Abstract
BackgroundUntil recently, anti-SSA/Ro antibodies were not considered pathogenic for severe heart disease in adults. Prolongation of the mean QTc interval in electrocardiograms of adult patients with anti-SSA/Ro-positive connective tissue disease has been reported and could contribute to complex arrhythmias in such patients. Furthermore, complete heart block may also be related to these autoantibodies.Case presentationWe describe the occurrence of fatal complete heart block in a euthyroid adult patient with undifferentiated connective tissue disease and polyglandular autoimmune syndrome type 2 associated with cardiovascular autonomic dysfunction who had normal QTc interval. The patient's serum contained anti-SSA/Ro.ConclusionThis case might indicate that, although the adult heart conduction system may be relatively resistant to the development of anti-Ro-associated complete heart block, cardiac arrest may develop and even be fatal.
Highlights
Until recently, anti-SSA/Ro antibodies were not considered pathogenic for severe heart disease in adults
In mixed (M) connective tissue disease (CTD), minor cardiac rhythm abnormalities appear in approximately half of the adult patients [2]
We report a patient with cardiovascular autonomic dysfunction and newly diagnosed undifferentiated (U) CTD in autoimmune polyglandular syndrome type 2 (APS-2) who developed complete atrioventricular block with fatal outcome
Summary
The role of anti-SSA/Ro antibodies in the development of congenital heart block is established [1]. Anti-SSA/Ro antibodies were not considered pathogenic for severe heart disease in adults. We report a patient with cardiovascular autonomic dysfunction and newly diagnosed undifferentiated (U) CTD in autoimmune polyglandular syndrome type 2 (APS-2) who developed complete atrioventricular block with fatal outcome. International Archives of Medicine 2009, 2:15 http://www.intarchmed.com/content/2/1/15 ized lymphadenopathy had developed and persisted despite sufficient hormone replacement with thyroxin and cortisone acetate. When complete heart block developed, electrolytes were normal, serial troponin T measurement ruled out acute coronary syndrome and post-resuscitation 12-lead ECG did not show sign of ischemia or myocarditis. The patient progressed to a persistent vegetative state and, just 2 months later, died a natural death in a long-term care facility of a repeat episode of cardiac arrest
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