Fetal manifestations of maternal anti‐Ro and La antibodies – more than complete heart block

Abstract

Complete heart block (CHB) is a potentially fatal condition occurring in approximately 1:10000 fetuses. Whilst it is well recognised that maternal anti‐Ro and La antibodies are associated with fetal CHB, there are multiple other manifestations of fetal exposure to these autoantibodies which are not widely appreciated and rarely diagnosed. The importance of identifying affected fetuses lies in the significantly increased risk of recurrence in future pregnancies, and the potential for treatments which may modify this risk. This paper presents several cases to highlight the varying fetal presentations of maternal anti‐Ro and La antibodies.

Autoimmune-associated Congenital Heart Block

“…More than half of CHB cases are associated with autoimmune and CHB remains a severe life-threatening disorder. [ 1 2 3 4 5 6 7 8 9 ] Autoimmune-associated CHB occurs in 2–5% pregnancies with positive anti-Ro/SSA (the most common one) and La/SSB antibodies, and it has a recurrence rate of 12–25% in a subsequent pregnancy. [ 4 5 6 10 ] The perinatal mortality of neonate with CHB researches to 30%, and even higher in the presence of endocardial fibroelastosis (EFE) and/or dilated cardiomyopathy (DCM).…”

“…The first sign of CHB, such as persistent fetal bradyarrhythmia, is usually noted between 18 th and 24 th gestational weeks (GWs). [ 7 9 13 14 ] In the mean time, it has been demonstrated that CAVB and cardiomyopathy may occur within 1 week following a normal echocardiogram. [ 6 13 15 ] The rapid deterioration of the tissue damages in conducting system only allows a short-time window for urgent prenatal evaluation, management, and therapy.…”

“…[ 5 ] The spectrum of manifestation is still expanding and the followings are recently reported: fetal prolonged PR interval, first-and second-degree AVB, EFE, prolongation of corrected QT interval, sinus bradycardia (SB), late-onset DCM, atrial flutter, valvular insufficiency, ventricular/valvular hyperechogenicity, pericardial effusion, and other heart disorders even in the absence of cardiac block. [ 2 4 5 6 7 ]…”

“…According to the latest opinions, once fetal cardiac involvement is determined, it should be included in the management of neonatal lupus syndrome (NLS), which is the most common presentation of autoimmunity in newborns. [ 4 5 6 7 13 ] The heart appeared to be particularly susceptible to damage in NLS. In addition to cardiac involvements, NLS may affect other systems and manifest involvement of multiple systems, such as cutaneous rashes, hepatobiliary disease, and/or thrombocytopenia or other types of cytopenia.…”

“…[ 2 6 8 16 17 18 19 20 21 22 23 24 25 ] Diverse clinical presentations need to be further dissected and classified. [ 2 3 4 5 6 7 8 9 10 ] Early detection of autoantibody-mediated CHB is critical but still challenging. It is urgently necessary to define the susceptible populations, from both maternal and fetal aspects.…”