Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero

Abstract

ObjectiveCongenital heart block (CHB) may develop in fetuses of Ro/SSA autoantibody-positive women. Given the rarity of CHB, information on comorbidity and complications later in life is difficult to systematically collect for large groups of patients. We therefore used nation-wide healthcare registers to investigate comorbidity and outcomes in patients with CHB and their siblings.MethodsData from patients with CHB (n= 119) and their siblings (n= 128), all born to anti-Ro/SSA-positive mothers, and from matched healthy controls (n= 1,190) and their siblings (n= 1,071), were retrieved from the Swedish National Patient Register. Analyses were performed by Cox proportional hazard modelling.ResultsIndividuals with CHB had a significantly increased risk of cardiovascular comorbidity, with cardiomyopathy and/or heart failure observed in 20 (16.8%) patients versus 3 (0.3%) controls, yielding a HR of 70.0 (95% CI 20.8 to 235.4), and with a HR for cerebral infarction of 39.9 (95% CI 4.5 to 357.3). Patients with CHB also had a higher risk of infections. Pacemaker treatment was associated with a decreased risk of cerebral infarction but increased risks of cardiomyopathy/heart failure and infection. The risk of systemic connective tissue disorder was also increased in patients with CHB (HR 11.8, 95% CI 4.0 to 11.8), and both patients with CHB and their siblings had an increased risk to develop any of 15 common autoimmune conditions (HR 5.7, 95% CI 2.83 to 11.69 and 3.6, 95% CI 1.7 to 8.0, respectively).ConclusionsThe data indicate an increased risk of several cardiovascular, infectious and autoimmune diseases in patients with CHB, with the latter risk shared by their siblings.

A Broader Perspective on Anti-Ro Antibodies and Their Fetal Consequences—A Case Report and Literature Review

“…There are data regarding the high risk of cardiovascular morbidity and infections in children with CHB [ 124 ]. Furthermore, there is an increased prevalence of systemic connective tissue disorder in this population, which in itself poses a threat of AV block or tachyarrhythmia later in life and requires close follow-up [ 124 ]. In this regard, a new entity was recently described, namely autoimmune channelopathies [ 51 ].…”

Factors associated with long-term cardiac dysfunction in neonatal lupus

“…A recent study following 119 patients with CHB from a Swedish population register identified that 16.8% had a diagnosis of heart failure and/or cardiomyopathy based on International Classification of Diseases coding at long-term follow-up 20. A previous Swedish study evaluated risks of postnatal cardiac dysfunction 15.…”

Effects of maternal medication on long-term outcome in congenital heart block remain to be established. Response to: ‘Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero’ by Satiset al

“…However, in the current epidemiological study on long-term outcome, exhaustive information on treatment was not available and was thus not included in the article. Notably though, the practice to treat incomplete or complete AV block in our country was not introduced until after year 2000, the effect of which may not be captured in the discussed study 2. A follow-up study investigating preventive procedures and predictors of fetal outcomes in Ro/SSA-positive pregnancies is under way.…”

“…We thank Satis and colleagues for opening the discussion on a potential influence of maternal immunomodulatory treatment on the long-term outcome of congenital heart block (CHB)1 and welcome their questions to help clarify some aspects of our study 2. Whether immunomodulatory treatment administered to the mother—as part of her own rheumatic disease treatment or intended to ameliorate the fetal cardiac inflammation in CHB—influences any outcome in CHB is a current matter of debate.…”