A Case of Neonatal Lupus Presenting with Myocardial Dysfunction in the Absence of Congenital Heart Block (CHB): Clinical Management and Brief Literature Review of Neonatal Cardiac Lupus

Samhita Jain; Ruggero Spadafora; Sarah Maxwell; Carlos Botas; Hythem Nawaytou; Emily von Scheven; Elizabeth E Crouch

doi:10.1007/s00246-022-03056-y

A Case of Neonatal Lupus Presenting with Myocardial Dysfunction in the Absence of Congenital Heart Block (CHB): Clinical Management and Brief Literature Review of Neonatal Cardiac Lupus

Pediatr Cardiol. 2023 Mar;44(3):736-739. doi: 10.1007/s00246-022-03056-y. Epub 2022 Dec 2.

Authors

Samhita Jain^#¹, Ruggero Spadafora^#², Sarah Maxwell³, Carlos Botas^{1

4}, Hythem Nawaytou⁵, Emily von Scheven⁶, Elizabeth E Crouch^{7

8}

Affiliations

¹ Division of Neonatology, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
² Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
³ Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁴ Kaiser Permanente San Francisco Medical Center, Division of Cardiology, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁵ Division of Cardiology, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁶ Division of Rheumatology, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
⁷ Division of Neonatology, Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA. elizabeth.crouch@ucsf.edu.
⁸ The Eli and Edythe Broad Center of Regeneration Medicine and Stem Cell Research, University of California San Francisco, San Francisco, CA, USA. elizabeth.crouch@ucsf.edu.

^# Contributed equally.

Abstract

Neonatal lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren's Syndrome A or B (SSA-SSB) autoantigens (Vanoni et al. in Clin Rev Allerg Immunol 53:469-476, 2017) which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart block (AVB) in newborns with a structurally normal heart (Martin in Cardiol Young 24: 41-46, 2014). NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe ventricular dysfunction in the absence of rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy (Trucco et al. in J Am Coll Cardiol 57:715-723, https://doi.org/10.1016/j.jacc.2010.09.044 , 2011), the most common cardiac presentations of NLE. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.

Keywords: Left ventricular dysfunction; Neonatal lupus.

Publication types

Review
Case Reports

MeSH terms

Atrioventricular Block* / diagnosis
Atrioventricular Block* / etiology
Atrioventricular Block* / therapy
Autoantibodies
Autoimmune Diseases*
Female
Humans
Infant, Newborn
Lupus Erythematosus, Systemic* / complications
Lupus Erythematosus, Systemic* / diagnosis
Pregnancy Complications*

Substances

Autoantibodies

Supplementary concepts

Congenital heart block
Neonatal Systemic lupus erythematosus