Simultaneous Aortic Valve and Arch Replacement With Bilateral Nephrectomy for Massive Polycystic Kidney Disease, Aortic Regurgitation and Dissecting Aneurysm

Menon, Ashvini; Sachithanandan, Anand; Singh, Harjot; Bhatt, Rupesh I.; Lewis, Michael; Bonser, Robert S.

doi:10.1016/j.athoracsur.2010.08.012

Cited by 8 publications

(7 citation statements)

References 6 publications

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“…The study selection process is represented in Figure 3. We report data from 27 cases ( Table 1) of ADPKD patients with AD, of which 25 were previously identified from the literature [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] and two from our clinical experience. A schematic representation of age, hypertension and CKD stage distribution in the study population is reported in the Figure 4.…”

Section: Resultsmentioning

confidence: 99%

“…However, additional manifestations are often described, including: intracranial, coronary and other arterial aneurysms, arterial dolichoectasias, aortic or cervicocephalic arteries dissections, and heart valve diseases . Although ADPKD‐AD association has been previously reported, only few cases are available in the literature and a systematic review has never been made. We report two cases of AD in ADPKD patients from our experience and perform a systematic analysis of the literature.…”

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confidence: 99%

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

et al. 2015

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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.

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Section: Resultsmentioning

confidence: 99%

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confidence: 99%

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

et al. 2015

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“…Presence of a heterozygous hypomorphic allele of one of these genes results in a significantly milder cystic disease, which could manifest in a few kidney cysts and be radiologically interpreted as SRC. Clinically, the presence of an association between thoracic aortic dissection and ADPKD, as well as abdominal aortic aneurysm and ADPKD, has been modestly proposed in the literature based on a very small number of case reports; however, no cohort studies of these patients have yet been performed. It is possible that some of the patients in our study had a hypomorphic ADPKD allele, which would explain the presence of cysts in the kidneys of patients with thoracic aortic aneurysm and dissection.…”

Section: Discussionmentioning

confidence: 99%

Simple Renal Cysts as Markers of Thoracic Aortic Disease

Ziganshin

Theodoropoulos

Salloum

et al. 2016

JAHA

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BackgroundThoracic aortic aneurysm is usually a clinically silent disease; timely detection is largely dependent upon identification of clinical markers of thoracic aortic disease (TAD); (bicuspid aortic valve, intracranial aortic aneurysm, bovine aortic arch, or positive family history). Recently, an association of simple renal cysts (SRC) with abdominal aortic aneurysm and aortic dissection was established. The aim of our study was to evaluate the prevalence of SRC in patients with TAD in order to assess whether the presence of SRC can be used as a predictor of TAD.Methods and ResultsWe evaluated the prevalence of SRC in 842 patients with TAD (64.0% males) treated at our institution from 2004 to 2013 and compared to a control group of patients (n=543; 56.2% males). Patients were divided into 4 groups: ascending aortic aneurysm (456; 54.2%); descending aortic aneurysm (86; 10.2%); type A aortic dissection (118; 14.0%); and type B aortic dissection (182; 21.6%). SRC were identified by abdominal computed tomography or magnetic resonance imaging of these patients. Prevalence of SRC is 37.5%, 57.0%, 44.1%, and 47.3% for patients with ascending aneurysm, descending aneurysm, type A dissection, and type B dissection, respectively. Prevalence of SRC in the control group was 15.3%. Prevalence of SRC was not significantly different between male and female aortic disease patients, despite reported general male predominance (2:1), which was also observed in our control group (1.7:1).ConclusionsThis study establishes an increased prevalence of SRC in patients with TAD. SRC can potentially be used as a marker for timely detection of patients at risk of TAD.

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“…2 Different types of vascular manifestations have been reported in ADPKD such as aneurysms and/or dissections, predominantly involving the intracranial arteries but also the aorta, vertebral and coronary arteries. [3][4][5] The presence of this wide array of vascular abnormalities has led to the hypothesis that polycystins, the proteins encoded by the PKD genes, expressed in vascular smooth muscle cells, might be required to maintain vascular integrity. [6][7][8][9] Though some studies did not find any association between ADPKD and aortic aneurysm, 10 others find that there was increased incidence of aortic aneurysm in patients with ADPKD ranging from 5-9.7% of patients.…”

Section: Discussionmentioning

confidence: 99%

A tale of an unfortunate outcome in COVID-era

et al. 2020

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Autosomal dominant polycystic kidney disease (ADPKD) primarily affects the kidneys; cysts can develop in other organs like liver and pancreas and sometimes complicated with formation of arterial aneurysms, brain being the commonest site. Aortic aneurysms can lead to thromboembolic events. Thromboembolism is also a recognized complication of coronavirus disease 2019 (COVID-19). Here, we present case history of an elderly diabetic, hypertensive patient with bilateral renal stones, who presented with gangrene of the left 5th toe and raised serum creatinine. He was later diagnosed as a case of COVID-19, ADPKD with aortic aneurysm, which was complicated with intraluminal thrombus formation and unfortunately, he died in course of treatment. Birdem Med J 2020; 10, COVID Supplement: 115-119

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Simultaneous Aortic Valve and Arch Replacement With Bilateral Nephrectomy for Massive Polycystic Kidney Disease, Aortic Regurgitation and Dissecting Aneurysm

Cited by 8 publications

References 6 publications

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

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

Simple Renal Cysts as Markers of Thoracic Aortic Disease

A tale of an unfortunate outcome in COVID-era

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