An infant with suspected right ventricular dysplasia presenting unique ventriculograms

Abstract

Summary:Angiocardiography was performed twice in a male infant at ten days after birth due to cyanosis during crying and at 4 years and 10 months of age for close examination of the heart prior to an operation for cerebral abscess. Outpouchings resembling multiple diverticula were observed in both ventricles, though more notably in the right ventricle, as well as slight enlargement and reduced ejection fraction of both ventricles. Evaluation of oxygen saturation indicated right-to-left shunt at the atrial level. Electrocardiography suggested a marked superior axis deviation, and chest roentgenography and twodimensional echocardiography revealed enlargement of the right atrium and right ventricle. Soon after the examinations, the patient died suddenly during sleep at home. Since autopsy was not performed, a definitive diagnosis could not be made, but right ventricular dysplasia appeared likely. The unique angiocardiographic findings and clinical course of this rare patient are described.

Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction

“…5 There are rare reports of patients presenting with marked right to left shunt, cyanosis, and cerebral abscess without any arrhythmia. 6 Our patient also had cyanosis and clubbing without any history of ventricular arrhythmias. Treatment options for serious arrhythmias include electrical ablations and implantable cardioverter defibrillator.…”