Table 4.

Rupture rates of intracranial aneurysms in patients with ADPKD

Author/Study (Referencea)CountryPatient No.Sex, % WomenAge,b yrUIAsFollow-Up, Patient-yrUIAs TreatedUIA RupturesRupture Rate per 100 Patient-yr (95% CI)
Patients with intracranial aneurysms detected by presymptomatic screening
Xu (19)China40535349144c000
Flahault (2)France19744122112d61e0.893 (0 to 2.6)
SanchisUnited States75645194668c700
All134631659241310.108 (0 to 0.3)
Patients with a negative presymptomatic screening
Schrier (3)United States1361247d00
Flahault (2)France1626740956d1f0.105 (0 to 0.31)
SanchisUnited States73756514783c2g0.041 (0 to 0.10)
All103554698630.043 (0 to 0.09)
Concurrent patients who had no presymptomatic screening
Flahault (2)France3041794d3h0.167 (0 to 0.36)
Patients participating in clinical trials
CRISP I-III (20)United States2416032274700
REPRISE (21)Global1366504716053i0.187 (0 to 0.40)
TEMPO 3:4 (22)Global1444483939572j0.051 (0 to 0.12)
TEMPO 4:4 (23)Global1083474235291 (1)k0.028 (0 to 0.08)
HALT PKD A (24)United States558493731250l0
HALT PKD B (25)United States486524925271 (1)m0.040 (0 to 0.12)
EVEROLIMUS (26)Germany433494484600
SUISSE (27)Switzerland1003932158d00
ALADIN (28)Italy855337219c0n0
DIPAK (29)Netherlands305534870200
All609519,41570.04 (0.01 to 0.06)
Total756829,119140.05 (0.02 to 0.07)
  • UIA, unruptured intracranial aneurysm; 95% CI, 95% confidence interval; CRISP I-II, Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease; REPRISE, Replicating Evidence of Preserved Renal Function: an Investigation of Tolvaptan Safety and Efficacy in ADPKD; TEMPO, Tolvaptan efficacy and safety in management of autosomal dominant polycystic kidney disease and its outcomes; HALT PKD, Halt Polycystic Kidney Disease; EVEROLIMUS, ADPKD clinical trial; SUISSE, ADPKD clinical trial; ALADIN, Long-acting somatostatin on disease progression in nephropathy due to autosomal dominant polycystic kidney disease; DIPAK, Developing interventions to halt progression of ADPKD.

  • a Information was obtained from the referenced articles and/or principal investigators in large clinical trials for autosomal dominant polycystic kidney disease.

  • b Mean or median.

  • c Estimated from the number of patients and mean follow-up.

  • d Estimated from the number of patients and median follow-up.

  • e Anterior communicating artery.

  • f Pericallosal artery.

  • g Middle cerebral artery and basilar artery.

  • h Anterior communicating artery in all three.

  • i Two patients randomized to placebo and one patient randomized to tolvaptan. First patient: intracranial aneurysm at basilar artery and paraclinoid segment of internal carotid artery (site of a previous clipping procedure 9 years earlier). Second patient: basilar tip and anterior cerebral artery. Third patient: intracranial aneurysm location not available. One additional patient randomized to placebo had surgical clipping of a ruptured aneurysm 11 years before enrolment in the REPRISE; during the REPRISE, the patients had a fall and a traumatic subarachnoid hemorrhage that resolved without intervention.

  • j One patient randomized to placebo and one patient randomized to tolvaptan. First patient: middle cerebral artery. Second patient: location not available. One additional patient had an intracranial hemorrhage without evidence of aneurysm.

  • k Middle cerebral artery. One additional patient suffered an aneurysmal rupture 10 months after concluding his participation in the TEMPO 4:4 after starting hemodialysis and having a bilateral nephrectomy.

  • l One patient had a subdural hematoma.

  • m This patient had a ruptured anterior communicating artery aneurysm clipped 8 years before enrolment, and a recurrent aneurysm between the clips ruptured during the study. One additional patient had a subdural hematoma, and another had an intracranial hemorrhage due to an arteriovenous malformation.

  • n One unruptured intracranial aneurysm was detected by presymptomatic screening.