P0635 - Cerebrovascular accident in a 9-month-old with cyanotic congenital heart disease

Introduction: In adults, cerebrovascular accidents (CVA) are often the result of atherosclerotic disease, underlying cardiac arrhythmias, or hypertension. CVA are rarely seen in pediatric patients, but cyanotic congenital heart disease (CHD) patients are at increased risk of a thromboembolic event. Cyanotic CHD patients with a single ventricle undergo multiple procedures in the palliation of their heart lesions including the Glenn procedure, a direct anastomosis of the superior vena cava to the pulmonary arteries. These procedures improve but do not resolve underlying systemic cyanosis, which leaves patients susceptible to polycythemia, anemia, and reduced end-organ perfusion at baseline. In addition, the underlying pathology of single-ventricle disease can result in increased pulmonary pressure, hypertension, and arrhythmias, which all contribute to the increased risk of cerebrovascular accidents. Patients with CHD have over 10 times the risk of CVA as their matched peers without cardiac lesions.

Case: We present the case of a 9-month-old male with a history of hypoplastic left heart syndrome (HLHS) status post Glenn repair (Figure 1) on aspirin who presented with abnormal repetitive movements of the right arm and leg for 24 hours. His family initially thought that his symptoms were due to a recent mechanical fall onto his head and right side, but CT head (Figure 2) in the Emergency Department showed a new left-sided middle cerebral artery stroke. On further workup, he was in sinus rhythm without evidence of deep vein thrombosis in any extremity, thrombus on echocardiogram, or embolus on magnetic resonance angiography. His jerking movements were characterized as seizures and resolved with initiation of antiepileptic drugs (AEDs). Although he was found to be a carrier of a von Willebrand disease variant, his cardiac lesion is thought to be the primary cause of his CVA. He now requires long-term anticoagulation and AED use, though he has made meaningful progress towards recovery.

Discussion: While adults with CVA often present with focal neurologic deficits, infants and children can present with more subtle symptoms such as seizures or diminished movements on one side of the body. As a result, cyanotic pediatric patients experiencing symptoms of CVA may present later or with nonspecific symptoms that delay treatment. Treatment delays may result in increased neurologic damage when combined with their underlying risk factors. Given that more patients with cyanotic heart lesions are living to adulthood, CVA should be considered in children and adults with cyanotic CHD who present with neurologic symptoms.

Conclusion: Pediatric patients with cyanotic heart disease have an increased risk of CVA. These patients may present later in their course and with more subtle symptoms than adults, requiring a high suspicion to avoid delays in diagnosis and management.