Prevalence of Inferior Vena Cava Compression in ADPKD

Abstract

Introduction: Kidney and liver cysts in autosomal dominant polycystic kidney disease (ADPKD) can compress the inferior vena cava (IVC), but IVC compression prevalence and its risk factors are unknown.Methods: Patients who have ADPKD (n ¼ 216) with abdominal magnetic resonance imaging (MRI) studies and age-/sex-matched controls (n ¼ 216) were evaluated for IVC compression as well as azygous vein diameter (a marker of collateral blood flow) and IVC aspect ratio (left-to-right dimension divided by anterior-to-posterior dimension with a value of 1 corresponding to a circular (high pressure) IVC caudal to compression.Results: Severe IVC compression ($70%) was observed in 33 (15%) ADPKD subjects and mild compression ($50% to <70%) was observed in 33 (15%) subjects; whereas controls had no IVC compression (P < 0.001). Severe IVC compression was associated with larger azygous vein (4.0 AE 1.3 mm versus 2.3 AE 0.8 mm without IVC compression; P < 0.001) and a more circular IVC cross-section upstream (mean IVC aspect ratio: 1.16 AE 0.27 vs. 1.69 AE 0.67, P < 0.001), suggesting higher pressure upstream from the compression. IVC compression was associated with older age, lower estimated glomerular filtration rate (eGFR), greater height-adjusted total kidney volumes, greater height-adjusted liver volume (ht-LV), and greater liver and renal cyst fractions (P < 0.001). No subject younger than 30 years had IVC compression, but ADPKD subjects $40 years old had 12-fold higher risk of IVC compression (95% confidence interval [CI]: 4.2-42.4), with highest predicted probability for Mayo Clinic classes 1D (59%; 95% CI: 39%-76%) and 1E (74%; 95% CI: 49%-90%) after adjustment (P < 0.001). Women with ht-LV $ 2000 ml/m had 83% (95% CI: 59%-95%) prevalence of IVC compression. Complications of IVC compression included deep vein thrombosis (DVT) and symptomatic hypotension.Conclusions: IVC compression is common in ADPKD patients >40 years old, with Mayo Clinic class 1D/E, and in females with ht-LV > 2000 ml/m.

Severe polycystic liver disease: An unsual cause of chronic Budd-Chiari Syndrome

“…Firstly, the hypertrophy of the hepatic cysts can be the cause of compression of the inferior vena cava, or of the supra-hepatic veins responsible for chronic Budd Chiari syndrome, as is the case in our patient [5,7]. Secondly, some cysts developed in the hepatic hilum can compress the portal bifurcation responsible for portal hypertension (PH) which can lead to transudative ascites, especially if the patient is malnourished [17][18][19]. Finally, at advanced stages of the disease, the evolution can be done towards the development of fi brosis, see hepatic cirrhosis, with all its complications including ascites.…”

Recurrent thromboses and major vessel compressions in autosomal dominant polycystic kidney disease

“…This is consistent with our single-center experience, where most pretransplant nephrectomies were indicated for spatial considerations of allograft placement, which is in line with practices elsewhere and expert recommendations [ 14 ]. However, a recent radiological study identified up to 21% ADPKD patients with extrinsic IVC compression by renal cysts, although any physiologic implications remain to be determined [ 11 ].…”

“…Mass effect and compression of other organs had been reported in ADPKD, although disproportionately as a result of liver cysts rather than kidney cysts. Few cases of thromboembolic events (TEs) as a result of major vessel compression had been reported [ 6–10 ], although inferior vena cava (IVC) compression of unclear significance and post-transplantation TE risks appear to be common among ADPKD patients [ 11 , 12 ].…”

The crucial role of the nephrologist in the management of pregnancy in women with adult autosomal dominant polycystic kidney disease

“…Un'altra complicanza importante correlata all'ADPKD durante la gravidanza è la tromboembolia (32). A causa dell'ingrandimento dei reni, in particolare del rene destro, e dell'ingrossamento del fegato, le vene iliache e la vena cava inferiore possono essere compresse, aumentando il rischio trombotico e di embolia polmonare (46,47). Lo stato pro-coagulante della gravidanza aumenta ulteriormente questo rischio.…”