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. 2022 Jul 28:10:904138.
doi: 10.3389/fped.2022.904138. eCollection 2022.

Cardiac manifestations in a Chinese cohort of fetuses from mothers with anti-Ro and anti-La antibodies

Xin Wang  1 , Xiao-Wei Liu  1 , Ling Han  2 , Meng-Tao Li  3 , Jiu-Liang Zhao  3 , Lin Sun  1 , Jian-Cheng Han  1 , Xiao-Feng Zeng  3 , Xin-Ping Tian  3 , Ying Zhao  1 , Yi-Hua He  1
Affiliations

Cardiac manifestations in a Chinese cohort of fetuses from mothers with anti-Ro and anti-La antibodies

Xin Wang et al. Front Pediatr. .

Abstract

Objectives: To analyze the clinical characteristics, echocardiographic features, and prognosis of fetuses based on three groups of cardiac manifestations associated with maternal anti-Ro and anti-La antibodies in China. This study included three groups: the isolated-arrhythmia, isolated-endocardial fibroelastosis (EFE), and mixed groups.

Methods: We prospectively evaluated 36 fetuses with cardiac manifestations due to maternal anti-Ro and anti-La antibodies from our center between 2016 and 2020 in China. Clinical and echocardiographic data were collected.

Results: There were 13 patients (36%) in the isolated-arrhythmia group, eight (22%) in the isolated-EFE group, and 15 (42%) in the mixed group. All patients in the isolated-EFE group presented with mild EFE. Severe EFE was identified in four patients (27%) in the mixed group. Atrioventricular block (AVB) was more common in the isolated-arrhythmia group (13, 100%) than in the mixed group (6, 40%; p = 0.001). Moderate-severe mitral regurgitation (p = 0.006), dilated cardiomyopathy (DCM, p = 0.017), and low cardiovascular profile scores (p = 0.013) were more common in the mixed group than in the other two groups. Twenty-one mothers decided to terminate the pregnancy and 15 fetuses were born with regular perinatal treatment. They all survived at 1 year of age. One patient in the isolated-arrhythmia group and two in the mixed group required a pacemaker due to third-degree AVB or atrioventricular junctional rhythm. Five patients in the isolated-EFE group and five in the mixed group had no DCM or heart failure and the location of mild EFE was significantly reduced.

Conclusion: Fetal cardiac manifestations due to maternal anti-Ro and anti-La antibodies can be divided into three groups, i.e., the isolated-arrhythmia, isolated-EFE, and mixed groups. AVB usually occurs in the isolated-arrhythmia group. Severe EFE, moderate-severe mitral regurgitation, and DCM mainly appear in the mixed group. Location of mild EFE significantly reduces after birth and the outcome of fetuses with mild EFE depends on the presence of arrhythmia and its subtypes.

Keywords: anti-Ro antibodies; atrioventricular block; echocardiography; endocardial fibroelastosis; prenatal diagnosis.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Conditions related to mothers carrying anti-Ro and anti-La antibodies. SLE, systemic lupus erythematosus; UAD, undifferentiated autoimmune disease; pSS, primary Sjögren syndrome; RA, rheumatoid arthritis; aSS, Sjögren syndrome associated with other systemic autoimmune diseases.
Figure 2
Figure 2
Distribution of endocardial fibroelastosis in fetuses with cardiac complications due to maternal anti-Ro and anti-La antibodies. PM, papillary muscle; LA, left atrium; RA, right atrium; MV, mitral valve; TV, tricuspid valve; AV, aortic valve; PV, pulmonary valve; RV, right ventricle; LV, left ventricle.
Figure 3
Figure 3
Distribution of 36 fetuses with cardiac manifestations due to maternal anti-Ro and anti-La antibodies. Bradyarrhythmia includes sinus bradycardia, junctional rhythm and ectopic atrial rhythm. Other types contain ventricular tachycardia and atrial flutter. EFE, endocardial fibroelastosis; AVB, atrioventricular block.
Figure 4
Figure 4
Outcome of fetuses with cardiac manifestations due to maternal anti-Ro and anti-La antibodies. TOP, termination of pregnancy; EFE, endocardial fibroelastosis; AVB, atrioventricular block; NSR, normal sinus rhythm.
Figure 5
Figure 5
The echocardiographic features of isolated mild EFE in a fetus due to maternal anti-Ro and anti-La antibodies. Triangles mark the areas of mild EFE in four-chamber view [(A) 30 gestational weeks; (B) 33 gestational weeks; (C) 1 month; (D) 1 year]. LV, left ventricle; RV, right ventricle.
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