Glucocorticoid-remediable aldosteronism (GRA) is a hereditary form of prima
ry hyperaldosteronism that presents with hypokalemia and hypertension from
childhood onward. GRA is characterized by the ectopic production of aldoste
rone in the cortisol-producing zona fasciculata under the regulation of adr
enocorticotrophic hormone. Despite the early age of onset, no previous repo
rts of pregnancy and GRA exist. Therefore, we set out to describe the mater
nal and fetal outcomes of pregnancy in women with GRA, Data regarding the b
lood pressure and pregnancy outcomes were collected in a retrospective char
t review of prenatal and hospital records of 35 pregnancies in 16 women wit
h genetically proven GRA. A total of 6% of pregnancies in women with GRA (G
RA+) were complicated by preeclampsia. The published rates of preeclampsia
in general obstetric populations vary from 2.5% to 10%. Despite the lack of
an apparent increase in the rate of preeclampsia, GRA+ women with chronic
hypertension had a high rate (39%) of pregnancy-aggravated hypertension. St
arting with a higher baseline blood pressure, maternal blood pressure plott
ed over the time course of pregnancy followed a quadratic curve similar to
that previously described in normal pregnancy. Mean gestational age at deli
very was 39.1 weeks. Mean birth weight, excluding the 3 sets of twins, was
3219 g, However, infants of GRA+ mothers with pregnancy-aggravated hyperten
sion tended to have lower birth weights than those that did not (3019 g ver
sus 3385 g, respectively; P=0.08), The primary cesarean section rate was 32
%, which is approximately double that seen in other general or hypertensive
obstetric populations, In summary, GRA+ women did not seem to have an incr
eased risk of preeclampsia. However, GRA+ women with chronic hypertension s
eem to be at an increased risk for an exacerbation of their hypertension du
ring pregnancy.