Primary endocardial fibroelastosis presenting in a young child as incessant ventricular tachycardia and dilated cardiomyopathy.

Endocardial fibroelastosis is a cardiomyopathy not commonly seen in the present age. We describe the case of a 4-year-old girl who presented with sustained incessant ventricular tachycardia, a severely dilated left ventricle, and cardiac dysfunction refractory to all medical management and even to ablation; she eventually underwent cardiac transplantation. The diagnosis was made only after histopathologic examination of the explanted heart showed clear evidence of endocardial fibroelastosis. Through this report, we would like to highlight the fact that primary endocardial fibroelastosis can masquerade as idiopathic dilated cardiomyopathy and that associated frequent premature ventricular contractions and nonsustained ventricular tachycardia require close monitoring. Progressive ventricular dilation and ventricular dysfunction can convey a poor prognosis. Sustained recalcitrant ventricular tachycardia in these patients can be a life-threatening event that requires emergent mechanical support and heart transplantation.

Successful Ablation of Antero-septal Accessory Pathway in the Non-Coronary Cusp in a Child.

A 15-year-old boy with Wolff-Parkinson-White syndrome underwent an electrophysiology study for symptoms of palpitations and persistence of pre-excitation during peak exercise. He was detected to have right antero-septal accessory pathway with relatively long effective refractory period and no inducible tachycardia. He had only transient normalization with cryoablation. Eight months later, he presented again with two episodes of seizures with preceding palpitations. Neurology evaluation was unremarkable with a normal electroencephalogram. In view of his symptoms in association with evidence of pre-excitation, he underwent a second electrophysiology study with ablation. Cryoablation in the anterior septum again achieved only transient normalization. Mapping in the non-coronary cusp identified an earliest accessory pathway potential. RF ablation was performed in the non-coronary cusp with immediate normalization of his electrocardiogram. At 6 month follow-up, he continues to have no pre-excitation on his EKG. Ablation of the anteroseptal accessory pathway in the non-coronary cusp can be safely performed in patients' refractory to conventional ablation sites and techniques.

Central venous catheter-associated pericardial tamponade in a 6-day old: a case report.

Introduction. Pericardial effusion (PCE) and tamponade can cause significant morbidity and mortality in neonates. Such cases have been reported in the literature in various contexts. Case Presentation. A 6-day old neonate with meconium aspiration syndrome and persistent pulmonary hypertension of newborn on high frequency oscillator ventilation and inhaled nitric oxide was referred to our hospital with a large pericardial effusion causing hemodynamic compromise. Prompt pericardiocentesis led to significant improvement in the cardio-respiratory status and removal of the central line prevented the fluid from reaccumulating. Cellular and biochemical analysis aided in the diagnosis of catheter related etiology with possibility of infusate diffusion into the pericardial space. Conclusion. We present this paper to emphasize the importance of recognizing this uncommon but serious complication of central venous catheters in intensive care units. We also discuss the proposed hypothesis for the mechanism of production of PCE.