Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve

Ferrazzini, G.; Fasnacht, Margrit; Arbenz, U; Seger, Reinhard; Biedermann, R.; Simma, Burkhard; Uehlinger, J.; Dangel, P.; Fanconi, Sergio

doi:10.1007/bf01712168

Cited by 10 publications

(4 citation statements)

References 13 publications

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“…Cardiac pacing is believed to decrease left ventricular (LV) size in children with isolated complete congenital AV block (CCAVB) and severe bradycardia 1 . From 5% to 11% of children with CCAVB develop dilated cardiomyopathy (DCMP), 2–4 whose etiology has mostly been attributed to autoimmune myocarditis 5,6 . Right ventricular (RV) apical pacing has been shown to induce LV electromechanical dyssynchrony, 7 myocardial histologic changes, 8 and LV dysfunction in the long term 9,10 .…”

Section: Introductionmentioning

confidence: 99%

“…1 From 5% to 11% of children with CCAVB develop dilated cardiomyopathy (DCMP), [2][3][4] whose etiology has mostly been attributed to autoimmune myocarditis. 5,6 Right ventricular (RV) apical pacing has been shown to induce LV electromechanical dyssynchrony, 7 myocardial histologic changes, 8 and LV dysfunction in the long term. 9,10 Direct association between RV pacing and development of severe dilated cardiomyopathy and overt heart failure in the young has not yet been demonstrated.…”

Section: Introductionmentioning

confidence: 99%

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Dilated Cardiomyopathy Associated with Dual‐Chamber Pacing in Infants:

Janoušek

Tomek

Chaloupecký

et al. 2004

Cardiovasc electrophysiol

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The etiology of dilated cardiomyopathy associated with congenital complete AV block has not yet been clarified. Two infants with AV block of autoimmune and surgical etiology, respectively, had received a dual-chamber right ventricular-based pacemaker and developed dilated cardiomyopathy with severe septal to left ventricular free-wall dyssynchrony 3.4 (0.9) years later. After 4 weeks of biventricular pacing and spontaneous junctional narrow QRS rhythm, respectively, both children showed significant improvement in left ventricular function along with reverse remodeling. Thus, electromechanical dyssynchrony associated with conventional right-ventricular-based DDD pacing may play a significant role in the development of dilated cardiomyopathy in the young.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Dilated Cardiomyopathy Associated with Dual‐Chamber Pacing in Infants:

Janoušek

Tomek

Chaloupecký

et al. 2004

Cardiovasc electrophysiol

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“…Aside from conducting and structural defects, inflammatory processes have also been described. These include pericarditis (26), endocarditis, myocarditis, and cardiomyopathy (27). In our patient, the transient heart shadow enlargement might be related to cardiac insufficiency.…”

Section: Discussionmentioning

confidence: 99%

Pneumocystis carinii Mimicking Neonatal Lupus Erythematosus‐Related Pneumonitis

Woźniacka

Robak

Sysa-Jędrzejowska

et al. 2003

Pediatric Dermatology

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Neonatal lupus erythematosus (LE) is a rare disease associated with the transplacental passage of maternal autoantibodies to infants who manifest congenital heart block, skin disease, and less commonly, hematologic and hepatic disease. Pulmonary disease is a rare manifestation of neonatal LE and has presented as transient pneumonitis. In this report we describe an infant with neonatal LE who had the classic skin and hematologic findings of the disease in addition to pulmonary disease which might be attributed to neonatal LE-related pneumonitis, but in fact was caused by a concomitant Pneumocystis carinii infection. This case demonstrates the importance of looking for other causes of pulmonary disease in neonatal LE patients.

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“…Em relação aos estudos cardíacos, o bloqueio átrio-ventricular congênito parece ser causado por processo inflamatório e não por uma lesão cardíaca que ocorreu num período crítico da embriogênese (50)(51)(52) . De fato, os anticorpos IgG que ultrapassam a barreira placentária comumente no terceiro trimestre podem interagir com o sistema de condução, que nesse período já se encontra formado e maduro.…”

Section: Fisiopatologiaunclassified

Síndrome do lúpus neonatal

Carvalho

Viana

Cruz³

et al. 2005

Rev. Bras. Reumatol.

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Neonatal Lupus Syndrome (NLS) is an autoimmune disease associated to the presence of autoantibodies against ribonucleoproteins SSA/Ro and/or SSB/La in the maternalfetal circulation and is characterized by isolated congenital heart block (ICHB) and/or cutaneous and hematological manifestations. Despite the rarity, NLS is the main cause of ICHB, which is responsible for the significant mortality (20%-30%) and morbidity of these patients. The neonatal lupus designation comes from the observation of cutaneous lesions similarities between NLS and systemic lupus erythematosus. The isolated term to define the CHB in NLS is employed to exclude cardiac abnormalities secondary to congenital structural malformations or infections. NLS is considered a model of passively acquired autoimmunity in which maternal autoantibodies cross the placenta and, once in fetal circulation, may play a role in the pathogenesis of the syndrome. The almost universal presence of anti-SSA/Ro and/or anti-SSB/La antibodies in the maternal and fetal circulation validates them as serological markers of NLS. Contrasting with the fetal cardiac tissue lesion, which is an irreversible process, cutaneous and hematological involvements are transitory manifestations and disappear after the clearance of maternal autoantibodies from the infant's circulation. It is important to notice that NLS represents a multidisciplinary challenge involving rheumatologists, obstetricians, neonatologists, dermatologists and pediatric cardiologists to identify the pregnancy at risk for NLS development and to introduce appropriate in utero or postnatal therapeutic strategies.

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Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve

Cited by 10 publications

References 13 publications

Dilated Cardiomyopathy Associated with Dual‐Chamber Pacing in Infants:

Dilated Cardiomyopathy Associated with Dual‐Chamber Pacing in Infants:

Pneumocystis carinii Mimicking Neonatal Lupus Erythematosus‐Related Pneumonitis

Síndrome do lúpus neonatal

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