Positive maternal anti‐SSA/SSB antibody–related fetal right ventricular endocardial fibroelastosis without atrioventricular block, reversal of endocardial fibroelastosis

Ngeth, Pises; Acherman, Ruben J.; Iriye, Brian; Rollins, Robert C.; Castillo, William; Herceg, Elizabeth; Evans, William N.

doi:10.1002/pd.2073

Cited by 8 publications

(6 citation statements)

References 7 publications

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“…Despite introduction of dexamethasone, fetal death occurred at 24 weeks, related to severe hydrops 9 . The 2 other case reports of mild EFE without CHB 12,13 seemed to show that maternal treatment had a favorable effect, but this must be interpreted cautiously in view of the spontaneous "improvement" we observed in echocardiograms after birth. Our short series does not suggest that the use of betamethasone affected the outcome.…”

Section: Discussionmentioning

confidence: 69%

“…The ultrasound at 3 months of age was considered normal. The second case was reported by Pises, et al 13 . At 25 weeks, EFE involving the right ventricle was observed, associated with mild pericardial effusion and tricuspid regurgitation.…”

Section: Discussionmentioning

confidence: 85%

“…Echocardiography was considered normal in 2 and showed only mild EFE in 1. These data are summarized in Table 1, which also includes 2 previous case reports 12,13 .…”

Section: Resultsmentioning

confidence: 99%

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A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies

Guettrot-Imbert¹,

Cohen²,

Fermont³

et al. 2010

J Rheumatol

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Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 85%

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A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies

Guettrot-Imbert¹,

Cohen²,

Fermont³

et al. 2010

J Rheumatol

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“…Environment trumped heredity! Two reports described foetal cardiomyopathies due to transplacental maternal antibodies that were completely reversed by treatment with steroids, 29 , 30 with reversal of endocardial fibroelastosis. The first ever reports of reversal of endocardial fibroelastosis, by effective treatment of the cause when done early enough .…”

Section: Mechanismsmentioning

confidence: 99%

Changing concepts of endocardial fibroelastosis

Lurie

2010

Cardiol Young

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Endocardial fibroelastosis is not a disease but a reaction of the endocardium. I review the history of the term with emphasis on the gradual understanding of the many causes of this reaction. I include a comprehensive list of diseases or other cardiac stresses that authors have reported in association, and I try to explain the mechanism of the reaction. Although endocardial fibroelastosis is rare today, I issue a warning of a possible epidemic recrudescence of some of the associated diseases. My hope is for nosologic purity, therefore that outworn but surviving concepts will be firmly rejected.

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“…In general, congestive heart failure in horses has a grave prognosis (Davis et al 2002). In contrast to veterinary medicine, prenatally diagnosed EFE has been successfully treated by dexamethasone in human medicine (Raboisson et al 2005, Pises et al 2009 In conclusion, EFE is a rare cardiac disorder affecting young horses. The six-month-old foal presented here developed progressive respiratory symptoms, likely caused by chronic pulmonary edema, which were initially considered to be due to bacterial infection.…”

Section: Figmentioning

confidence: 99%

Idiopathic endocardial fibroelastosis in a foal with biventricular heart failure

Bertram¹,

Dietert²,

Gronenberg³

et al. 2018

PHK

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A six-month-old foal was initially presented with recurrent, progressive respiratory symptoms over a period of three months. During fixation for medical treatment of a suspected bacterial infection of the respiratory tract, the horse developed acute circulatory failure and was referred to an equine clinic. Cardiac auscultation revealed holosystolic murmur centered around the mitral valve, whilst thoracic auscultation and radiography revealed pulmonary edema and a highly enlarged cardiac silhouette. The foal collapsed during echocardiographic examination and was humanely euthanized. At necropsy, the heart was enlarged with severe dilation of all four chambers and the endocardium was markedly thickened and diffusely opacified. Histopathologically, the endocardium was also severely thickened to up to 601 µm by a significantly increased amount of collagen fibers and discontinuous and irregularly arranged elastic fibers, consistent with severe endocardial fibroelastosis (EFE). In contrast, five control horses with a median age of six months lacking cardiac alterations had a mean left ventricular endocardial thickness of 29.8 ±17.3 µm with few, regularly arranged collagen and elastic fibers. Further pathological findings included pulmonary edema and fibrosis, detection of hemosiderin-laden macrophages in the alveoli, vascular and perivascular fibrosis, as well as ascites and were consistent with biventricular heart failure. Idiopathic EFE has very rarely been described in young horses and other animals and its intravital diagnosis is very difficult. However, EFE should be considered as a differential diagnosis in foals with acute or chronic congestive heart failure. As in the present case, therapeutic approaches have to date always been unrewarding in animals and therefore, prognosis is generally considered poor.

show abstract

Positive maternal anti‐SSA/SSB antibody–related fetal right ventricular endocardial fibroelastosis without atrioventricular block, reversal of endocardial fibroelastosis

Cited by 8 publications

References 7 publications

A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies

A New Presentation of Neonatal Lupus: 5 Cases of Isolated Mild Endocardial Fibroelastosis Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies

Changing concepts of endocardial fibroelastosis

Idiopathic endocardial fibroelastosis in a foal with biventricular heart failure

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