Congenital Portosystemic Shunts In Dogs Research Articles

Prospective Study Comparing Outcome following Complete Polypropylene Suture Ligation versus Partial Thin Film Band Attenuation of Congenital Portosystemic Shunts in Dogs.

The main objective was to conduct a prospective study reporting the outcome for dogs with an extrahepatic congenital portosystemic shunt (CPSS) treated with a 'complete ligation where possible' philosophy. The second aim was to compare the outcomes following complete (C) polypropylene suture ligation versus partial thin film band (TFB) attenuation of a CPSS in dogs. Dogs that could not tolerate acute complete shunt ligation at surgery received partial shunt attenuation with TFB. Peri-operative complications, mortality, follow-up imaging findings, pre- and post-operative bile acid stimulation test results and details of any revision surgery performed were recorded. A follow-up health-related quality of life questionnaire enabled the calculation of a postoperative clinical shunt score, a quality of life score, and determined if any dogs were still on a hepatic diet and/or other medical management at a minimum of 6 months after surgery. Of the 110 dogs enrolled, 57 received complete ligation and 53 received partial TFB attenuation. Peri-operative mortality, the occurrence of post-attenuation neurological complications, the occurrence of multiple acquired shunts, the postoperative clinical shunt score and quality of life score were not significantly different between the two groups. Dogs in the C group were older, heavier and demonstrated a greater number of shunt classifications where the entry into the systemic circulation was the phrenic vein or azygous vein. Dogs in the TFB group had a greater number of unchanged bile acid concentrations after surgery, were more likely to remain on the hepatic diet and/or medical management after surgery and underwent a greater number of revision surgeries. There was variability in the precision of both ultrasound and computed tomographic angiography follow-up imaging compared to intra-operative mesenteric portovenography findings at revision surgery. Overall, dogs with an extrahepatic portosystemic shunt receiving either complete acute shunt ligation or partial TFB shunt attenuation are expected to have an excellent long-term clinical outcome and there is no reason to suggest that a dog able to tolerate complete acute shunt closure should be denied the benefit of this.

Congenital Portosystemic Shunts in Dogs and Cats: Treatment, Complications and Prognosis.

Congenital portosystemic shunts (CPSS) are a common vascular anomaly of the liver in dogs and cats. Clinical signs of CPSS are non-specific and may wax and wane, while laboratory findings can raise the clinical suspicion for CPSS, but they are also not specific. Definitive diagnosis will be established by evaluation of liver function tests and diagnostic imaging. The aim of this article is to review the management, both medical and surgical, complications, and prognosis of CPSS in dogs and cats. Attenuation of the CPSS is the treatment of choice and may be performed by open surgical intervention using ameroid ring constrictors, thin film banding, and partial or complete suture ligation or by percutaneous transvenous coil embolization. There is no strong evidence to recommend one surgical technique over another. Medical treatment strategies include administration of non-absorbable disaccharides (i.e., lactulose), antibiotics, and dietary changes, and are indicated for pre-surgical stabilization or when surgical intervention is not feasible. After CPSS attenuation, short- and long-term post-surgical complications may be seen, such as post-operative seizures and recurrence of clinical signs, respectively. Prognosis after surgical attenuation of CPSS is generally favorable for dogs and fair for cats.

Congenital Portosystemic Shunts in Dogs and Cats: Classification, Pathophysiology, Clinical Presentation and Diagnosis.

Congenital portosystemic shunts (CPSS) are abnormal vascular communications between the portal and the systemic circulation, bypassing the hepatic parenchyma and resulting in liver hypoplasia and hepatic insufficiency. Such connections develop in utero and persist postnatally. CPSS are among the two most common congenital vascular anomalies of the liver in small animals, along with primary hypoplasia of the portal vein without portal hypertension (PHPV without PH). CPSS can be extrahepatic (ECPSS), most commonly diagnosed in small and toy breed dogs and cats, or intrahepatic (ICPSS), most commonly seen in large breed dogs. Single ECPSS is the most common type encountered in both dogs and cats. Clinical signs of CPSS are non-specific and may wax and wane, while laboratory findings can raise clinical suspicion for CPSS, but they are also not specific. Definitive diagnosis will be established by evaluation of liver function tests, such as determination of fasting plasma ammonia (FA) levels, and pre- and postprandial serum bile acids concentrations, and diagnostic imaging. The purpose of this article is to review the definition, classification, pathogenesis, clinical presentation, and diagnosis of CPSS in dogs and cats, highlighted by the authors' clinical experience.

Morphometric and cytological disturbances of pancreatic islets evoked by congenital portosystemic shunt in dogs.

In 46 dogs with congenital portosystemic shunt (cPSS) histopathological examination of the pancreas, including measuring of the islet area, was performed, and the results were compared with those obtained in 6 control dogs without cPSS. Pancreatic islets were found in 43 (94%) dogs with cPSS and in all control animals. Mean area of the pancreatic islets was significantly lower in cPSS patients (median of 2219.4 μm2, interquartile range [IQR] from 1559.0 to 3146.2 μm2, range from 485.4 to 10333.4 μm2) than in control dogs (median of 8705.5 μm2, IQR from 8284.4 to 9329.2 μm2, range from 7689.9 to 9624.2 μm2) (p⟨0.001). The area of pancreatic islets was weakly, but significantly, positively correlated with the body weight of dogs (r=0.32, p=0.026), but not with the age or sex. Vacuoles were found in the cytoplasm of pancreatic islet cells in 37 (87%) dogs with cPSS and in none of the control animals (p⟨0.001). Their presence was not linked to the sex, breed, age or body weight. Extracellular homogenous eosinophilic masses were present in pancreatic islets in 5 (12%) cPSS patients and in none of control animals. Connective tissue hyperplasia was found in pancreatic islets of 4 (9%) dogs with cPSS and in none of the control dogs. These results indicate that cPSS severely affects the pancreas, as shown by significantly reduced area of the islets, and the presence of eosinophilic masses in the pancreas and/or intracellular vacuoles.

Postattenuation neurologic signs after surgical attenuation of congenital portosystemic shunts in dogs: A review

The development of postattenuation neurologic signs (PANS) is a poorly understood and potentially devastating complication after surgical attenuation of congenital portosystemic shunts in dogs. Postattenuation neurologic signs include seizures but also more subtle neurologic signs such as depression, behavioral changes, tremors, and twitching. They most commonly occur within 7 days postoperatively and are typically unrelated to hyperammonemia, hypoglycemia, or electrolyte disturbances. This narrative review summarizes the findings of 50 publications from 1988-2020 that report occurrence of PANS. While most published reports included only dogs affected by postattenuation seizures (PAS), others included dogs with any form of PANS. Overall, PANS (including PAS) affected 1.6%-27.3% of dogs, whereas incidence of PAS ranged from 0%-18.2%. The etiology of PANS remains unknown; however, several theories have been proposed. Risk factors include preoperative hepatic encephalopathy, increasing age, and possibly certain breeds and extrahepatic shunt morphology. There is increasing evidence that prophylactic antiepileptic drugs do not prevent PANS. Treatment is centered around controlling neurologic signs with antiepileptic drugs and providing supportive intensive care. The 30-day survival rate in studies that included a minimum of four dogs affected by PANS was 0%-100% (median, 50.0%) and 0%-75.0% (median, 37.5%) for those with PAS. Mortality associated with PANS was typically related to occurrence of generalized seizure activity. Prognostic factors positively associated with short-term survival included having a history of preoperative seizures and development of focal seizures only. If affected dogs survived to discharge, survival for several years was possible, and the majority of neurologic signs manifested as part of the phenomenon of PANS appeared to resolve.

Outcome of non-surgical dietary treatment with or without lactulose in dogs with congenital portosystemic shunts

Background Congenital portosystemic shunts (CPSS) are vascular anomalies, allowing portal blood to bypass the hepatic parenchyma, thereby accumulating toxic substances such as ammonia in the systemic circulation resulting in hepatic encephalopathy. Aim To evaluate the outcome of non-surgically treated dogs with a CPSS. Methods Case records of 78 dogs with a single congenital CPSS confirmed by ultrasound and/or computed tomography between September 2003 and February 2015 were reviewed. Median age at diagnosis of CPSS in dogs was 10.8 months (range 2–133 months). Non-surgical treatment was started as an adjusted diet (a diet restricted in protein) with or without lactulose. Owners were contacted by telephone to determine survival time and presumed cause of death, if applicable. In addition, a questionnaire was used to retrospectively assess quality of life (QoL) and CPSS scores in 37 dogs before and during non-surgical treatment. Differences between Kaplan–Meier curves were tested by a Log rank test. Results Overall estimated median survival time (EMST) was 38.5 months (range 1 day − 91 months; 78 dogs). No significant differences between EMSTs were found between dogs with extra- (n = 48) or intrahepatic (n = 29) shunts, nor between treatment with only an adjusted diet, or an adjusted diet combined with lactulose. During non-surgical treatment, significant improvement in perceived QoL and CPSS scores were found (P < 0.01). Conclusion Our study demonstrated that an overall median EMST of 3.2 years was reached and that owners retrospectively perceived that non-surgical treatment resulted in an improved QoL and clinical performance, irrespective of intrahepatic or extrahepatic CPSS location.

Congenital portosystemic shunt in dogs: review

Portosystemic shunt is a condition commonly seen in dogs. This disease is a vascular anomaly that makes communication between the systemic circulation and the portal circulation. Because of this anomalous vascular communication, portal blood from the drainage of organs such as the stomach, intestine, pancreas and spleen, passes directly into the systemic circulation, diverting from the liver that is responsible for detoxification of various substances. Therefore, the patient will manifest numerous clinical signs with possible neurological or systemic involvement only due to the accumulation of toxins in the blood. The treatment of choice for the portosystemic shunt is the ligation of the anomalous vessel, to which the ameroid constricting ring will be the most approached in this article.

Intrahepatic congenital portosystemic shunts in dogs: short- and long-term outcome of suture attenuation.

To report the short- and long-term outcomes of one- or two-staged suture attenuation for complete closure of intrahepatic congenital portosystemic shunts in dogs. Retrospective cohort study of dogs surgically treated for intrahepatic congenital portosystemic shunts between February 2000 and March 2015. Long-term follow-up was conducted by telephone conversations with the referring veterinary surgeon, owner, or both. In total, 55 dogs had suture attenuation of their intrahepatic congenital portosystemic shunt; 10 dogs (18·2%) tolerated complete attenuation, whilst 45 dogs (81·8%) tolerated partial attenuation. Postoperative complications occurred in 24 dogs (43·6%), and six dogs (10·9%) died. Repeat surgery was performed in 33 of 39 dogs (84·6%) that had previously undergone partial attenuation, and 27 of these (84·9%) ultimately achieved complete shunt attenuation. One dog (3·0%) died following second surgery, resulting in an overall postoperative mortality of seven of 55 (12·7%). Detailed follow-up was available for 22 dogs that were still alive at a median of 29 months after surgery (7·4 to 103·1) with a subjectively good quality of life. Of 17 dogs (82·4%), 14 with complete attenuation in one or two surgeries had an excellent outcome compared with one of five dogs (20%) with persistent shunting. Staged suture ligation resulted in a high proportion of complete attenuation and reduced persistent shunting compared with a single surgery. Repeat surgery was associated with fewer complications than the first surgery. The proportion of dogs with an excellent outcome was greater for those that had complete attenuation in one or two surgeries compared with those with persistent shunting.

Treatment of intrahepatic congenital portosystemic shunts in dogs: a systematic review.

The aim of this study was to establish the evidence base for the treatment of intrahepatic congenital portosystemic shunts in dogs through a systematic review of the pertinent literature. Studies were filtered for evidence to answer the question "Which of the treatment options for intrahepatic CPSS in dogs offers the best short- and long-term outcome?" Studies were assigned a level of evidence based on a system published by the Oxford Centre for Evidence-Based Medicine. Thirty-two studies were included in the review. Twenty-six provided level 4 evidence and six provided level 5 evidence. There were no level 1, 2 or 3 studies. One study compared surgical treatment with medical management and one study compared suture ligation with ameroid constrictor placement. The remaining studies were case series describing the outcome for one treatment method alone. Methods and timings of assessments of short- and long-term outcomes were highly varied, making direct comparisons challenging. The evidence regarding the treatment of intrahepatic congenital portosystemic shunts in dogs is weak, with only two studies directly comparing treatments. There is a lack of evidence regarding short- and long-term outcomes on which to base clinical decisions.

Morphology of splenocaval congenital portosystemic shunts in dogs and cats.

To describe the anatomy of congenital portosystemic shunts involving the splenic vein communicating with the caudal vena cava at the level of the epiploic foramen. A retrospective review of a consecutive series of dogs and cats managed for congenital portosystemic shunts. Ninety-eight dogs and eight cats met the inclusion criteria of a congenital portosystemic shunt involving the splenic vein communicating with the prehepatic caudal vena cava plus recorded intra-operative mesenteric portovenography or computed tomography angiography and gross observations at surgery. All cases (both dogs and cats) had a highly consistent shunt that involved a distended gastrosplenic vein that communicated with the caudal vena cava at the level of the epiploic foramen via an anomalous left gastric vein. The morphology of the shunt type described appeared to be a result of an abnormal communication between the left gastric vein and the caudal vena cava and the subsequent development of preferential blood flow through an essentially normal portal venous system. The abnormal communication (shunt) was through the left gastric vein and not the splenic vein, as might have been expected. This information may help with surgical planning in cases undergoing shunt closure surgery.

Surgical attenuation of spontaneous congenital portosystemic shunts in dogs resolves hepatic encephalopathy but not hypermanganesemia.

Hypermanganesemia is commonly recognized in human patients with hepatic insufficiency and portosystemic shunting. Since manganese is neurotoxic, increases in brain manganese concentrations have been implicated in the development of hepatic encephalopathy although a direct causative role has yet to be demonstrated. Evaluate manganese concentrations in dogs with a naturally occurring congenital shunt before and after attenuation as well as longitudinally following the changes in hepatic encephalopathy grade. Our study demonstrated that attenuation of the shunt resolved encephalopathy, significantly reduced postprandial bile acids, yet a hypermanganasemic state persisted. This study demonstrates that resolution of hepatic encephalopathy can occur without the correction of hypermanganesemia, indicating that increased manganese concentrations alone do not play a causative role in encephalopathy. Our study further demonstrates the value of the canine congenital portosystemic shunt as a naturally occurring spontaneous model of human hepatic encephalopathy.

Attenuation of congenital portosystemic shunt reduces inflammation in dogs.

Liver disease is a major cause of morbidity and mortality. One of the most significant complications in patients with liver disease is the development of neurological disturbances, termed hepatic encephalopathy. The pathogenesis of hepatic encephalopathy is incompletely understood, which has resulted in the development of a wide range of experimental models. Congenital portosystemic shunt is one of the most common congenital disorders diagnosed in client owned dogs. Our recent studies have demonstrated that the pathophysiology of canine hepatic encephalopathy is very similar to human hepatic encephalopathy, which provides strong support for the use of dogs with a congenital portosystemic shunt as a naturally occurring model of human hepatic encephalopathy. Specifically, we have demonstrated an important role for ammonia and inflammation in the development of hepatic encephalopathy in dogs with a congenital portosystemic shunt. Despite the apparent importance of inflammation in driving hepatic encephalopathy in dogs, it is unclear whether inflammation resolves following the successful treatment of liver disease. We hypothesized that haematological and biochemical evidence of inflammation, as gauged by neutrophil, lymphocyte and monocyte concentrations together with C-reactive protein concentrations, would decrease following successful treatment of congenital portosystemic shunts in dogs. One hundred and forty dogs with a congenital portosystemic shunt were enrolled into the study. We found that the proportion of dogs with a monocyte concentration above the reference range was significantly greater in dogs with hepatic encephalopathy at time of initial diagnosis. Importantly, neutrophil and monocyte concentrations significantly decreased following surgical congenital portosystemic shunt attenuation. We also found a significant decrease in C-reactive protein concentrations following surgical attenuation of congenital portosystemic shunts. Our study demonstrates that haematological and biochemical indices of inflammation reduce following successful treatment of the underlying liver disorder.

Long-term survival and quality of life in dogs with clinical signs associated with a congenital portosystemic shunt after surgical or medical treatment.

To compare long-term survival and quality of life data in dogs with clinical signs associated with a congenital portosystemic shunt (CPSS) that underwent medical or surgical treatment. Prospective cohort study. 124 client-owned dogs with CPSS. Dogs received medical or surgical treatment without regard to signalment, clinical signs, or clinicopathologic results. Survival data were analyzed with a Cox regression model. Quality of life information, obtained from owner questionnaires, included frequency of CPSS-associated clinical signs (from which a clinical score was derived), whether owners considered their dog normal, and (for surgically treated dogs) any ongoing medical treatment for CPSS. A Mann-Whitney U test was used to compare mean clinical score data between surgically and medically managed dogs during predetermined follow-up intervals. 97 dogs underwent surgical treatment; 27 were managed medically. Median follow-up time for all dogs was 1,936 days. Forty-five dogs (24 medically managed and 21 surgically managed) died or were euthanized during the follow-up period. Survival rate was significantly improved in dogs that underwent surgical treatment (hazard ratio, 8.11; 95% CI, 4.20 to 15.66) than in those treated medically for CPSS. Neither age at diagnosis nor shunt type affected survival rate. Frequency of clinical signs was lower in surgically versus medically managed dogs for all follow-up intervals, with a significant difference between groups at 4 to 7 years after study entry. Surgical treatment of CPSS in dogs resulted in significantly improved survival rate and lower frequency of ongoing clinical signs, compared with medical management. Age at diagnosis did not affect survival rate and should not influence treatment choice.

Markers of angiogenesis associated with surgical attenuation of congenital portosystemic shunts in dogs.

BackgroundDogs with congenital portosystemic shunts (CPSS) have hypoplasia of the intrahepatic portal veins. Surgical CPSS attenuation results in the development of the intrahepatic portal vasculature, the precise mechanism for which is unknown, although new vessel formation by angiogenesis is suspected.HypothesisThat the degree of portal vascular development and the increase in portal vascularization after CPSS attenuation is significantly associated with hepatic vascular endothelial growth factor (VEGF) and VEGF receptor 2 (VEGFR2) gene expression and serum VEGF concentration.AnimalsClient‐owned dogs with CPSS undergoing surgical treatment. Forty‐nine dogs were included in the gene expression data and 35 in the serum VEGF data.Materials and MethodsDogs surgically treated by partial or complete CPSS attenuation were prospectively recruited. Relative gene expression of VEGF and VEGFR2 was measured in liver biopsy samples taken at initial and follow‐up surgery using quantitative polymerase chain reaction. Serum VEGF concentration was measured before and after CPSS attenuation using a canine specific ELISA. Statistical significance was set at the 5% level (P ≤ .05).ResultsThere was a significant increase in the mRNA expression of VEGFR2 after partial attenuation (P = .006). Dogs that could tolerate complete attenuation had significantly greater VEGFR2 mRNA expression than those that only tolerated partial attenuation (P = .037). Serum VEGF concentration was significantly increased at 24 (P < .001) and 48 (P = .003) hours after attenuation.Conclusions and Clinical ImportanceThese findings suggest that intrahepatic angiogenesis is likely to occur after the surgical attenuation of CPSS in dogs, and contributes to the development of the intrahepatic vasculature postoperatively.

Markers of hepatic regeneration associated with surgical attenuation of congenital portosystemic shunts in dogs

Dogs with congenital portosystemic shunts (CPSS) have liver hypoplasia and hepatic insufficiency. Surgical CPSS attenuation results in liver growth associated with clinical improvement. The mechanism of this hepatic response is unknown, although liver regeneration is suspected. This study investigated whether markers of liver regeneration were associated with CPSS attenuation. Dogs treated with CPSS attenuation were prospectively recruited. Residual liver tissue was collected for gene expression analysis (seven genes) from 24 CPSS dogs that tolerated complete attenuation, 25 dogs that tolerated partial attenuation and seven control dogs. Relative gene expression was measured using quantitative polymerase chain reaction (qPCR). Blood samples were collected before, 24 h and 48 h post-surgery from 36 CPSS dogs and from 10 control dogs. Serum hepatocyte growth factor (HGF) concentration was measured using a canine specific enzyme-linked immunosorbent assay (ELISA). HGF mRNA expression was significantly decreased in CPSS compared with control dogs (P = 0.046). There were significant increases in HGF (P = 0.050) and methionine adenosyltransferase 2 A (MAT2A; P = 0.002) mRNA expression following partial CPSS attenuation. Dogs with complete attenuation had significantly greater MAT2A (P = 0.024) mRNA expression compared with dogs with partial attenuation. Serum HGF concentration significantly increased 24 h following CPSS attenuation (P < 0.001). Hepatic mRNA expression of two markers of hepatocyte proliferation (HGF and MAT2A) was associated with the response to surgery in dogs with CPSS, and serum HGF significantly increased following surgery, suggesting hepatocyte proliferation. These findings support the concept that hepatic regeneration is important in the hepatic response to CPSS surgery.

More answers needed on congenital portosystemic shunts in dogs and cats

Veterinary RecordVolume 172, Issue 14 p. 360-361 Research More answers needed on congenital portosystemic shunts in dogs and cats Adrian W. Philbey BVSc, PhD, MANZCVSc, MRCVS, Corresponding Author Adrian W....

Treatment of extrahepatic congenital portosystemic shunts in dogs – what is the evidence base?

A variety of surgical treatments and medical therapies are recommended for dogs with extrahepatic congenital portosystemic shunts (CPSS). The objective of this review was to assess the evidence base for the management of extrahepatic CPSS in dogs. An online bibliographic search was performed in November 2010 to identify articles relating to the question "Which of the treatment options for extrahepatic CPSS in dogs offers the best short- and long-term outcomes?" Articles were assigned a level of evidence based on a modified grading system. Thirty-eight articles were included in the review. Thirty-six articles were classified as grade 4 and two as grade 5. The timings and methods of assessment of short- and long-term outcomes varied widely between studies. One prospective study (grade 4a) showed that surgically treated dogs survived significantly longer than medically treated dogs. Four retrospective studies (grade 4b) compared the outcome of two surgical techniques but there were no statistically significant differences between treatment groups in terms of complications or outcome. The review found that the evidence base for the treatment of extrahepatic CPSS is weak. There is a lack of evidence of short- and long-term outcomes to recommend one treatment over another.

Use of aDacron Shape‐Memory Intravascular Coil to Achieve Slow, Progressive Occlusion of the Jugular Vein in Dogs

To investigate the ability of shape memory Dacron polymer vascular coils to induce the complete, gradual occlusion of the canine jugular vein. Observational pilot study. Nine purpose-bred dogs. Eighteen coils were deployed in nine dogs using fluoroscopic-guided percutaneous transvenous coil implantation. Individual coil formulations varied around a Dacron polymer base. Jugular vein diameter, percent vessel occlusion, and thrombus echogenicity were monitored at weekly intervals using ultrasonography. Affected jugular veins were harvested at 6 weeks post-implantation and histopathological analysis was performed to assess adventitial fibrosis, intimal layer thickening, and inflammation. Ten coils migrated from the jugular veins to the pulmonary vasculature within 0-2 weeks following implantation. Three jugular veins achieved at least 90% occlusion at six weeks. Histopathology of these jugular veins revealed marked perivascular thickening and fibrovascular proliferation, increased infiltration of macrophages, neutrophils and lymphocytes, and abundant fibroplasia. Complete, gradual occlusion of a jugular vein was achieved in three dogs. Significant vessel wall reaction and inflammation can induce gradual vessel occlusion when a Dacron coil remains implanted within the jugular vein. Dacron polymer coils could be a feasible treatment option for the gradual occlusion of congenital portosystemic shunts in dogs using minimally invasive, percutaneous transvenous implantation.

Hepatic gene expression and plasma albumin concentration related to outcome after attenuation of a congenital portosystemic shunt in dogs

In dogs with a congenital portosystemic shunt (CPSS), the outcome after CPSS attenuation is difficult to predict but is most likely related to hepatic and vascular proliferation that follows the attenuation. The aim of this study was to evaluate the prognostic value of shunt localization (extrahepatic vs. intrahepatic), plasma albumin concentration and hepatic mRNA expression of 19 genes involved in hepatic and vascular growth. The study population consisted of 48 dogs that were referred for surgical ligation of a single intrahepatic or extrahepatic CPSS. Gene expression was measured in intraoperatively sampled hepatic tissue with quantitative real-time PCR. Albumin, methionine adenosyltransferase 2α (MAT2α) and HGF activator (HGFac) were positively associated with complete recovery after CPSS attenuation using multivariate statistical analyses. Individual outcome could be correctly predicted in 83% of dogs using albumin, MAT2α and HGFac as high or low values compared to cut-off values of 19.5 g/L, 0.457 and 0.974, respectively. These variables predicted outcome after CPSS ligation better than shunt localization or albumin alone. Other evaluated gene products were not correlated with outcome.

Evidence of Inheritance of Intrahepatic Portosystemic Shunts in Irish Wolfhounds

The etiogenesis of congenital portosystemic shunt in dogs is not understood. In Irish Wolfhounds, intrahepatic portosystemic shunt (IHPSS) is thought to be hereditary, but the mode of inheritance is unknown. To document the genetic background and investigate the potential mode of inheritance of IHPSS in Irish Wolfhounds. Three mature, privately owned, affected siblings and their progeny produced in 2 litters. Prospective, observational study. Two test matings of 1 affected sire with 2 of his affected sisters were used to determine the inheritance pattern. Affection status was determined by measuring venous blood ammonia concentrations, detection of the shunt by ultrasonography and confirmation during surgical attenuation of the intrahepatic shunting vessel. In 1 litter of 5 pups all had an IHPSS. In the other litter 5 of 11 pups were affected. Both left- and right-sided shunts occurred in both litters. No sex predisposition was evident among affected dogs. Our results show that IHPSS in Irish Wolfhounds is a familial disorder that is likely genetic. It is unlikely that the mode of inheritance is monogenic. A digenic, triallelic trait could explain the observed occurrence of IHPSS but other modes of inheritance cannot be excluded.