Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Transvenous Permanent Pacemaker Implantation in 14 Month Old baby with Congenital Complete Heart Block.

Congenital Complete Heart Block in utero has been diagnosed more frequently with the clinical use of fetal echocardiography. The fetus with complete heart block may remain asymptomatic or develop congestive heart failure. Pacemaker therapy in children involves unique issues regarding patient size, growth, development and possible presence of congenital heart disease. Historically, epicardial pacing was more common in children. As technology has improved, generators and leads have become smaller and more advanced, allowing transvenous pacing system in children. Pacemaker therapy is even technically feasible in infants and neonates. We present a 14mth old baby of just 7.5kg who presented to us with Adams Stokes and subtle signs of congestive heart failure that after stabilization was implanted a transvenous MRI compatible permanent pacemaker (VVIR).

Outcome of pregnant mothers with systemic lupus erythematosus (focusing on congenital heart block) / 소아과

PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

A case of neonatal lupus syndrome with congenital complete heart block / 대한산부인과학회잡지

The neonatal lupus syndrome is characterized by skin lesions, hepatic and hematologic abnormalities and congenital heart block. Congenital heart block which is believed to be caused by transplacental passage of the anti-Ro (SSA)/La (SSB) antibodies from mother to infant, is known to occur in 1 in 20,000 live births. In contrast to other manifestation of neonatal lupus syndrome, which usually subside within 6 months after birth, congenital heart block is a permanent and potentially fatal complication. We experienced a case of neonatal lupus syndorme with congenital complete heart block in a newborn of asyptomatic mother with anti-Ro (SSA)/ La (SSB) antibodies.

A Case of Congenital Complete Heart Block

In complete heart block(CHB), there is a complete failure of the atrial impulse leading into a ventricular response, the atria and ventricles beat independently, with the latter having a slower rate. Approximately one-third of infants with congenital CHB have associated structural heart disease such as corrected transposition of the great arteries, single ventricle, and the heterotaxy syndrome. For those patients without associated anomalies in whom bradycardia causes heart failure unresponsive to drugs, a pacemaker is necessary. We report a case of congenital complete heart block showing a ventricular beat of 57 /min despite atrial beat of 125/min. The mother of the baby was asymptomatic but her serologic tests were positive for connective tissue disease. The patient had positive serologic tests for anti-Ro antibody and anti-La antibody but associated structural heart anomalies were not found. He remained well without signs and symptoms of heart failure and no treatment was required except 02 supply.

Two Cases of Neonatal Arrhythmia Observed by Fetal Echocardiography / 대한주산의학회잡지

M-mode, pulsed Doppler and Doppler color flow mapping, in addition to two-dimensional echocardiography, have greatly improved imaging of the fetal heart through identification of abnormal cardiac anatomy and rhythm in utero. The early detection of cardiac disturbance in utero permits alteration in obstetric management such as delivery in a high-risk center for optimal neonatal care and/or decision in optimal delivery time. We report two cases of the neonatal arrhy-thmia which were observed by fetal echocardiography. In the first case, female baby showed neonatal arrhythmias including tachycardia and brady-cardia until 3 days after birth, and then turned to bradyarrhythmia due to non-conducted atrial bigeminy. These events lead us to review the fetal echocardiographs of the patient carefully. Premature atrial contractions were observed in her fetal echocardiography. At 2 months after birth, the patient's electrocardiogram showed normal sinus rhythm. Severe neonatal bradycardia of the second case was due to congenital complete heart block, identified clearly by electrocardiogram after birth. This case also showed complete heart block in her fetal echocardiography. After insertion of the temporary pacemaker, cardiomegaly was improved. Both the neonate and the mother had positive anti-SSA/Ro autoantibody. But any other symptoms and signs of neonatal lupus did not appear in the neonate. Patient's mother also did not show any symptoms and signs of systemic lupus erythematosus. Since the prognosis depends upon the cause of bradyarrhythmia in fetus and neonates, differential diagnosis is important in obstetric management and optimal neonatal treatment.