title: Aortic dissection in patients with autosomal dominant polycystic kidney disease: a series of two cases and a review of the literature.
abstract: Aortic dissection (AD) is the most common life-threatening disease involving the aorta. It is rarely associated with systemic disorders such as Autosomal Dominant Polycystic Kidney Disease (ADPKD), a genetic syndrome characterized by cystic degeneration of kidneys, possible presence of cysts in other organs and extra-renal manifestations, including cardiovascular disorders. We performed a systematic literature search focused on the occurrence of AD associated with ADPKD (25 cases identified), and reported two cases from our experience. We selected data on sex, age, family history of ADPKD and/or AD, habitus, hypertension, renal function, presence of hepatic/pancreatic/splenic cysts, clinical presentation of AD, AD type according to the Stanford classification, treatment and outcome. Furthermore we compared this dataset with the data of the overall population with AD from the International Registry of Acute Aortic Dissection (IRAD). Stanford A type AD was documented in 62% of patients. As expected, the initial manifestation of AD was most commonly chest and back pain (80%). The mean age of AD occurrence appears significantly reduced in ADPKD patients compared to the general population with AD (49 ± 12 vs 62 ± 14, P < 0.001). Of note, our analysis shows a remarkably higher frequency of hypertension (90%) compared to the overall AD population (75%), although not significantly (P = 0.133). AD should be always ruled out in ADPKD subjects with chest and back pain symptoms, despite them being young and at lower risk. A careful preventive monitoring as well as an optimal blood pressure control may reduce the risk of AD and improve the outcome of these patients.
authors.affiliation: Heart Department, University Hospital 'San Giovanni di Dio e Ruggi d'Aragona', Salerno, Italy.
authors.forename: Angelo
authors.initials: A
authors.lastname: Silverio
authors.forename: Costantina
authors.initials: C
authors.lastname: Prota
authors.forename: Marco
authors.initials: M
authors.lastname: Di Maio
authors.forename: Maria Vincenza
authors.initials: MV
authors.lastname: Polito
authors.forename: Francesco Maria
authors.initials: FM
authors.lastname: Cogliani
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Citro
authors.forename: Alberto
authors.initials: A
authors.lastname: Gigantino
authors.forename: Severino
authors.initials: S
authors.lastname: Iesu
authors.forename: Federico
authors.initials: F
authors.lastname: Piscione
mesh_terms: D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D001027:Aortography / Q000379:methods; D001416:Back Pain / Q000209:etiology; D019917:Blood Vessel Prosthesis Implantation; D002637:Chest Pain / Q000209:etiology; D057510:Endovascular Procedures; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis; D012307:Risk Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: aortic dissection; autosomal dominant policystic kidney disease; chronic kidney disease; hypertension