Late Presentation of Double Inlet Right Ventricle Complicated by Infective Endocarditis and Neurological Sequelae: A Case Report

Abstract

Delayed diagnosis of cyanotic congenital heart disease may lead to devastating cardiovascular and neurological complications. Cerebral embolism in infective endocarditis (IE) is often linked to a delayed congenital heart disease (CHD) diagnosis. This case highlights the complications of delayed CHD detection, focusing on an 8-year-old boy presented with recurrent focal seizures, previous episode of left-sided hemiparesis, dyspnea, untreated dental infections, central cyanosis, clubbing, and a holosystolic murmur. Diagnostic examination revealed double inlet right ventricle (DIRV) with single-ventricle physiology, a large ventricular septal defect (VSD) with right-to-left shunt, severe pulmonary stenosis, possible infective endocarditis, and valve vegetation. Brain magnetic resonance imaging (MRI) demonstrated a cerebral abscess and chronic cerebral infarct. The patient was treated with a 6-week antibiotic regimen and was scheduled for bi-directional cavo-pulmonary shunt (BCPS) surgery. Children presenting with unexplained cyanosis or neurological manifestations should undergo comprehensive cardiac evaluation to facilitate early diagnosis and prevent severe complications.