32 years old woman is referred to the emergency room for kidney failure that was discovered in pre-employment screening examination. The patient has blood urea nitrogen (BUN) of 100 mg/dL (normal: 8-20 mg/dL) And serum creatinine of 8.4mg/dL ( normal 0.7-1.3 mg/dL ). Her only complaint is bilateral flank pain. family history reveals that her mother and one sibling have a renal failure and receive hemodialysis. her mother has had a recent stroke the patient’s blood pressure is 170/100 mm Hg. Heart examination reveals a mid systolic click and murmur that increases with Valsalva maneuver. kidneys are palpable bilaterally. which of the following is the most likely diagnosis?