Cargando…
Neonatal lupus with left bundle branch block and cardiomyopathy: a case report
BACKGROUND: Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391615/ https://www.ncbi.nlm.nih.gov/pubmed/32727396 http://dx.doi.org/10.1186/s12872-020-01637-4 |
_version_ | 1783564673339097088 |
---|---|
author | Rumancik, Brad Haggstrom, Anita N. Ebenroth, Eric S. |
author_facet | Rumancik, Brad Haggstrom, Anita N. Ebenroth, Eric S. |
author_sort | Rumancik, Brad |
collection | PubMed |
description | BACKGROUND: Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies. CASE PRESENTATION: A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis. CONCLUSIONS: We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course. |
format | Online Article Text |
id | pubmed-7391615 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-73916152020-08-04 Neonatal lupus with left bundle branch block and cardiomyopathy: a case report Rumancik, Brad Haggstrom, Anita N. Ebenroth, Eric S. BMC Cardiovasc Disord Case Report BACKGROUND: Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies. CASE PRESENTATION: A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis. CONCLUSIONS: We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course. BioMed Central 2020-07-29 /pmc/articles/PMC7391615/ /pubmed/32727396 http://dx.doi.org/10.1186/s12872-020-01637-4 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Rumancik, Brad Haggstrom, Anita N. Ebenroth, Eric S. Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title | Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title_full | Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title_fullStr | Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title_full_unstemmed | Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title_short | Neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
title_sort | neonatal lupus with left bundle branch block and cardiomyopathy: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391615/ https://www.ncbi.nlm.nih.gov/pubmed/32727396 http://dx.doi.org/10.1186/s12872-020-01637-4 |
work_keys_str_mv | AT rumancikbrad neonatallupuswithleftbundlebranchblockandcardiomyopathyacasereport AT haggstromanitan neonatallupuswithleftbundlebranchblockandcardiomyopathyacasereport AT ebenrotherics neonatallupuswithleftbundlebranchblockandcardiomyopathyacasereport |