Acute pulmonary thromboembolism occurring during treatment with tolvaptan in a patient with autosomal-dominant polycystic kidney disease

Autosomal-dominant polycystic kidney disease (ADPKD) is the most prevalent cystic kidney disease, with approximately half of the patients reaching end-stage renal disease by the age of 60. Tolvaptan prevents renal cyst growth by inhibiting intracellular cyclic AMP and is recommended for patients with ADPKD. Reports of thrombotic complications with tolvaptan have been limited. We report a case of a 60-year-old man who developed thromboembolisms during tolvaptan treatment for ADPKD. The patient started tolvaptan in July 2014. He was brought to our hospital in February 2015 with a sudden onset of dyspnea and chest pain after 6 days of persistent watery diarrhea. Blood tests revealed enhanced coagulation and fibrinolysis, and contrast-enhanced computed tomography confirmed the presence of multiple thromboembolisms. Venous thromboembolism (VTE) with acute pulmonary and lower extremity thrombi was diagnosed, and the patient was immediately admitted. Tolvaptan was discontinued on admission, and intravenous fluid loading and monteplase were started. Subsequently, chest pain and dyspnea resolved, with thrombi resolution occurring by day 14; the patient was discharged on day 18 in stable condition. VTE was attributed to continued tolvaptan during diarrhea and dehydration; tolvaptan itself was not associated with enhanced coagulability. Dehydrated patients with ADPKD, such as the patient in this case, are at an increased risk for thrombus formation. Proper education should be provided to maintain appropriate fluid status and discontinue tolvaptan upon volume depletion.