PKD1 and autosomal dominant polycystic kidney disease: For CKD stages G3b to G5, up to 30% of adult cases are attributable to Mendelian disorders.279 Autosomal dominant polycystic kidney disease is caused by loss-of-function variants in PKD1 or PKD2 and accounts for up to 10% of ESRD cases.280 Typical of monogenic kidney diseases, mortality in autosomal dominant polycystic kidney disease stems from the high prevalence of cardiovascular complications, with 60% of patients experiencing hypertension before GFR decline and 48% of hypertensive patients exhibiting LV hypertrophy.281