Clinical presentation and early predictors for poor outcomes in pediatric myocarditis: A retrospective study.

Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. The initial diagnosis of myocarditis is usually based on clinical presentation, but this widely ranges from the severe sudden onset of a cardiogenic shock to asymptomatic patients. Early recognition is essential in order to monitor and start supportive treatment prior to the development of severe adverse events. Of note, many cases of fulminant myocarditis are usually misdiagnosed as otherwise minor conditions during the weeks before the unexpected deterioration. To provide diagnostic clues to make an early recognition of pediatric myocarditis. To investigate early predictors for poor outcomes. We conducted a retrospective cross-sectional single-center study from January 2008 to November 2017 at the Pediatric Department of our institution, including children A total of 42 patients [69% male; median age of 8 (1.5-12) years] met study inclusion criteria. Chest pain (40%) was the most common specific cardiac symptom. Respiratory tract symptoms (cough, apnea, rhinorrhea) (38%), shortness of breath (35%), gastrointestinal tract symptoms (vomiting, abdominal pain, diarrhea) (33%), and fever (31%) were the most common non-cardiac initial complaints. Tachycardia (57%) and tachypnea (52%) were the most common signs on the initial physical exam followed by nonspecific signs of respiratory tract infection (44%) and respiratory distress (35%). Specific abnormal signs of heart failure such as heart murmur (26%), systolic hypotension (24%), gallop rhythm (20%), or hepatomegaly (20%) were less prevalent. Up to 43% of patients presented an early poor outcome, and 16% presented a late poor outcome. In multivariate analysis, an initial left ventricular ejection fraction (LVEF) The diagnosis of myocarditis in children is challenging due to the heterogeneous and unspecific clinical presentation. The presence of LVE

Role of the Renin-Angiotensin-Aldosterone System in Dystrophin-Deficient Cardiomyopathy

Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin-angiotensin-aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration.Ye