Myocarditis is a challenging & potentially life-threatening disease associated with high morbidity

 "Myocarditis is a challenging & potentially life-threatening disease associated with high morbidity in some paediatric patients, due to its ability to present as an acute & fulminant disease & to ultimately progress to dilated cardiomyopathy"

Myocarditis in Paediatric Patients: Unveiling the Progression to Dilated Cardiomyopathy and Heart Failure

by 

Inês Teixeira Farinha

 ^1,* and

Joana Oliveira Miranda

 ^2,3

¹

Faculty of Medicine of Porto University, Porto 4200-319, Portugal

²

Department of Paediatric Cardiology, Centro Hospitalar São João, Porto 4200-319, Portugal

³

Department of Physiology and Cardiothoracic Surgery, Faculty of Medicine of Porto University, Porto 4200-319, Portugal

^*

Author to whom correspondence should be addressed.

Academic Editor: Joel D. Schilling

J. Cardiovasc. Dev. Dis. 2016, 3(4), 31; https://doi.org/10.3390/jcdd3040031

Received: 29 August 2016 / Revised: 29 October 2016 / Accepted: 3 November 2016 / Published: 8 November 2016

(This article belongs to the Special Issue Heart Failure Pathogenesis and Management)

Abstract

Myocarditis is a challenging and potentially life-threatening disease associated with high morbidity in some paediatric patients, due to its ability to present as an acute and fulminant disease and to ultimately progress to dilated cardiomyopathy. It has been described as an inflammatory disease of the myocardium caused by diverse aetiologies. Viral infection is the most frequent cause of myocarditis in developed countries, but bacterial and protozoal infections or drug hypersensitivity may also be causative agents. The prompt diagnosis in paediatric patients is difficult, as the spectrum of clinical manifestation can range from no myocardial dysfunction to sudden cardiac death. Recent studies on myocarditis pathogenesis have revealed a triphasic nature of this disease, which influences the diagnostic and therapeutic strategies to adopt in each patient. Endomyocardial biopsy remains the gold standard for diagnosing myocarditis, and several non-invasive diagnostic tools can be used to support the diagnosis. Intravenous immunoglobulin has become part of routine practice in the treatment of myocarditis in paediatric patients at many centres, but its true effect on the cardiac function has been the target of many studies. The aim of this review is to approach the recently discovered facets of paediatric myocarditis regarding its progression to dilated cardiomyopathy. 

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1. Introduction

Myocarditis is a non-familial form of inflammatory heart muscle disease, in the absence of predominant acute or chronic ischaemia [1,2,3]. It is, by definition, an inflammation of the myocardium, which may also extend to the pericardium and endocardium [4]. Diagnosis is established by histological, immunologic and immunohistochemical criteria, as it was defined by the World Health Organization (WHO)/International Society and Federation of Cardiology (ISFC), in 1995 [5]. It is an important cause of morbidity and mortality in children, due to its association with cardiac dysfunction and dilated cardiomyopathy (DCM), which may represent the chronic phase of the disease [5,6].

2. Aetiology

Acute myocarditis has multiple causes, including viruses, protozoa, bacteria, fungi, toxins, drugs, metabolic abnormalities, hypersensitivity reactions and systemic autoimmune diseases [7]. Often the underlying agent causing the disease is not identified [4].

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0. Conclusions

Myocarditis is a potentially life-threatening disease often clinically disguised as a benign one. Its tendency to occur in young patients makes it one of the most frequent causes of DCM in this age group. The amount of research made in experimental animal models in the last few decades has allowed a better understanding of this disease, particularly its triphasic nature. However, assessing an unambiguous prompt diagnosis of myocarditis and correctly staging it probably remains the hardest challenge for clinicians, as this information is useful to initiate a specific treatment and improve the outcome. Therefore, further clinical trials are needed in a quest for novel efficient diagnosis and treatment strategies, which could eventually lead to a reduction in paediatric morbidity and mortality.