Congenital Heart Block (CHB)
from OrphaChat — a Rare Disease Podcast Hosted by RCH
Congenital heart block (CHB) is a rare but life-threatening condition where the electrical signals between the heart’s upper and lower chambers are disrupted, often appearing in infants before or shortly after birth. The provided sources identify maternal autoantibodies, specifically anti-Ro/SSA and anti-La/SSB, as the primary cause of inflammation and irreversible scarring in the fetal heart’s conduction system. While many mothers are asymptomatic at the time of diagnosis, those with Sjögren’s syndrome or lupus are at a notably higher risk for these complications. Modern medical management focuses on early detection through frequent fetal echocardiograms and home heart rate monitoring to identify rhythm changes before they progress. Hydroxychloroquine and corticosteroids are highlighted as key pharmaceutical interventions used to reduce the risk of recurrence and manage cardiac inflammation during pregnancy. Despite these treatments, advanced cases often lead to permanent bradycardia, necessitating the lifelong use of cardiac pacemakers for survivors.
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