. Author manuscript; available in PMC: 2017 Aug 10.

Published in final edited form as: Nat Rev Rheumatol. 2015 Mar 24;11(5):301–312. doi: 10.1038/nrrheum.2015.29

Table 1.

Main characteristics of autoimmune EFE

Features	Feature details	Cases (n)
CHB (n = 116)	No AV block	22 (19%)
	AV block	94 (81%)
	Type I	4 of 38 (11%)^*
	Type II	2 of 38 (5%)^*
	Type III	32 of 38 (84%)^*

Outcome (n = 103)	Survival	50 (49%)
	Death	53 (51%)
	Intrauterine death	27 of 39 (69%)^‡
	Neonatal death	12 of 39 (31%)^‡

Maternal disease (n = 31)	Asymptomatic Ro/La carriers	17 (55%)
	SLE	9 (29%)
	Hypothyroidism	2 (6%)
	Mixed connective tissue disease	1 (3%)
	Raynaud phenomenon	1 (3%)
	Arthritis	1 (3%)

Pacemaker required (n = 17)	Yes	13 (76%)
Pacemaker required (n = 17)	No	4 (24%)

Data include 116 patients from 14 studies.^{30,32,33,40,50,51,73–80}

Only these studies.^{33,75,78–80}

^‡

Only these studies.^{32,33,40,50,51,7–80}

Abbreviations: AV, atrioventricular; CHB, congenital heart block; EFE, endocardial fibroelastosis; ND, not defined; SLE, systemic lupus erythematosus.