Aortic dissection in patients with autosomal dominant polycystic kidney disease: A series of two cases and a review of the Literature

Abstract

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.

Cardiovascular Complications of Energy Drinks

“…The sympathetic surge and associated acute increase in blood pressure and heart rate associated with ED consumption described above is associated with precipitation of aortic dissection in those with an underlying predisposition [1,68].…”

MRI in autosomal dominant polycystic kidney disease

“…Other aneurysms and vascular anomalies, including aneurysms of the aorta and coronary arteries, cervicocephalic and thoracic aortic dissections, aortic root dilatation, and cerebral dolichoectasia have been reported, but the available data are insufficient to recommend screening strategies …”

“…[44][45][46] Other aneurysms and vascular anomalies, including aneurysms of the aorta and coronary arteries, cervicocephalic and thoracic aortic dissections, aortic root dilatation, and cerebral dolichoectasia have been reported, but the available data are insufficient to recommend screening strategies. 45,47 MITRAL VALVE PROLAPSE. The prevalence of mitral valve prolapse detected with color Doppler echocardiography in a cohort of 109 ADPKD with PKD1 was 26%, compared with 14% in 73 unaffected relatives and 10% in 73 healthy controls.…”

Syndromes with aortic involvement: pictorial review

“…While the disease is most known for the development of enlarged kidneys with multiple bilateral cysts, a number of non-cystic abnormalities occur (1). Among these are cardiovascular manifestations, including intracranial aneurysms and dolichoectasia, aortic and cervicocephalic artery aneurysms and dissections, coronary artery aneurysms, atrial septal aneurysms, and mitral valve prolapse ( Figure 6) (1,26,27). Bicuspid aortic valve, aortic coarctation, and even interrupted aortic arch have been described in association with ADPKD (28).…”

“…However, screening may be reasonable in an ADPKD patient with a family history of a specific vascular abnormality such as aortic dilation (26). Moreover, vascular complications such as aortic dissection should be suspected in symptomatic patients with ADPKD despite their young age (27). As in other disorders, such findings are well demonstrated by CT and MRI.…”