to note that all known diagnoses