Pregnancy in women with heritable aortic diseases - a 15 year single center experience

Background

The risk of pregnancy in women with heritable aortic diseases (HTAD) is high, but supporting data are scarce. Regarding the difficulties in decision making in a spectrum of rare diseases. This cohort shows the prospective clinical course of consecutively treated pregnant women in a large specialized center with integrated interdisciplinary care as proposed by the ESC 2025 Guidelines.

Purpose

WHO classifies pregnant women with HTAD at highest risk for complications among the spectrum of cardiovascular diseases. While care in specialized canters is promoted to lower the risk, it is suggested that most severe complications occur in undiagnosed women. This cohort includes women with confirmed HTAD as well as those with a pregnancy related complication leading to diagnosis.

Methods

We prospectively included 118 women with 159 pregnancies from 2010 to 2025. 64 women with Marfan-Syndrome, 18 with Loeys-Dietz-Syndrome, 5 with vascular Ehlers-Danlos-Syndrome, 9 with small muscle cell diseases, 15 with non classified syndromal and aortic features, 9 with non classified non-syndromal features and 6 women with bicuspid aortic valves.

Results

Early clinical events (during pregnancy or within 8 weeks postpartum) occured in 14 women (11,8%) including typ A- or B- aortic dissection, dura leakage or out of proportion aneurysm growth with interventional need.

10 women (8,4%) experienced late clinical events within 2 years postpartum including elective aortic repair, type B dissection, valvular repair and a case of hypoxic brain injury and death.

Despite premature birth with good outcome there were no adverse events regarding the newborns.

Predominant birth mode was sectio caesarea, especially in Marfan patients without aortic complications due to complex eye and lens situations.

Dural ectasia does not seem to diminish the effect of spinal anesthesia.

Conclusions

The risk of severe complications during pregnancy in women in this consecutive cohort with HTAD is high and potentially life-threatening.

Complications during pregnancy predominantly affected women without a confirmed diagnosis or treatment in a specialized centre including preconception counselling and protective medication (ß-Blockers).

This strongly emphasizes the need for early diagnosis and routine preconception counselling early in adulthood.