Ampliação de aorta ascendente com a técnica de Brom em paciente com síndrome de Williams-Beuren Enlargement of the ascending aorta with Brom technique in a patient with Williams-Beuren syndrome

CLINICAL DATAFull-term male baby at 2.49 kg from Limeira do Oeste,Minas Gerias, Brazil. Mother with 32 years of age who usedcarbamazepine. In the neonatal period, the baby wasdiagnosed with genetic disorder possibly due to constanthypercalcemia, congenital heart disease, right inguinalhernia and fimosis. The baby had follow-up visits with acardiologist until 7 years and 9 months of age, at whichpoint he was at 27 kg and when he started to present fatigueduring moderate and great efforts. The patient was referredto surgery, and also presented difficulties with reading andwriting from a mild cognitive defect; he was very kind, andhad a small nose and full lips (due to his syndrome).Physical examination revealed a good condition of thepatient: eutrophic, afebrile, acyanotic. Regular cardiacrhythm, high murmur +++/6+ at left and right sternal margin,without opening snap and palpable fremitus in the sternalfurcula. Normal pulmonary auscultation. Weak abdomen,liver at 1cm from the right costal edge, no palpable spleen,presence of normal hydro-aereal sounds without abdominalmurmurs. Palpable and symmetric pulses in the extremities.ELECTROCARDIOGRAMSinus arrhythmia, heart rate of 72bpm, SÂP +60o, SÂQRS-30°, PR 0.16s, QTC 0.42s. Left atrial overload, conductiondisorder in the right branch evidenced at V2. RSR’ patternwith QRS duration of 0.10s, in addition to an alteration ofthe anterior septal ventricular repolarization (Figure 1).RADIOGRAMVisceral