The Significance of Congenital Portosystemic Shunts in Congenital Heart Disease and the Bizarre Phenomenon of Alternating Portosystemic and Systemic-Portal Shunting.

Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article.

Blood flows from the SCN toward the OVLT within a new brain vascular portal pathway.

The suprachiasmatic nucleus (SCN) sets the phase of oscillation throughout the brain and body. Anatomical evidence reveals a portal system linking the SCN and the organum vasculosum of the lamina terminalis (OVLT), begging the question of the direction of blood flow and the nature of diffusible signals that flow in this specialized vasculature. Using a combination of anatomical and in vivo two-photon imaging approaches, we unequivocally show that blood flows unidirectionally from the SCN to the OVLT, that blood flow rate displays daily oscillations with a higher rate at night than in the day, and that circulating vasopressin can access portal vessels. These findings highlight a previously unknown central nervous system communication pathway, which, like that of the pituitary portal system, could allow neurosecretions to reach nearby target sites in OVLT, avoiding dilution in the systemic blood. In both of these brain portal pathways, the target sites relay signals broadly to both the brain and the rest of the body.

Comparison of hematologic variables between dogs with congenital intrahepatic and extrahepatic portosystemic shunts.

BACKGROUND: Dogs with congenital intrahepatic portosystemic shunt (IHPSS) are predisposed to gastrointestinal inflammation, ulceration, and bleeding, unlike dogs with congenital extrahepatic portosystemic shunt (EHPSS). Limited information is available about hematologic differences between dogs with IHPSS and dogs with EHPSS. OBJECTIVE: Compare hemogram variables between dogs with IHPSS and EHPSS. We hypothesized that hematologic variables would differ between the 2 populations, with a higher frequency and severity of anemia and microcytosis in dogs with IHPSS. ANIMALS: Twenty-six client-owned dogs with IHPSS and 35 client-owned dogs with EHPSS. METHODS: Retrospective cross-sectional study. Dogs were included if a CBC was performed before shunt attenuation. Contingency analysis was performed to determine if the frequency of clinical signs and of hematologic variables below the reference range differed between groups. Hematologic and selected biochemical variables were compared between groups using an analysis of covariance with age as a covariate. RESULTS: Gastrointestinal clinical signs (IHPSS, 81% vs EHPSS, 34%; P = .01), anemia (31% vs 6%; P = .01), microcytosis (77% vs 29%; P = .002), and hypochromia (77% vs 49%; P = .03) were more common in dogs with IHPSS than in dogs with EHPSS. Dogs with IHPSS had lower packed cell volume (34% vs 41%, P = .04), hemoglobin concentration (11.5 g/dL vs 13.7 g/dL, P = .03), mean corpuscular volume (57 fL vs 65 fL; P = .001), and mean corpuscular hemoglobin concentration (32 g/dL vs 33 g/dL; P = .04) than dogs with EHPSS. CONCLUSIONS AND CLINICAL IMPORTANCE: Dogs with IHPSS had a higher frequency of anemia, microcytosis, and hypochromia and exhibited more gastrointestinal clinical signs.

Anatomical classification of feline congenital extrahepatic portosystemic shunts based on CT angiography: A SVSTS and VIRIES multi-institutional study in 231 cats.

The prevalence of anatomical-based subtypes of feline congenital extrahepatic portosystemic shunts (EHPSS) has not been completely elucidated. The goal of this study was to use CT angiography to create an anatomical-based nomenclature system for feline congenital EHPSS. Additionally, subjective portal perfusion scores were generated to determine if intrinsic portal vein development was associated with different shunt conformations or patient age at the time of CT. The SVSTS and VIRIES list services were used to recruit cases. Data collected included patient DOB, gender, breed, weight, CT date, and reported diagnosis. Shunts were classified based upon (1) the shunt portal vessel(s) of origin, (2) the shunt systemic vessel(s) of insertion, and (3) any substantial portal vessels contributing to the shunt. Additionally, hepatic portal perfusion was subjectively scored between 1 (poor/none) and 5 (good/normal) based on the caliber of the intrahepatic PVs. A total of 264 CT scans were submitted from 29 institutions. Due to exclusion criteria, 33 (13%) were removed, leaving 231 CT scans to be included. Twenty-five different EHPSS anatomies were identified with five classifications accounting for 78% of all shunts (LGP [53%], LGC-post [11%], LCG [7%], LGC-pre [4%], and PC [4%]). Shunt origin involved the left gastric vein in 75% of the described classifications. Significant differences were identified among the five most common shunt types with respect to age at the time of CT scan (P = .002), breed (P < .001), and subjective portal perfusion score (P < .001). This refined anatomical classification system for feline EHPSS may enable improved understanding, treatment comparisons, and outcome prediction for cats with these anomalies.

The development of early human lymphatic vessels as characterized by lymphatic endothelial markers.

Lymphatic vessel development studies in mice and zebrafish models have demonstrated that lymphatic endothelial cells (LECs) predominantly differentiate from venous endothelial cells via the expression of the transcription factor Prox1. However, LECs can also be generated from undifferentiated mesoderm, suggesting potential diversity in their precursor cell origins depending on the organ or anatomical location. Despite these advances, recapitulating human lymphatic malformations in animal models has been difficult, and considering lymphatic vasculature function varies widely between species, analysis of development directly in humans is needed. Here, we examined early lymphatic development in humans by analyzing the histology of 31 embryos and three 9-week-old fetuses. We found that human embryonic cardinal veins, which converged to form initial lymph sacs, produce Prox1-expressing LECs. Furthermore, we describe the lymphatic vessel development in various organs and observe organ-specific differences. These characterizations of the early development of human lymphatic vessels should help to better understand the evolution and phylogenetic relationships of lymphatic systems, and their roles in human disease.

Enlargement of the hepatic artery is present in dogs with a congenital extrahepatic portosystemic shunt and is independent of shunt insertion into the systemic circulation.

The accurate diagnosis of portovascular anomalies has been facilitated by improvements in diagnostic imaging technology. In humans, hepatic arterial blood flow changes in response to the reduction in portal blood flow. The hepatic arterial buffer response characterizes an intrinsic regulatory mechanism in response to reduced portal venous blood flow, which results in hepatic arterial enlargement. At the authors' institution, enlargement of the hepatic artery has been anecdotally observed in a population of dogs with extrahepatic portosystemic shunting, consistent with previous literature that documents variability in hepatic arterial size. In this retrospective, blinded, analytical study, a hepatic artery:aorta (Ha:Ao) ratio was assessed on CT studies from 112 dogs, with (n = 43) and without (n = 69) an extrahepatic congenital portosystemic shunt in order to compare the hepatic artery size independent of body weight between the two populations. A significant increase in the Ha:Ao ratio was documented in dogs with an extrahepatic portosystemic shunt (EHPSS) compared with those dogs with no EHPSS independent of the location of shunt insertion into the systemic circulation (P < .001). Three cases had repeat CT after surgery, and all had Ha:Ao ratio reductions following treatment. The authors propose that this may be an additional imaging feature observed in dogs with an EHPSS.

In vivo testing of novel nitric oxide-releasing nanoparticles for alleviating heart failure using the zebrafish embryo model.

Heart failure (HF) is a multifactorial, heterogeneous systemic disease that is considered one of the leading causes of death and morbidity worldwide. It is well-known that endothelial dysfunction (ED) plays an important role in cardiac disease etiology. A reduction in the bioavailability of nitric oxide (NO) in the bloodstream leads to vasoconstriction and ED. Many studies indicated diminishment of peripheral arteries vasodilation that is mediated by the endothelium in the of patients with chronic HF. With the advancement of nanomedicine, nanotechnology can provide adequate solutions for delivering exogenous NO with the aid of nanoparticles (NPs) to treat ED. The properties of superparamagnetic iron oxide nanoparticles (SPIONs) enable both passive and active delivery of drugs. This prompted us to investigate the efficacy of our newly-developed hydrogel nanoparticles (NO-RPs) for the delivery and sustained release of NO gas to alleviate cardiac failure and inflammation in the heart failure zebrafish model. The hydrogel NO-RPs incorporate SPIONS and NO precursor. The sustainend release of NO in the NO-RPs (4200 s), overcomes the problem of the short half life of NO in vivo which is expected to ameliorate the reduced NO bioavailabilty, and its consequences in endothelial and cardiac dysfunction. Zebrafish embryos were used as the animal model in this study to determine the effect of SPIONs-loaded NO-RPs on the cardiovascular system. Cardiac failure was induced in 24hpf embryos by exposure to aristolochic acid (AA)(0.25, 0.5 µM) for 8 h, followed by the SPIONs-loaded NO-RPs (0.25, 0.5 mg/ml) for 48 h, experimental groups included: control group which is healthy non treated zebrafish embryos, AA injured zebrafish embryos (HF) model,and NO-RP treated HF zebrafish embryos. Survival rate was assessed at 72hpf. Cardiac function was also evaluated by analyzing cardiac parameters including heartbeat, major blood vessels primordial cardinal vein and dorsal aorta (PCV &DA) diameter, blood flow velocity in PCV & DA vessels, cardiac output, and PCV & DA shear stresses. All cardiac parameters were analyzed with the aid of MicroZebraLab blood flow analysis software from Viewpoint. In addition, we studied the molecular effects of the developed NO-RPs on the mRNA expression of selected pro-inflammatory markers: IL-6, and Cox-2. Our findings demonstrated that the NO-RPs improved the survival rate in the heart failure zebrafish model and reversed heart failure by enhancing blood flow perfusion in Zebrafish embryos, significantly. In addition, RT-PCR results showed that the NO-RPs significantly reduced the expression of pro-inflammatory markers (lL-6&COX-2) in the heart failure zebrafish model. Our study confirmed that the developed SPIONs-loaded NO-RPs are effective tool to alleviate cardiac failure and inflammation in the HF zebrafish model.

Live imaging of Fibronectin 1a-mNeonGreen and Fibronectin 1b-mCherry knock-in alleles during early zebrafish development.

Within the developing embryo, cells assemble and remodel their surrounding extracellular matrix during morphogenesis. Fibronectin is an extracellular matrix glycoprotein and is a ligand for several members of the Integrin adhesion receptor family. Here, we compare the expression pattern and loss of function phenotypes of the two zebrafish fibronectin paralogs fn1a and fn1b. We engineered two fluorescently tagged knock-in alleles to facilitate live in vivo imaging of the Fibronectin matrix. Genetic complementation experiments indicate that the knock-in alleles are fully functional. Fn1a-mNeonGreen and Fn1b-mCherry are co-localized in ECM fibers on the surface of the paraxial mesoderm and myotendinous junction. In 5-days old zebrafish larvae, Fn1a-mNeonGreen predominantly localizes to the branchial arches, heart ventricle, olfactory placode and within the otic capsule while Fn1b-mCherry is deposited at the pericardium, proximal convoluted tubule, posterior hindgut and at the ventral mesoderm/cardinal vein. We examined Fn1a-mNeonGreen and Fn1b-mCherry in maternal zygotic integrin α5 mutants and integrin ß1a; ß1b double mutants and find distinct requirements for these Integrins in assembling the two Fibronectins into ECM fibers in different tissues. Rescue experiments via mRNA injection indicate that the two fibronectins are not fully inter-changeable. Lastly, we examined cross-regulation between the two Fibronectins and find fn1a is necessary for normal Fn1b fibrillogenesis in the presomitic mesoderm, but fn1b is dispensable for the normal pattern of Fn1a deposition.

Effects and mechanisms of Porphyromonas gingivalis outer membrane vesicles induced cardiovascular injury.

BACKGROUND: The outer membrane vesicles (OMVs) derived from Porphyromonas gingivalis (P. gingivalis) have long been acknowledged for their crucial role in the initiation of periodontitis. However, the implications of P. gingivalis OMVs in the context of cardiovascular disease (CVD) remain incompletely understood. This study aimed to clarify both the impact and the underlying mechanisms through which P. gingivalis OMVs contribute to the propagation of distal cardiovascular inflammation and trauma. METHODS: In this study, various concentrations (0, 1.25, 2.5, and 4.5 µg/µL) of P. gingivalis OMVs were microinjected into the common cardinal vein of zebrafish larvae at 48 h post-fertilization (hpf) to assess changes in cardiovascular injury and inflammatory response. Zebrafish larvae from both the PBS and the 2.5 µg/µL injection cohorts were harvested at 30 h post-injection (hpi) for transcriptional analysis. Real-time quantitative PCR (RT-qPCR) was employed to evaluate relative gene expression. RESULTS: These findings demonstrated that P. gingivalis OMVs induced pericardial enlargement in zebrafish larvae, caused vascular damage, increased neutrophil counts, and activated inflammatory pathways. Transcriptomic analysis further revealed the involvement of the immune response and the extracellular matrix (ECM)-receptor interaction signaling pathway in this process. CONCLUSION: This study illuminated potential mechanisms through which P. gingivalis OMVs contribute to CVD. It accentuated their involvement in distal cardiovascular inflammation and emphasizes the need for further research to comprehensively grasp the connection between periodontitis and CVD.

Comparison of the diagnostic yield of 3 and 5 mm laparoscopic liver biopsy forceps in cats.

OBJECTIVE: To determine whether 3 and 5 mm laparoscopic cup biopsy forceps provide samples of equivalent diagnostic quality in cats. STUDY DESIGN: Experimental study. ANIMALS: Twelve colony cats undergoing a concurrent nutrition study. METHODS: Two biopsy forceps (3 and 5 mm) and three biopsy techniques (twist, pull, and twist + pull) were used to collect 68 laparoscopic liver samples. Biopsies were performed consecutively with the 3 and 5 mm biopsy sites adjacent to each other. Data analyzed included the number of portal triads and hepatic lobules, tissue crush and fragmentation, overall sample area (mm2 ), sample weight, and agreement regarding morphologic diagnosis. RESULTS: The 5 mm forceps provided more hepatic lobules, portal triads, and a larger tissue weight and histologic area (mm2 ) (p < .01). The twist and pull techniques provide more hepatic lobules and portal triads compared to the twist + pull technique while the twist + pull technique resulted in greater tissue crush compared to the twist technique (p = .0097). There was good agreement for morphological diagnosis between the 3 and 5 mm samples using the twist + pull technique but not for the twist or pull techniques. CONCLUSION: Liver samples can be safely collected with 3 or 5 mm laparoscopic biopsy forceps and provide sufficient tissue for histopathology analysis in cats, with minimal artifact. The diagnostic accuracy of 3 mm samples remains unknown. CLINICAL SIGNIFICANCE: Although 3 mm laparoscopic cup biopsy forceps provided samples of sufficient diagnostic quality for histopathologic interpretation in cats, further studies are required to assess their diagnostic accuracy.

Clinical presentation and short-term outcomes of dogs ≥15 kg with extrahepatic portosystemic shunts.

OBJECTIVE: To describe demographics, clinical presentation, shunt anatomy, clinical progression, and complications in large dogs ≥15 kg with single extrahepatic portosystemic shunts (EHPSS) treated with or without surgery. STUDY DESIGN: Multicenter retrospective (10 university hospitals, one private referral institution). ANIMALS: Dogs ≥15 kg (n = 63). METHODS: Medical records of dogs ≥15 kg diagnosed with EHPSS between January 01, 2005 and December 31, 2020 were reviewed. Dogs had a minimum follow-up of 90 days. Signalment, clinical signs, diagnostics, shunt anatomy, treatment interventions, and perioperative complications were assessed. RESULTS: Median age was 21.9 months (IQR: 9-36.8). The breed most represented was the Golden retriever (17/63 dogs). Portocaval (17/63) and splenocaval (15/63) shunt configurations were most common. Portal vein hypoplasia was noted in 18 imaging reports. Of the surgically treated dogs, 14/45 (35.6%) had short-term complications, and 3/45 (6.7%) had shunt-related deaths. Medical management was discontinued in 15/40 and reduced in 9/40 of surviving dogs who had surgical attenuation. All medically managed, nonattenuated dogs (18/18) were maintained on their original shunt-related medication regimens. CONCLUSIONS: Clinical presentation of dogs ≥15 kg with extrahepatic portosystemic shunts was similar to the more commonly reported small breed dogs. Surgical management of single EHPSS in large dogs ≥15 kg had similar clinical short-term outcomes as small breed dogs. CLINICAL SIGNIFICANCE: Clinicians should be aware that large breed dogs with EHPSS share similar characteristics and clinical outcomes to small breed dogs. The significance of the presence of a hypoplastic portal vein warrants further research. Surgical treatment is a viable option for large breed dogs with EHPSS.

Clinical outcomes for 20 cats with congenital extrahepatic portosystemic shunts treated with ameroid constrictor ring attenuation (2002-2020).

OBJECTIVE: To report the clinical perioperative, short-term, and long-term outcomes for cats undergoing ameroid ring constrictor (ARC) attenuation of a congenital extrahepatic portosystemic shunt (EHPSS). STUDY DESIGN: Retrospective case series from a single veterinary teaching hospital (2002-2020). ANIMALS: Twenty client-owned cats with EHPSS. METHODS: Data collected from medical records included signalment, history, physical examination, clinicopathologic testing, medications, diagnostic imaging, intraoperative findings, perioperative complications, and postoperative clinical outcomes. Long-term clinical outcome was obtained from a standardized owner interview or medical records. RESULTS: Perioperative complications were reported in five cats out of 20, including blindness (two cats), ascites (one cat), head pressing (one cat), and seizures and death (one cat). Short-term clinical outcome was excellent in 14/18 cats, good in 2/18 cats, and poor in 2/18 cats that were available for follow up, and long term clinical outcome was excellent in 15/18, good in 1/18 cats, and poor in 2/18 cats that were available for follow up. CONCLUSION: Long-term clinical outcome was good or excellent in 16/18 of cats available for follow up. Perioperative complications were reported in five cats. CLINICAL SIGNIFICANCE: Surgical attenuation of EHPSS with an ARC can result in resolution of clinical signs and biochemical abnormalities in the majority of cats. The perioperative complication rate for feline patients with EHPSS attenuated with an ARC was lower than reported historically. Seizures may persist in the long term despite normal bile acid stimulation test results, complete blood count, and biochemistry analysis.

Patient-Caregiver Portal System in Palliative Oncology: Assessment of Usability and Perceived Benefit.

BACKGROUND: The engagement of family caregivers in oncology is not universal or systematic. OBJECTIVE: We implemented a process intervention (ie, patient-caregiver portal system) with an existing patient portal system to (1) allow a patient to specify their caregiver and communication preferences with that caregiver, (2) connect the caregiver to a unique caregiver-specific portal page to indicate their needs, and (3) provide an electronic notification of the dyad's responses to the care team to inform clinicians and connect the caregiver to resources as needed. METHODS: We assessed usability and satisfaction with this patient-caregiver portal system among patients with cancer receiving palliative care, their caregivers, and clinicians. RESULTS: Of 31 consented patient-caregiver dyads, 20 patients and 19 caregivers logged in. Further, 60% (n=12) of patients indicated a preference to communicate equally or together with their caregiver. Caregivers reported high emotional (n=9, 47.3%), financial (n=6, 31.6%), and physical (n=6, 31.6%) caregiving-related strain. The care team received all patient-caregiver responses electronically. Most patients (86.6%, 13/15 who completed the user experience interview) and caregivers (94%, 16/17 who completed the user experience interview) were satisfied with the system, while, of the 6 participating clinicians, 66.7% agreed "quite a bit" (n=1, 16.7%) or "very much" (n=3, 50%) that the system allowed them to provide better care. CONCLUSIONS: Our findings demonstrate system usability, including a systematic way to identify caregiver needs and share with the care team in a way that is acceptable to patients and caregivers and perceived by clinicians to benefit clinical care. Integration of a patient-caregiver portal system may be an effective approach for systematically engaging caregivers. These findings highlight the need for additional research among caregivers of patients with less advanced cancer or with different illnesses.

Percutaneous transvenous coil embolization (PTCE) for treatment of single extrahepatic portosystemic shunt in dogs.

BACKGROUND: There is limited information regarding percutaneous transvenous coil embolization (PTCE) for single extrahepatic portosystemic shunt (PSS). This study aimed to describe the procedure and outcome of PTCE in dogs with a single extrahepatic PSS. Forty-two privately owned dogs were included in this study. All dogs were diagnosed with extrahepatic PSS by computed tomography (CT). Preoperative CT images were used to evaluate the diameter of the PSS for coil placement. A multipurpose balloon catheter was percutaneously inserted into the PSS via the jugular vein, and transvenous retrograde portography (TRP) and measurement of blood pressure in the PSS (pPSS) were performed during balloon inflation; one or more embolization coils were implanted via the catheter. RESULTS: In most cases, preoperative median fasting and postprandial serum total bile acid (TBA) concentrations were high (fasting, 86.5 µmol/L [ 3.7-250.0 µmol/L]; postprandial, 165.5 µmol/L [ 1.5-565.0 µmol/L]). CT revealed that 30 dogs had left gastrophrenic shunt; eight had left gastroazygos shunt; and one each had left gastrocaval, splenocaval, splenophrenic, and left colocaval shunt. TRP revealed that intrahepatic portal vascularity was clearly detectable in all dogs. The median values of pPSS before and during the balloon occlusion were 4.8 mmHg [2.0-13.0 mmHg] and 8.6 mmHg [5.0-18.0 mmHg], respectively. The median number and diameter of coils used were 2 coils [1 - 5 coils] and 8.0 mm [4.0 - 12.0 mm], respectively. The median times of irradiation and PTCE were 9 min [4-26 min] and 40 min [23-75 min], respectively. The median fasting and postprandial TBAs significantly decreased to 8.2 µmol/L [0.3-45.1 µmol/L, n = 38, p = 0.0028] and 19.8 µmol/L [0.3-106.7 µmol/L, n = 38, p = 0.0018], respectively, approximately 1 month after PTCE. The clinical success rate of PTCE without requirement for a second surgery was 95.2% (40/42 dogs). During revision surgery, one dog underwent surgical ligation and, in another dog, an ameroid constrictor was placed. CONCLUSIONS: PTCE was clinically effective in treating single extrahepatic PSS in dogs. Preoperative CT and TRP prior to PTCE might be clinically valuable for choosing the size of embolization coils, deciding the appropriate location of coil implantation, and estimating the number of coils to be implanted. PTCE is a promising alternative to conventional surgical procedures for single extrahepatic PSS in dogs.

Computed tomographic measures of renomegaly vary among dogs with different types of congenital portosystemic shunts.

Renomegaly has been reported in dogs with congenital portosystemic shunts (PSS). However, no study has objectively evaluated the degree of renomegaly in dogs with different types of PSS. The purpose of this retrospective, analytical, cross-sectional study was to determine kidney size (renal length-to-L2 vertebral body ratio; RL/L2 ratio) using CT in dogs with different types of PSS and correlate with clinical information. A medical record search for dogs with a PSS diagnosed using CT between 2016 and 2020 was conducted. Breed, age, sex, body weight, and biochemistry results were recorded. Kidney and L2 vertebral body lengths were measured using multiplanar reformatted CT images, and the RL/L2 ratio was calculated. Dogs were categorized into four groups based on PSS morphology for comparisons: intrahepatic (IH; n = 19), extrahepatic portocaval (EHPC; n = 20), extrahepatic portoazygos (EHPA; n = 7), or extrahepatic portophrenic (EHPP, n = 7). The RL/L2 ratio (mean ± SD) was largest in IH (3.55 ± 0.38) and EHPC (3.55 ± 0.38), followed by EHPP (3.10 ± 0.23), and EHPA (2.78 ± 0.18). RL/L2 ratio was significantly larger in EHPC and IH (vs. EHPA and EHPP [P < .01]). Significant correlations between kidney size and creatinine, alkaline phosphatase, albumin, total protein, and ammonia were present. Renomegaly was observed in 86.8% of dogs with PSS overall, but it was uncommon in dogs with EHPA and less common in dogs with EHPP, as these two groups showed clinical signs later in life, made evident by older age at presentation. The authors suggest that the severity of hepatic dysfunction and the shunted blood volume may influence the development of renomegaly in dogs with PSS.

The Portal Vein: A Comprehensive Review.

Radiologists are familiar with the appearances of a normal portal vein; variations in its anatomy are commonplace and require careful consideration due to the implications for surgery. These alterations in portal vein anatomy have characteristic appearances that are clearly depicted on CT, MR, and US images. Similarly, there are numerous congenital and acquired disorders of the portal vein that are deleterious to its function and can be diagnosed by using imaging alone. Some of these conditions have subtle imaging features, and some are conspicuous at imaging but poorly understood or underrecognized. The authors examine imaging appearances of the portal vein, first by outlining the classic and variant anatomy and then by describing each of the disorders that impact portal vein function. The imaging appearances of portal vein abnormalities discussed in this review include (a) occlusion from and differentiation between bland thrombus and tumor in vein and the changes associated with resultant hepatic artery buffer response changes, cavernous transformation of the portal vein, and portal biliopathy; (b) ascending thrombophlebitis of the portal vein (pylephlebitis); (c) portal hypertension and its causes and sequelae; (d) the newly described disease entity portosinusoidal vascular disorder; and (e) intra- and extrahepatic shunts of the portal system, both congenital and acquired (including Abernethy malformations), and the associated risks. Current understanding of the pathophysiologic processes of each of these disorders is considered to aid the approach to reporting. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Repeated specific canine pancreatic lipase measurements do not identify multiple acquired portosystemic shunts in dogs after extrahepatic portosystemic shunt attenuation.

BACKGROUND: In dogs with portal hypertension (PH), spec cPL is suggested to be increased despite normal pancreatic histology. After attenuation of congenital extrahepatic portosystemic shunts (cEHPSS), multiple acquired portosystemic shunt (MAPSS) can develop as consequence of sustained PH. Presence of MAPSS affects future therapeutic options and prognosis. OBJECTIVE: Evaluate if spec cPL concentrations increase postoperatively in dogs that develop MAPSS and can thus serve as an indicator of PH. ANIMALS: Twenty-four dogs with cEHPSS. METHODS: Dogs classified according to surgical outcome after cEHPSS attenuation (8 with MAPSS [group M], 9 with closed cEHPSS [group C] and 7 with patent blood flow through the original cEHPSS, without evidence of MAPSS [group P]). Spec cPL was measured in preoperative samples (T0), 4 days (T1) and 1 (T2) and 3- to 6-months (T3) after surgery. RESULTS: Spec cPL was within reference interval (<200 µg/L) at all timepoints except at T1. At T1, 2 dogs in group M (321 and >2000 µg/L) and also 1 in group C (688 µg/L) and 1 in group P (839 µg/L) had increased spec cPL concentrations. No differences in spec cPL concentrations between groups or changes over time were identified. CONCLUSIONS AND CLINICAL IMPORTANCE: Spec cPL is not consistently increased in dogs that develop MAPSS after cEHPSS attenuation and has no potential as a biomarker for the identification of MAPSS after cEHPSS attenuation.

Urine-based Detection of Congenital Portosystemic Shunt in C57BL/6 Mice.

Sporadic occurrence of congenital portosystemic shunt (PSS) at a rate of ∼1 out of 10 among C57BL/6 J mice, which are widely used in biomedical research, results in aberrancies in serologic, metabolic, and physiologic parameters. Therefore, mice with PSS should be identified as outliers in research. Accordingly, we sought methods to, reliably and efficiently, identify PSS mice. Serum total bile acids ≥ 40 µm is a bona fide biomarker of PSS in mice but utility of this biomarker is limited by its cost and invasiveness, particularly if large numbers of mice are to be screened. This led us to investigate if assay of urine might serve as a simple, inexpensive, noninvasive means of PSS diagnosis. Metabolome profiling uncovered that Krebs cycle intermediates, that is, citrate, α-ketoglutarate, and fumarate, were strikingly and distinctly elevated in the urine of PSS mice. We leveraged the iron-chelating and pH-lowering properties of such metabolites as the basis for 3 urine-based PSS screening tests: urinary iron-chelation assay, pH strip test, and phenol red assay. Our findings demonstrate the feasibility of using these colorimetric assays, whereby their readout can be assessed by direct observation, to diagnose PSS in an inexpensive, rapid, and noninvasive manner. Application of our urinary PSS screening protocols can aid biomedical research by enabling stratification of PSS mice, which, at present, likely confound numerous ongoing studies.

Assessment of portal system hemodynamics for the prediction of portal vein thrombosis in cirrhosis-A systematic review and meta-analysis.

BACKGROUND: The pathogenesis of portal vein thrombosis (PVT) in cirrhosis is multifactorial, with altered hemodynamics being proposed as a possible contributor. The present systematic review was conducted to study the role of assessment of portal hemodynamics for the prediction of PVT in patients with cirrhosis. METHODS: Three databases (Medline, Embase, and Scopus) were searched from inception to February 2023 for studies comparing portal venous system parameters in patients with cirrhosis developing PVT with those not. Results were presented as mean difference (MD) or odds ratio (OR) with their 95% confidence intervals (CIs). RESULTS: A total of 31 studies (patients with cirrhosis: 19 studies, patients with cirrhosis undergoing splenectomy: 12 studies) were included. On pooling the data from multivariable analyses of the included studies, a larger portal vein diameter was a significant predictor of PVT in patients with cirrhosis without or with splenectomy with OR 1.74 (1.12-2.69) and OR 1.55 (1.26-1.92), respectively. On the other hand, a lower portal vein velocity (PVV) was a significant predictor of PVT in cirrhotics without or with splenectomy with OR 0.93 (0.91-0.96) and OR 0.71 (0.61-0.83), respectively. A PVV of <15 cm/s was the most commonly used cut-off for the prediction of PVT. Patients developing PVT also had a significantly higher splenic length, thickness, and splenic vein velocity. CONCLUSION: The assessment of portal hemodynamic parameters at baseline evaluation in patients with cirrhosis may predict the development of PVT. Further studies are required to determine the optimal cut-offs for various parameters.

Intramural esophageal location of a portosystemic shunt in a dog with two congenital portosystemic shunts on CT angiography.

A 5-year-old male neutered pug with hematuria was presented to a referral hospital after identification of an extrahepatic portosystemic shunt (EHPSS) during abdominal ultrasonography. Computed tomographic-angiography revealed two anomalous blood vessels (left gastroazygous and left gastrophrenic). The left gastroazygous vessel followed an atypical path within the dorsolateral esophageal wall before entering the azygous vein. The morphology of this highly unusual vessel has not, based on the authors' review of the literature, been previously reported. In combination with a second anomalous vessel, this resulted in a unique presentation of an EHPSS. Computed tomography-angiography was essential for diagnosis and surgical planning in this case.