How do you approach the management of ADPKD in pregnancy, considering the need to stop tolvaptan therapy?

I generally consider transition to pregnancy with respect to both tolvaptan and ACEi along the same time frame... discuss when initiating counseling about becoming pregnant and discontinue use when patient is about to start actively trying to conceive.

Additional counseling may be warranted re: blood pressure control with non-acei agent and also preeclampsia risk. There may be some accelerated risk to kidney disease progression from pregnancy in ADPKD, although difficult to quantify and certainly not a reason to dissuade patients from pregnancy in motivated individuals. This risk seems to be particularly increased above a threshold of three pregnancies although based on retrospective data with confounding factors (Chapman et al., PMID 787372...