Objective: To assess the prevalence of cardiac abnormalities in fetuses of women with clinical or serologic evidence of systemic lupus erythematosus (SLE).
Methods: A retrospective review of fetal echocardiograms performed secondary to maternal SLE or connective tissue antibodies with or without fetal complete heart block (CHB) was performed to evaluate the prevalence of fetal structural heart disease, valve regurgitation, abnormal ventricular function, pericardial effusion, endocardial fibroelastosis and rhythm abnormalities.
Results: Forty-one fetuses were studied. One fetal demise occurred. Three fetuses had structural abnormalities, including d-transposition of the great arteries (n = 1) and pulmonic stenosis (n = 2). Seventeen fetuses had valve regurgitation. The prevalence of valve regurgitation was: tricuspid (n = 15), mitral (n = 6), pulmonic (n = 4) and aortic (n = 2). Two fetuses with pulmonic insufficiency had pulmonic stenosis. Four fetuses had CHB, one of which developed atrial flutter. Prolongation of the mechanical AV interval occurred in five fetuses; none developed CHB.
Conclusions: In addition to CHB and myocardial dysfunction, structural heart defects occur in fetuses of women with serologic or clinical evidence of SLE. In our series, this occurred more frequently than reported for the general population. Valve regurgitation was present in all fetuses with CHB and many in sinus rhythm and may represent cardiac inflammation. Pulmonic insufficiency in utero may be a marker for a structural valve abnormality.