Primary cardiac fibroma in infants: A case report and review of cases of cardiac fibroma managed through orthotopic heart transplant.

Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.

Risk-Stratification Strategy for Sudden Cardiac Death in the Very Young Children with Asymptomatic Ventricular Preexcitation.

Asymptomatic VPE refers to the presence of this abnormal ECG pattern in the absence of any symptoms. The natural history in these patients is usually benign, and most children (60%) with VPE are usually asymptomatic. However, Sudden Cardiac Death (SCD) has been reported to be the initial symptom in many patients too. The increased risk of SCD is thought to be due to the rapid conduction of atrial arrhythmias to the ventricle, via the AP, which degenerates into Ventricular Fibrillation (VF). The best method to identify high-risk patients with asymptomatic VPE for SCD is the characterization of the electrophysiological properties of the AP through an Electrophysiological Study (EPS). Also, catheter ablation of the AP with radiofrequency as definitive treatment to avoid SCD can be performed by the same procedure with high rates of success. However, the uncertainty over the absolute risk of SCD, the poor positive predictive value of an invasive EPS, and complications associated with catheter ablation have made the management of asymptomatic VPE challenging, even more in those children younger than 8-year-old, where there are no clear recommendations. This review provides an overview of the different methods to make the risk stratification for SCD in asymptomatic children with, as well as our viewpoint on the adequate approach to those young children not included in current guidelines.

N-terminal probrain natriuretic peptide as biomarker for diagnosis of Kawasaki disease.

Kawasaki disease (KD) is a systemic childhood vasculitis with peculiar tropism for the heart. Coronary artery aneurysms are the primary cause of morbidity and mortality in these patients. The timely administration of gammaglobulin decreases the risk for development of coronary artery aneurysms, highlighting the importance of early KD recognition. However, the most significant dilemma in the management of KD is the diagnosis itself. In this article, we review the recent literature focusing on the diagnostic utility of N-terminal probrain natriuretic peptide as a biomarker for diagnosis of KD. The main conclusion is that N-terminal probrain natriuretic peptide is an useful biomarker for KD diagnostic that represents a valuable addition to the current diagnostic workup of patients with suspected KD, increasing the diagnostic accuracy.

Atypical Presentation of Incomplete Kawasaki Disease: A Peripheral Facial Nerve Palsy.

BACKGROUND: Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1-30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%). CASE REPORT: We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD. The initial diagnosis was not made during the first 10 days of illness (therapeutic window for immunoglobulin treatment) as he was suspected to have hand-mouth-foot disease. We believe that both the delay in diagnosis and treatment of an atypical presentation of KD, combined with the more severe vasculitis and inflammatory burden reported in these cases, contributed to the development of CAA in our patient. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the importance of considering KD diagnosis in children with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP. Atypical cases like this may be at increased risk of CAA because of delayed diagnosis and a higher inflammatory burden; therefore, a more aggressive treatment approach may be necessary.