Main Site of EFE | Mothers | Children/Fetuses | Last Followup | |||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Case | Sex | RA | LA | RV | LV | Other | Maternal Diagnosis | SSA Ab | SSB Ab | Tx | Age at EFE Diagnosis | Other NLES | Outcome | Age at Last Followup (yrs) | ECG | Echocardiography |
1 | M | − | + | +/− | − | Mitral A Aortic A | Asymp | + | + | − | 22 wks | + | 39 wks 3200 g | 4 | N | Mild EFE of LA |
2 | F | − | − | − | + | IVS Mitral A Aortic A | SLE | + | − | − | 22 wks | + | 35 wks 1790 g | 4 | N | N |
3 | M | − | + | − | − | Mitral A Pericard effusion Sinus bradycardia | Asymp | + | + | Betam | 22 wks | / | Abortion at 30 wks | / | / | / |
4 | F | − | + | − | − | Mitral A Aortic A Pericard effusion Suprav T | SLE | + | + | Betam | 22 wks | + | 33 wks 1655 g | 7 | N | N |
5* | F | − | − | ++ | − | Mitral A Tricuspid A | ** | ** | ** | − | At birth | + | 36.5 wks 2120 g | 17 | No EFE Abnormal VF Tricuspid regurgitation | |
Raboisson12 | F | − | − | + | + | Mitral A Aortic A | Asymp | + | + | Dexam | 29 wks | − | 38.5 wks 2900 g | 3 mo | N | N |
Pises13 | NA | − | − | + | − | Pericard effusion Tricuspid regurgitation | SLE | + | + | Dexam IVIG | 25 wks | − | 33 wks NA | 4 mo | N | Mild EFE of tricuspid A |
↵* This child was retrospectively included and was not investigated for hematologic or hepatic NLES. Diagnosis of cutaneous NLES was made retrospectively.
↵** Same mother as Case 4: at that time, the mother was symptom-free and her antibody status was unknown. EFE: endocardial fibroelastosis; RA: right atrium; LA: left atrium; RV: right ventricle; LV: left ventricle; Tx: treatment; NLES: neonatal lupus erythematosus syndrome; ECG: electrocardiogram; VF: ventricular filling; NA: not available; IVS: interventricular septum; Pericard: pericardial effusion; A: annulus; Suprav T: Supraventricular tachycardia; SLE: systemic lupus erythematosus; Asymp: asymptomatic mothers; N; normal; betam: betamethasone; dexam: dexamethasone; IVIG: intravenous immunoglobulin.