title: The coexistence of acute aortic dissection with autosomal dominant polycystic kidney disease--description of two hypertensive patients.
abstract: BACKGROUND
  Acute aortic dissection is a rare but potentially lethal disease characterized by high early mortality when left untreated. However, survival may be significantly improved by the timely institution of appropriate medical and/or surgical therapy. Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary disorders, accounting for 8-10% of the cases of end-stage renal disease. Hypertension occurs frequently and is an early manifestation of ADPKD in approximately 50-70% of non-azotemic patients. ADPKD, often caused by mutations in the PKD1 gene, is associated with life-threatening vascular abnormalities that are commonly attributed to the frequent occurrence of hypertension.
  
  
  METHODS
  We present two cases of hypertensive patients with acute aortic dissection (type A Stanford) coexisting with ADPKD.
  
  
  RESULTS
  A 54-year-old male hypertensive patient and a 47-year-old male hypertensive patient, both with known ADPKD were diagnosed and operated with acute dissection of ascending aorta. Both patients were characterized by severe hypertension; therefore, in both cases, degree of hypertension is likely to be causative for this vascular complication. However, since ADPKD is associated with the vascular complications mentioned above, abnormality of the structure of the arterial wall of the thoracic aorta cannot be ruled out.
  
  
  CONCLUSIONS
  Aortic dissection is a rare but potentially disastrous complication, and clinicians should always consider this when dealing with patients with ADPKD who present with chest pain or collapse.
authors.affiliation: Department of Hypertension, Institute of Cardiology, Warsaw, Poland.
authors.forename: Mariola
authors.initials: M
authors.lastname: Peczkowska
authors.forename: Andrzej
authors.initials: A
authors.lastname: Januszewicz
authors.forename: Władysław
authors.initials: W
authors.lastname: Grzeszczak
authors.forename: Dariusz
authors.initials: D
authors.lastname: Moczulski
authors.forename: Hanna
authors.initials: H
authors.lastname: Janaszek-Sitkowska
authors.forename: Marek
authors.initials: M
authors.lastname: Kabat
authors.forename: Andrzej
authors.initials: A
authors.lastname: Biederman
authors.forename: Piotr
authors.initials: P
authors.lastname: Hendzel
authors.forename: Aleksander
authors.initials: A
authors.lastname: Prejbisz
authors.forename: Iwona
authors.initials: I
authors.lastname: Cendrowska-Demkow
authors.forename: Tomasz
authors.initials: T
authors.lastname: Zieliński
authors.forename: Magdalena
authors.initials: M
authors.lastname: Januszewicz
mesh_terms: D000784:Aortic Dissection / Q000209:etiology* / Q000601:surgery; D006801:Humans; D006973:Hypertension / Q000150:complications* / Q000188:drug therapy / Q000601:surgery; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000235:genetics