Spontaneous rupture of atrioventricular valve tensor apparatus as late manifestation of anti-Ro/SSA antibody-mediated cardiac disease
| Autor: | Michael R. Liske, D. Woodrow Benson, Deborah Fruitman, Bo-Yee Ngan, Marie Wahren-Herlineus, S. Yen Ho, Bettina F. Cuneo, Edgar Jaeggi |
|---|---|
| Rok vydání: | 2010 |
| Předmět: |
Adult
medicine.medical_specialty Biopsy Heart Rupture Gestational Age Ultrasonography Prenatal Diagnosis Differential Fatal Outcome Fetal Heart Pregnancy Mitral valve Internal medicine medicine Humans Atrioventricular Block Papillary muscle Maternal-Fetal Exchange Atrioventricular valve medicine.diagnostic_test business.industry Pregnancy Outcome Dilated cardiomyopathy Endocardial fibroelastosis Papillary Muscles medicine.disease Pregnancy Complications medicine.anatomical_structure Antibodies Antinuclear Cardiology Mitral Valve Female Cardiology and Cardiovascular Medicine business Atrioventricular block |
| Zdroj: | The American journal of cardiology. 107(5) |
| ISSN: | 1879-1913 |
| Popis: | Atrioventricular (AV) block and endocardial fibroelastosis associated with dilated cardiomyopathy are the most common clinical manifestations of anti-Ro/SSA-mediated fetal cardiac disease. Valvar dysfunction has not been a prominent feature of this disease; however, recent anecdotal cases have suggested an association between rupture of the AV valve tensor apparatus and maternal anti-Ro/SSA antibodies. In the present study, we have described the clinical and laboratory findings and reviewed the published data for infants of anti-Ro/SSA-positive pregnancies with AV valve insufficiency due to chordal rupture from the papillary muscles. The histopathologic features of the papillary muscle and ventricular free wall and septum biopsy specimens were examined and compared to the sections of AV leaflets from 6 autopsied fetuses with anti-Ro/SSA-mediated complete AV block without chordal disruption. Specific epitopes to the p200 region of Ro52, and Ro60 antibodies were evaluated in cases with chordal rupture. Severe AV valve insufficiency was detected prenatally (as early as 34 weeks of gestation) or postnatally (as late as 182 days) after areas of patchy echogenicity were noted in the papillary muscle at 19 to 22 weeks of gestation. Postnatally, urgent valve surgery was performed in 5 of 6 patients; 1 of 6 patients died preoperatively. All patients tested positive for Ro52. Valve leaflet tissue from the autopsy specimens was normal. The ventricular free wall and septum biopsy specimens from a patient with chordal rupture showed normal tissue; however, the papillary muscle biopsy specimens demonstrated severe atrophy with near total replacement of myocytes by fibrosis and dystrophic calcifications, and negative immunochemistry findings. In conclusion, these findings have defined an underappreciated complication of fetal antibody-mediated cardiac inflammation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect