A Case of Congenital Long OT Syndrome with Pseudo - Atrioventricular Block

The congenital long-QT syndrome (LQTS) is characterized by recurrent syncope, prolonged QT intervals, QT interval lability, polymorphic ventricular tachycardia, and sudden death. We report a case of congenital long QT syndrome in a 28-day-old male infant who presented with syncope, bradycardia with 2: 1 pseudo-atrioventricular block and a markedly prolonged QT inteval. One episode occured after crying and degenerated into ventricular fibrillation and terminated after cardioversion. A VVI type cardiac pacemaker was implanted. Subsequently, the infant's heart rate was over 110/min and 2: 1 AV block and any other arrhythmia were absent. The infant recovered from the accompanied pneumonia and sepsis and was discharged 47 days after adrnission. However, 13 days after discharge, the infant returned to our hospital ER with syncope. Ventricular fibrillation ceased after cardioversion. Despite medication with propranolol, ventricular tachycardia persisted. The infant expired the day after he was discharged against medical advice.

A Case of Neonatal Lupus Syndrome with Congenital Heart Block

Neonatal lupus erythematous is a rare syndrome, which is characterized by a transient lupus dermatitis, hematologic abnormalities and isolated congenital heart block. We detected the atrioventricular dissociation, bradycardia and pericardial effusion by fetal echocardiography in a female fetus at 25th weeks of gestational age. The baby was born in the 38th week of pregnancy by Cesarean section with 1790 gram of body weight. In spite of atrioventricular dissociation with bradycardia and pericardial effusion, cardiac pacemaker was not needed during neonatal period because she was doing well, no evidence of congestive heart failure and around 80 beat per minute of heart rate. The serologic markers for diagnosis of neonatal lupus are the autoantibodies specific to SS-A/Ro and/or SS-B/La. These antibodies are produced by the mother and passed to the fetus through the placenta. Autoantibodies of cytoplasmic SS-A antigens or SS-B antigen were found in the blood of this patient and her mother. We report a case of neonatal lupus syndrome with congenital atrioventricular dissociation with Rt. Bundle branch block and hematologic abnormality.