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Objective: Costello syndrome is an autosomal dominant disorder caused by mutations in HRAS gene, which produces a protein involved in controlling cell division and growth. It is a very rare clinical condition, with about 200 to 300 confirmed cases worldwide. One of the most serious manifestations is cardiac arrhythmia, potentially fatal. Objective: We propose an account of a clinically diagnosed case like Costello syndrome, and discuss the management of associated cardiac arrhythmia. Methods: Case report after chart review, and review of literature. Results: An infant of female, 1 year and 3 months old, with clinical diagnosis of Costello syndrome, was admitted for evaluation of cardiac arrhythmia. She presented hypertrophic cardiomyopathy in echocardiography,. The Holter exam revealed atrial and supraventricular tachycardia with aberrance, unstable left atrium, refractory cardiac arrhythmia and blocked atrial premature beats. It was decided to start amiodarone 5mg/kg/day and captopril 1mg/kg/day, with reassessment after two weeks of treatment. There was improvement in cardiac auscultation, but she maintained the ECG pattern. Due to the general prognosis of the patient, it was decided by outpatient treatment, and there was clinical and electrocardiographic improvement. Conclusion: There are no specific guidelines for the treatment of cardiac arrhythmias in Costello syndrome. Patients who develop this complication have a poorer prognosis, and it is needed to decide, together with the family, the best conduct.

Costello syndrome: Case report and review of diagnostic approach