Neonatal lupus with left bundle branch block and cardiomyopathy: a case report (original) (raw)
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In-utero Presentation of Complete Atrio-ventricular Block - Not Always Neonatal Lupus!
British Biomedical Bulletin, 2014
Complete atrio-ventricular block (CAVB) presenting in-utero, as widely known, is secondary to neonatal lupus unless proved otherwise. We hereby report a neonate with CAVB associated with heart defects presenting in-utero, without any evidence of neonatal lupus. Hence, we emphasize the association of heart defects in CAVB as the leading factor in diagnosing the etiology.
Congenital heart block in neonatal lupus: The pediatric cardiologist’s perspective
The Indian Journal of Pediatrics, 2002
Clinical presentation. Congenital heart block (CHB) in the absence of major structural abnormalities is associated with maternal antibodies to Ro (SS-A) and La (SS-B). CHB is most commonly diagnosed between 18 and 24 wk of gestation, and may be first, second or third degree (complete). Mortality approaches -20%, and most surviving children require pacemakers. Affected infants may develop cardiomyopathy. Abnormalities in the skin, liver and blood of neonates are also associated with anti-Ro/La antibodies, and are usually self-limiting; these manifestations and CHB are collectively referred to as neonatal lupus syndromes (NLS). Investigation of pathogenesis. Recent studies demonstrate that Ro/La ribonucleoproteins appear on the surface of apoptotic fetal cardiocytes and are recognized by their cognate antibodies, promoting an inflammatory response. Mice immunized with Ro/La proteins have offspring with conduction abnormalities. In vitro, human serum and IgG with anti-Ro/La antibodies affect the conducting properties of isolated animal heart tissue.
International Journal of Medical Students, 2013
Background: Complete Congenital Heart Block (CCHB) is a rare disease of the newborn that carries significant morbidity and mortality. CCHB can be diagnosed early or late in life. In newborns, it is usually associated with maternal autoantibodies or a congenital cardiac structural abnormality. The most common presentation of CCHB is bradycardia that can be diagnosed by an electrocardiogram. Results: This is a case report of a male infant born to a mother with an autoimmune disease, Systemic Lupus Erythematosus (SLE), who was found to have third degree heart block at birth. Conclusion: Early diagnosis and prompt management of the case is important for a better prognosis and prevention of associated complications. Neonates with CCHB should be managed at a tertiary care center and the only definite treatment is insertion of a pacemaker. Moreover, prenatal diagnosis and specific obstetric counseling of pregnant women with SLE along with careful monitoring with serial ultrasonography and...
Complete Congenital Heart Block in a Newborn Associated
2013
Background: Complete Congenital Heart Block (CCHB) is a rare disease of the newborn that carries significant morbidity and mortality. CCHB can be diagnosed early or late in life. In newborns, it is usually associated with maternal autoantibodies or a congenital cardiac structural abnormality. The most common presentation of CCHB is bradycardia that can be diagnosed by an electrocardiogram. Results: This is a case report of a male infant born to a mother with an autoimmune disease, Systemic Lupus Erythematosus (SLE), who was found to have third degree heart block at birth. Conclusion: Early diagnosis and prompt management of the case is important for a better prognosis and prevention of associated complications. Neonates with CCHB should be managed at a tertiary care center and the only definite treatment is insertion of a pacemaker. Moreover, prenatal diagnosis and specific obstetric counseling of pregnant women with SLE along with careful monitoring with serial ultrasonography and echocardiography are of paramount importance in prevention of the disease in subsequent offspring.
Journal of The American College of Cardiology, 2001
We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects.
Neonatal Lupus with Congenital Atrioventricular Block and Myocarditis
Pacing and Clinical Electrophysiology, 1987
BHARATI, S., ET AL.: Neonatal lupus with congenital atrioventricular block and myocarditis. This is a case of a child with neonalal lupus and congenital atrioventricuiar (AV) block, born to a mother with asymptomatic, systemic lupus erythematosus ISLE). The child, despite pacemaker insertion, died ofsepticemia and myocarditis at the age of three months. Although the association 0/ neonatal lupus with congenital AV block is welZ-recognized, there are only few pathologic studies of the conduction system reported in the literature. This is such a study in which we emphasize that, due to an altered immune system in the child, septicemia may be the cause of death in some cases
A review of congenital heart block
PubMed, 2003
Congenital heart block is a rare disorder. It has an incidence of about 1 in 22,000 live births. It may be associated with high mortality and morbidity. This should generate a high index of suspicion for early diagnosis and aggressive therapy when appropriate. The congenital heart block associated with neonatal lupus is considered a form of passively acquired autoimmune disease in which maternal autoantibodies to the intracellular ribonucleoproteins Ro (SS-A) and La (SS-B), cross the placenta and injure the previously normal fetal heart. Women with serum titers of anti-Ro antibody carry a 3% risk of having a child with neonatal lupus syndrome. Recurrence rates are about 18%. We believe that serial echocardiograms should be acquired so that early diagnosis is made and aggressive therapy administered, if signs of conduction system disease such as PR interval prolongation by Doppler are found, so as to optimize the outcome. Establishment of guidelines for therapy have been set empirically, should signs of congenital heart block develop. Those patients whose congenital heart block is associated with structural heart disease have a higher morbidity and mortality, which is determined more by the underlying structural congenital heart disease than it is by the need for a pacemaker per se.
Recurrent congenital heart block in neonatal lupus
Clinical Rheumatology, 2007
Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.
Isolated congenital heart block
Texas Heart Institute Journal from the Texas Heart Institute of St Luke S Episcopal Hospital Texas Children S Hospital, 2010
Isolated congenital heart block, frequently seen in mothers who have connective-tissue disease, can be transmitted to the fetus through transplacental passage of anti-Ro/SSA and anti-La/SSB autoantibodies. Even if the antibodies appear transiently in the fetal circulation, the block is permanent and can require pacemaker implantation. Complete congenital heart block is seen in 1% to 5% of neonates born to mothers who carry these autoantibodies. Herein, we report the case of a baby-born to a 31-year-old asymptomatic woman-who manifested congenital heart block in utero, at 30 weeks of gestation. During gestation and following birth, no further problems were detected. At her last follow-up appointment, 8 years later, the girl still had no clinical symptoms, and exercise increased her heart rate despite complete heart block. We report this case for its unique presentation, and we discuss isolated congenital heart block within the context of the inadequate literature on its pathogenesis and treatment.