Successful preventive treatment of congenital heart block during pregnancy in a woman with systemic lupus erythematosus and anti-Sjgren's syndrome A/Ro antibody

Chung-Han Yang, Ji-Yih Chen, Shih-Ching Lee, Shue-Fen Luo
Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Chang Gung Memorial Hospital, Tao-yuan County, Taiwan

Received: May 17, 2004 Revised: April 1, 2005 Accepted: April 19, 2005

Corresponding author: Dr. Shue-Fen Luo, MD, Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Chang Gung Memorial Hospital, 5 Fu-shin St, Kwei-san Hsiang, Tao-yuan County 333, Taiwan. E-mail:

Congenital heart block (CHB) that is a manifestation of neonatal lupus syndrome (NLS) carries a poor prognosis. The treatment response of established heart block in NLS is usually unsatisfactory. Preventive treatment during pregnancy, however, before the critical period of cardiac development, can prevent the development of CHB. A Taiwanese woman with systemic lupus erythematosus (SLE) was positive for anti-Sjgren's syndrome A (SSA)/Ro antibody. Her first pregnancy resulted in intra-uterine fetal death. Her second pregnancy resulted in CHB, despite dexamethasone treatment, and neonatal death at age 1 day despite pacemaker implantation. During her third pregnancy, dexamethasone was given starting at week 10, azathioprine at week 18, and plasmapheresis was performed every other day for 5 times starting at week 20 of gestation. Cesarean section was performed due to oligohydramnion at week 31 of gestation and a healthy girl was delivered. This case suggests that judicious use of fluorinated glucocorticoids, immunosuppressants, and plasmapheresis may prevent development of CHB in pregnant women with SLE who are anti-SSA/Ro antibody positive and have previous children with CHB.

Key words: Congenital heart defects, heart block, plasmapheresis, SS-A antibodies, systemic lupus erythematosus

J Microbiol Immunol Infect 2005;38:365-369.