Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Besides congenital heart blocks (CHB), others manifestations of cardiac neonatal lupus erythematosus syndrome (NLES) include endocardial fibroelastosis (EFE), and dilated cardiomyopathy. Recently, autopsy of fetuses and children with cardiac NLES showed that EFE was under-diagnosed by echocardiography. We investigated the utility of cardiac magnetic resonance (CMR) for myocardial characterization, in children exposed in uteroto anti-SSA/Ro antibodies (Ab), with focus on inflammation and fibrosis.
Methods: Children born to mothers with anti-SSA/Ro Ab were enrolled in a prospective study performed from December 2011 to June 2012. The control group consisted of 6 healthy children born to mothers without anti-SSA Ab. All the children had a complete cardiac testing with electrocardiogram, echocardiography and a CMR assessments (T2-weighted sequence for myocardial edema, T1-weighted sequence before and after intravenous administration of gadolinium for hyperemia, and late gadolinium enhancement). Positive findings in 2 of 3 sequences were considered diagnostic of myocarditis, and positive late gadolinium enhancement indicative of fibrosis.
Results: 23 children with (n=15, group 1) and without (n=8, group 2) cardiac NLES were included. Cardiac manifestations in group 1 were complete CHB (n=10), 2nd degree CHB (n=2), 1st degree CHB (n=1), supraventricular tachycardia (n=2). EFE was present on the in utero echocardiography in 7.
Group 2 included 3 children with cutaneous manifestations of NLES, and 5 symptom free children (2 had siblings with NLES manifestations).
11 children (10 in group 1 and 1 in group 2) had abnormal CMR whereas only 3 (all in group 1) had abnormal postnatal echocardiography (table1). Interestingly, no myocardial inflammation was observed after 3 months of life. The 6 children of the control group (anti-SSA-) had normal CMR.
CMR was performed twice in 3 children (2 with CHB and 1 with supraventricular tachycardia and EFE). In 2 children, the second MRI showed stability or an improvement of inflammation after 5 and 11 days respectively. In the third child (with CHB), an extension of fibrosis was observed after 9 months. This child died of heart failure at 2.8 years old despite cardiac pacing.
Conclusion: CMR is more sensitive than echocardiography to show myocarditis and/or fibrosis associated to anti-SSA antibodies. Further studies are needed to correlate CMR characteristics with late onset dilated cardiomyopathy.
|
|
Normal CMR |
Normal but incomplete CMR (without injection) |
Inflammation |
Inflammation and fibrosis |
Fibrosis |
|
Children |
11 |
2 |
2 |
2 |
6 |
|
Median age of children at CMR (years) |
1.75 [0.01-8.54] |
0.93 |
0.17 |
0.14 |
2.14 [0.01- 7.6] |
|
Number of children |
|
|
|
|
|
|
– group 1 (SSA+, cardiac NLES) |
4 |
2 |
2 |
1 |
6 |
|
– group 2 (SSA+, no cardiac NLES) |
7 |
|
|
1 |
|
|
In utero echocardiography |
|
|
|
|
|
|
– normal |
11 |
1 |
|
1 |
2 |
|
– EFE |
|
1 |
2 |
1 |
3 |
|
– Not available |
|
|
|
|
1 |
|
Post natal echocardiography |
|
|
|
|
|
|
– normal |
11 |
2 |
1 |
1 |
5 |
|
– EFE |
|
|
1 |
1 |
1 |
Disclosure:
N. Costedoat-Chalumeau,
None;
A. Maltret,
None;
K. Levesque,
None;
S. Kutty,
None;
E. Villain,
None;
P. Ou,
None;
G. Guettrot-Imbert,
None.
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