Maternal collagen vascular disease associated with fetal heart block and degenerative changes of the atrioventricular valves.
We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insufficiency occurred following placement of an endocardial pacing lead, and severe mitral insufficiency occurred acutely at 2.5 months of age. Both valves were noted at surgery to have fibrotic and calcific chordae tendineae that had ruptured from their papillary muscles. An inflammatory reaction within the fetal heart related to transplacental passage of Sjögren's antibody was likely responsible.
Weber
H S
HS
Section of Pediatric Cardiology, Pennsylvania State University Childrens Hospital, Hershey.
Myers
J L
JL
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D002815:Chordae Tendineae / Q000473:pathology / Q000601:surgery; D003095:Collagen Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D004452:Echocardiography; D005260:Female; D006327:Heart Block / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006350:Heart Valve Prosthesis; D006801:Humans; D007231:Infant, Newborn; D008431:Maternal-Fetal Exchange / Q000502:physiology; D008943:Mitral Valve / Q000473:pathology / Q000601:surgery; D008944:Mitral Valve Insufficiency / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D010210:Papillary Muscles / Q000473:pathology / Q000601:surgery; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D012422:Rupture, Spontaneous; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014261:Tricuspid Valve / Q000473:pathology / Q000601:surgery; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D016216:Ultrasonography, Prenatal
D001323:Autoantibodies