Pregnancy in Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder occurring in approximately 1:1000 individuals. ADPKD is characterized by gradual cyst expansion and kidney enlargement and is a slowly progressive disorder where patients typically initiate renal replacement therapy in the sixth decade of life. The vast majority of women with ADPKD become pregnant in the third or fourth decade, often before knowing that they have ADPKD, in the setting of normal kidney function or chronic kidney disease Stage 1. In ADPKD, pregnancy outcomes for mother and baby differ from the general population, and long-term consequences of maternal complications from pregnancy are common in ADPKD. In the current era of genetic testing, options to consider pre-implantation genetic screening are becoming more available. This chapter will review renal physiologic and anatomic changes that occur in pregnancy, the potential impact of ADPKD on maternal and fetal outcomes, medical management during pregnancy, the impact of pregnancy on long-term outcomes in women with ADPKD, and options for families with ADPKD planning to undergo pregnancy with regard to genetic testing.