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Current State of Pediatric Heart Failure.

ABSTRACT: Pediatric heart failure (HF) represents an important cause of morbidity and mortality in childhood. There is an overlapping relationship of HF, congenital heart disease, and cardiomyopathy. The goal of treatment of HF in children is to maintain stability, prevent progression, and provide a reasonable milieu to allow somatic growth and optimal development. Current management and therapy for HF in children are extrapolated from treatment approaches in adults. There are significant barriers in applying adult data to children because of developmental factors, age variation from birth to adolescence, and differences in the genetic expression profile and β-adrenergic signaling. At the same time, there are significant challenges in performing well-designed drug trials in children with HF because of heterogeneity of diagnoses identifying a clinically relevant outcome with a high event rate, and a difficulty in achieving sufficient enrollment. A judicious balance between extrapolation from adult HF guidelines and the development of child-specific data on treatment represent a wise approach to optimize pediatric HF management. This approach is helpful as reflected by the increasing role of ventricular assist devices in the management of advanced HF in children. This review discusses the causes, epidemiology, pathophysiology, clinical manifestations, conventional medical treatment, clinical trials, and the role of device therapy in pediatric HF.

SUBMITTER: Das BB

PROVIDER: S-EPMC6069285 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature

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Current State of Pediatric Heart Failure.

Das Bibhuti B BB

Children (Basel, Switzerland) 20180628 7

Pediatric heart failure (HF) represents an important cause of morbidity and mortality in childhood. There is an overlapping relationship of HF, congenital heart disease, and cardiomyopathy. The goal of treatment of HF in children is to maintain stability, prevent progression, and provide a reasonable milieu to allow somatic growth and optimal development. Current management and therapy for HF in children are extrapolated from treatment approaches in adults. There are significant barriers in appl ...[more]

PMID: 29958420

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Project description:AimsFabry disease (FD) is often associated with heart failure (HF). However, data on HF prevalence, prognosis, and applicability of echocardiographic criteria for HF diagnosis in FD remain uncertain.Methods and resultsWe evaluated patients with genetically proven FD for symptoms and natriuretic peptides indicating HF. We then analysed the diagnostic utility of the currently recommended European Society of Cardiology (ESC) echocardiographic criteria for HF diagnosis and their relationship to natriuretic peptides. Finally, we examined the association between HF and echocardiographic criteria with mortality and cardiovascular events during follow-up. Of 116 patients with FD, 48 (41%) had symptomatic HF (mean age 58 ± 11 years, 62% male). HF with preserved ejection fraction (HF-pEF) was diagnosed in 43 (91%) patients, representing the dominant phenotype. Left ventricular mass index (LVMi) had the highest diagnostic utility (sensitivity 71% and specificity 83%) for HF diagnosis in FD, followed by E/e' > 9 (sensitivity 76% and specificity 78%) and global longitudinal strain (GLS) <16% (sensitivity 54% and specificity 88%). Log N-terminal pro-brain natriuretic peptide correlated significantly with LVMi (r = 0.60), E/e' (r = 0.54), and GLS (r = 0.52) (all Ps < 0.001) but not with left ventricular ejection fraction (r = -0.034, P = 0.72). During follow-up (mean 1208 ± 444 days), patients diagnosed with HF had a higher rate of all-cause mortality and worsening HF (33% vs. 1.5%, P < 0.001). Abnormal LVMi, E/e' > 9, and GLS < 16% were all associated with higher all-cause mortality and worsening HF.ConclusionsThis study found a high prevalence of symptomatic HF in FD patients. HF-pEF was the dominant phenotype. LVMi, E/e', and GLS yielded the highest diagnostic utility for HF diagnosis and were significantly correlated with natriuretic peptides levels. Echocardiographic criteria proposed by current ESC HF guidelines apply to Fabry patients and predict cardiovascular events. At follow-up, Fabry patients with HF diagnosis had high event rates and significantly worse prognosis than patients without HF.

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Current State of Pediatric Heart Failure.

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Current State of Pediatric Heart Failure.

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