1. Pediatric Cardiovascular Surgery Service of São José do Rio Preto – Hospital de Base – São José do Rio Preto Medical School – São

Doença de Ebstein: o grau de disfunção do ventrículo direito e a conduta cirúrgica Ebstein’s disease: the level of right ventricular dysfunction and the surgical approach CLINICAL DATA Full-term female child, 2.8 kg born by c-section. First-time mother denied use of medications during pregnancy. The hospital discharge occurred two days after birth, with the child breastfeeding well and without signs or symptoms of congenital heart disease. At 6 months of age, the child was referred to our Service with a history of fatigue while breastfeeding, abundant sweating and tachycardia even at rest. With the diagnosis confirmed, therapy was initiated with diuretic and six-month returns were scheduled. With 3 years of life the child began to complain of mild fatigue on efforts and cyanosis with peripheral saturation of 80% in ambient air. On physical examination the patient was in good general condition, cyanotic +/4+, normotensive and with appropriate weight gain. The ictus cordis was normopositioned, normal heart sound and no murmurs. Abdomen normal. Well perfused extremities, pulses present and symmetrical. ELECTROCARDIOGRAM AND HOLTER Sinus rhythm with first-degree AV block, heart rate of 115 beats/min. SÂQRS between +90 ° and +180°, PR 200 ms, QRS 80 ms. Right axis deviation with right atrial overload (Figure 1). Holter ratified the electrocardiographic findings and showed presence of isolated supraventricular ectopy