Ebstein’s anomaly

In 1976, a final report from the International Register of Lithium Babies contained information on 225 infants exposed to lithium in the first trimester. Cardiovascular anomalies occurred in 18 (8%) of the newborns, of which 6 (2.7%) had Ebstein s anomaly (Weinstein, 1976). A comprehensive review of this report and the multiple studies that have been conducted since (Cohen et ah, 1994) revealed that the risk of congenital defects after in utero exposure to lithium is less than previously anticipated. Currently, the risk for Ebstein s anomaly after first-trimester exposure to lithium is estimated to be 1 in 1000 (0.1%) (Altshuler et ah, 1996), or 10-20 times greater than the general population risk of 1 in 20,000 (Weinstein, 1976). Thus, the absolute risk for Ebstein s anomaly is small. 

Nora JJ, Nora HA, Toews WH Lithium, Ebstein s anomaly, and other congenital heart defects. Lancet 2 594-595, 1974... 

The risk of Ebstein s anomaly in infants exposed to hthium in utero is 0.1%-0.7% (Edmonds and Oakley 1990 Jacobson et al. 1992 Kallen and Tandberg 1983 Zalzstein et al. 1990), compared with 0.01 % in the general population. The overall risk of major con-... 

Benign leukocytosis Weight gain Narrow therapeutic index Tremor Potentially fatal toxicity Alopecia Risk of Ebstein s anomaly with first-trimester exposure... 

Dunner DL, Eieve RR Clinical factors in lithium carbonate prophylaxis failure. Arch Gen Psychiatry 30 229-233, 1974 Edmonds LD, Oakley GP Ebstein s anomaly and maternal lithium exposure during pregnancy. Teratology 41 551-552, 1990 Emrich HM Studies with oxcarbazepine (Trileptal) in acute mania. Int Clin Psychopharmacol 5 83-88, 1990... 

Zalzstein E, Koren G, Einarson T, et al A case-control study on the association between first trimester exposure to lithium and Ebstein s anomaly. Am J Cardiol 65 817-818, 1990... 

The issue of lithium-induced dysmorphogenesis is not settled. An earlier report suggested an increase in cardiac anomalies—especially Ebstein s anomaly —in lithium babies, and it is listed as such in Table 59-1 in this book. However, more recent data suggest that lithium carries a relatively low risk of teratogenic effects. Further research is needed in this important area. 

The cardiovascular teratogenicity of lithium has been summarized in a review of managing bipolar disorder during pregnancy and postpartum (473). While the risk of Ebstein s anomaly is increased, likely 10-20 times more than in the general population, the absolute risk (0.05-0.10%) is small. Fetal ultrasonography was advised at 18-20 weeks of gestation in cases of first trimester lithium exposure (488). 

Evidence of increase in cardiac anomalies (especially Ebstein s anomaly) in infants whose mothers took lithium during pregnancy... 

In pregnancy (see pl86 for detail) Cardiac malformation (Ebstein s anomaly) Foetal malformations, including Spina bifida Cleft palate Cardiac defects Cognitive dysfunction... 

The use ofLt in early pregnancy may be associated with an increase in the incidence of cardiovascular anomalies of the newborn, especially Ebstein s malformation. The antimanic anticonvulsants valproic acid and probably carbamazepine have an associated risk of irreversible spina bifida that may exceed 1/100, and so do not represent a rational alternative for pregnant women. In balancing the risk versus benefit of using LG in pregnancy, it is important to evaluate the risk of untreated manic-depressive disorder and to consider conservative measures, such as deferring intervention until symptoms arise or using a safer treatment, such as a neuroleptic or ECT... 

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