Describe an unusual presentation of adult congenital heart disease.

Patients with cyanotic congenital heart disease are at higher risk for neurologic complications, including stroke and cerebral abscess. However, neurologic sequelae are rarely the presenting symptom in these patients.

Case report

A 38 year-old previously healthy male presented with four days of brief spells of right arm and face marching paresthesias and twitching. He migrated from Ethiopia ten years prior, with minimal access to medical care. He was afebrile, but oxygen saturation was 85%. On examination, he had finger clubbing, a right ventricular heave and palpable P2, and a 2/6 systolic murmur at the left sternal border. Neurologic examination revealed weakness of his right wrist and finger extensors and hyperreflexia of his right side.

Laboratory evaluation revealed an elevated hemoglobin of 19.9 g/dL. Chest x-ray demonstrated an enlarged right ventricle and increased prominence of the pulmonary arteries. Head CT demonstrated a 2.2 cm left postcentral gyrus mass with surrounding vasogenic edema. Brain MRI noted internal diffusion restriction, suspicious for a cerebral abscess. Transthoracic echocardiogram demonstrated a large ventricular septal defect with Eisenmenger physiology.

Blood cultures were drawn, and the patient was started on levetiracetam and empiric antibiotics. He subsequently underwent a craniotomy with aspiration and irrigation. The surgical aspirate grew streptococcus anginosus. His antibiotics were narrowed to IV penicillin G and oral metronidazole to complete a six-week course, and his right hemiparesis gradually improved over this time.

Our patient presented with focal seizures secondary to a cerebral abscess, and was found to have underlying cyanotic congenital heart disease with the presence of a right-to-left shunt. Neurologists should be aware of the neurologic complications associated with cyanotic congenital heart disease and be able to recognize common laboratory, imaging, and physical exam findings in this patient population.