Partial anomalous pulmonary venous connection with portosystemic shunt in a cat

A nine-month-old castrated male domestic shorthair presented for evaluation with a three-month history of hematuria. Portosystemic shunts and calculi within the bladder were suspected, and computed tomography angiography was performed. Computed tomography angiography identified an extrahepatic portosystemic shunt and a partial anomalous pulmonary venous connection, with the lobar vein of the right caudal lobe draining into the caudal vena cava. After anesthesia was administered to the cat, tachypnea and wheezing respiratory sounds were observed, and thoracic radiography revealed the right middle lung lobe atelectasis and an unstructured interstitial pattern in the left cranial lobe. Echocardiography showed left and right atrial enlargement and slight interventricular septal flattening in diastole. Based on these findings, cardiogenic pulmonary edema was suspected, and the cat was treated with furosemide. The clinical symptoms were resolved the next day. Closure of the extrahepatic portosystemic shunt was performed on days 47 and 157. Left atrial enlargement and interventricular septal flattening were attenuated after the procedure. At the time of writing this report (seventeen months after diagnosis), the cat exhibited no clinical signs, but subjective right atrial enlargement remained at approximately the same level. This report represents the first case of a partial anomalous pulmonary venous connection and a portosystemic shunt in a cat.     

SIMULTANEOUS CONGENITAL AND ACQUIRED EXTRAHEPATIC PORTOSYSTEMIC SHUNTS IN TWO DOGS

Two dogs with simultaneous congenital and acquired portosystemic shunts are reported. The first dog was an eight-month-old, male Golden Retriever with a history of peritoneal effusion, polyuria/polydipsia, and stunted growth. The dog had a microcytic, hypochromic anemia, a mildly elevated AST, and a moderate to severely elevated preprandial and postprandial serum bile acids. Transcolonic portal scintigraphy confirmed the presence of a portosystemic shunt. An intraoperative mesenteric portogram was performed. Two conjoined congenital extrahepatic portosystemic shunts and multiple acquired extrahepatic portosystemic shunts were identified. The second dog was a five-month-old, mixed breed with two week history of peritoneal effusion. Abdominal ultrasound and transcolonic scintigraphy were used to diagnose a portosystemic shunt. A single extrahepatic portosystemic shunt, portal hypertension, and multiple acquired collateral shunts were identified at surgery. The histologic alterations observed in these dogs were consistent with a portosystemic shunt. In these dogs, the presence of congenital and acquired portosystemic shunts and histopathologic findings are considered to represent a combination of congenital portosystemic shunts and noncirrhotic portal hypertension or portal vein hypoplasia.     

Surgical Management of Multiple Congenital Intrahepatic Shunts in Two Dogs

Objective —To present details of an unusual type of portosystemic shunt and its surgical management in two dogs.
Animals —Two young dogs that had a tentative diagnosis of a portosystemic shunt on the basis of clinical signs and serum biochemical abnormalities. Abdominal ultrasonography and contrast portography demonstrated multiple intrahepatic shunts. In both cases, the multiple shunts arose from a single branch of the portal vein.
Outcome—It was possible to locate and attenuate flow through the shunts via a transportal venotomy under conditions of hepatic vascular occlusion. Clinical and biochemical abnormalities resolved after surgery in both dogs. Postoperative sonography revealed complete obliteration of the shunt plexus in one of the dogs.     

Portosystemic shunts in cats: seven cases (1976-1984)

Congenital portosystemic venous shunt causing signs of hepatic encephalopathy was diagnosed in 7 cats. The left gastric vein served as the shunt in four of these. Increases in blood ammonia and postprandial serum bile acids were the most consistent serum biochemical abnormalities. Excessive variation in red blood cell shape was a common but nonspecific hematologic finding. The jejunal-mesenteric venous injection of contrast material was the preferred method of portography to diagnose portosystemic shunts. Two cats were treated successfully by partial surgical occlusion of their shunts.     

Serum hyaluronic acid in dogs with congenital portosystemic shunts

Objectives : To compare the serum level of hyaluronic acid in dogs with congenital portosystemic shunt with that in healthy dogs and to investigate the perioperative change in serum hyaluronic acid following shunt attenuation. Methods : Blood samples were obtained from 29 congenital portosystemic shunt dogs before the operation, and 2 and 4 weeks after the operation from 17 and 7 dogs, respectively. The serum hyaluronic acid level of these dogs was measured and compared with that of 10 healthy beagles. Results : The median preoperative hyaluronic acid level in dogs with congenital portosystemic shunt was significantly elevated compared with that in healthy dogs. Furthermore, the median postoperative hyaluronic acid level significantly decreased compared with the median preoperative levels in congenital portosystemic shunt dogs. Clinical Significance : In the case of dogs with congenital portosystemic shunt, the reduction of intrahepatic portal blood flow might lower the clearance rate of hyaluronic acid in hepatic sinusoidal endothelial cells, so hyaluronic acid clearance could be improved by attenuation of a shunt vessel. Hence, serum hyaluronic acid levels might be useful to evaluate liver function and also have the potential to evaluate successful attenuation of a shunt vessel in dogs with congenital portosystemic shunt. Further investigations are required to clarify whether serum hyaluronic acid offers significant benefits over existing markers such as serum bile acid or ammonia concentrations.     

Intrahepatic portosystemic venous shunt in a neonatal roan antelope (Hippotragus equinus cottoni).

A 6-day-old, female roan antelope (Hippotragus equinus cottoni) was diagnosed with a single intrahepatic portosystemic venous shunt at necropsy. Clinical signs had included weakness, lethargy, hypothermia, diarrhea, and a weak suckle response. Multiple seizure episodes were associated with hypoglycemia and characterized by vocalization, muscle fasciculations, and disorientation. Hematologic abnormalities included anemia with hypochromasia, anisocytosis, poikilocytosis, and leukopenia with neutropenia and lymphopenia. Serum biochemical abnormalities included elevations in blood urea nitrogen and total serum bile acid concentration. A portosystemic vascular anomaly should be a differential diagnosis for nonthriving, exotic ruminant calves with overt or subtle neurologic signs, persistent hypoglycemia, and/or elevated bile acids. In very young calves, total bile acid concentration may be more useful in establishing a diagnosis than blood ammonia concentration.     

Portosystemic shunt in an alpaca cria

A 5-month-old alpaca cria was examined for chronic poor growth and repeated episodes of diarrhea. Examination of feces for parasites yielded negative results. Serum bile acid and blood ammonia concentrations were high. Subsequent examination by ultrasonography, percutaneous splenic portography, and colonic scintigraphy did not reveal evidence of a portosystemic vascular anomaly. Exploratory celiotomy with mesenteric vein portography revealed a colonic vein shunt in the caudal portion of the abdomen from the caudal vena cava to the portal vein. The shunt vessel was ligated without incident. Following surgery, the cria began to gain weight and was more alert. Eighteen months after surgery, the cria was doing well, although it had loose feces and was slightly small for its age. Portosystemic shunts are rare in cattle and horses but should be considered in alpacas with chronic poor growth when parasitism has been ruled out.     

Use of transcolonic portal scintigraphy to evaluate efficacy of cellophane banding of congenital extrahepatic portosystemic shunts in 16 dogs

OBJECTIVE: To evaluate the efficacy of cellophane banding of single congenital extrahepatic portosystemic shunts in dogs using transcolonic portal scintigraphy. To investigate the portal circulation of those dogs with elevated postoperative shunt fractions to determine the cause of the persistent shunting. Further, to evaluate whether presenting signs, clinical pathology findings and liver histopathology are predictive of outcome. DESIGN: Prospective study of 16 dogs presenting with single congenital extrahepatic portosystemic shunts. PROCEDURE: Dogs with single extrahepatic portosystemic shunts attenuated by cellophane banding underwent portal scintigraphy and bile acids tolerance testing pre- and post-operatively. Dogs identified with elevated shunt fractions at 10 weeks post-operatively underwent mesenteric portovenography. Qualitative hepatic histopathology from all dogs was reviewed by a veterinary pathologist and assigned a semi-quantitative score to identify any abnormalities that may predict surgical outcome. RESULTS: At 10 weeks post cellophane banding, 10 of 16 cases (63%) had normal shunt fractions, whilst six dogs (37%) had increased shunt fractions and seven dogs (44%) had increased serum bile acids. Of these dogs, mesenteric portovenography revealed incomplete closure of the shunt in three dogs (18.6%) and multiple acquired shunts in three dogs (18.6%). Liver histopathology findings were similar for all dogs, regardless of outcome. CONCLUSIONS: Cellophane banding is an efficacious method for complete gradual occlusion of single extrahepatic shunts when the shunt vessel is attenuated to < or = 3 mm. Transcolonic portal scintigraphy is a reliable method for assessment of shunt attenuation and, unlike serum bile acids, is not influenced by other causes of liver dysfunction.     

The pathophysiology and laboratory diagnosis of congenital portosystemic shunts in dogs

Congenital portosystemic shunts generally arise as single vascular anomalies that cause the portal blood to bypass the liver and enter the systemic venous circulation directly. The liver is primarily affected, as it is deprived of perfusion by portal hepatotrophic factors such as insulin, glucagon, and amino acids. There is progressive hepatic atrophy, and as a consequence, dysfunction.

Hepatic encephalopathy can result from increased levels of ammonia and gamma-aminobutyric acid within the systemic circulation. Variably toxic amines, captans and short chain fatty acids may act as false neurotransmitters. Hypoglycaemia will exacerbate the effects of these substances. Increased concentrations of ammonia and uric acid in the urine predispose to the precipitation of ammonium biurate crystals and the formation of calculi.

Haematological changes include anaemia, microcytosis, hypoproteinaemia, leucocytosis, and coagulation abnormalities. Gastrointestinal effects are common. They may be displayed as anorexia, vomiting, ptyalism, pica, diarrhoea, or polyphagia. Most dogs are less than 1 year of age at initial presentation.

Diagnosis from a laboratory viewpoint will involve a consideration of the history, clinical findings, haematology, serum biochemistry and urinalysis. If the findings are suggestive of a congenital portosystemic shunt, the demonstration of elevated fasting or, more consistently, post-prandial serum bile acid concentrations, and subsequent histological examination of a liver biopsy will provide a definitive diagnosis.     

The pathophysiology and laboratory diagnosis of congenital portosystemic shunts in dogs

Abstract Congenital portosystemic shunts generally arise as single vascular anomalies that cause the portal blood to bypass the liver and enter the systemic venous circulation directly. The liver is primarily affected, as it is deprived of perfusion by portal hepatotrophic factors such as insulin, glucagon, and amino acids. There is progressive hepatic atrophy, and as a consequence, dysfunction. Hepatic encephalopathy can result from increased levels of ammonia and gamma-aminobutyric acid within the systemic circulation. Variably toxic amines, captans and short chain fatty acids may act as false neurotransmitters. Hypoglycaemia will exacerbate the effects of these substances. Increased concentrations of ammonia and uric acid in the urine predispose to the precipitation of ammonium biurate crystals and the formation of calculi. Haematological changes include anaemia, microcytosis, hypoproteinaemia, leucocytosis, and coagulation abnormalities. Gastrointestinal effects are common. They may be displayed as anorexia, vomiting, ptyalism, pica, diarrhoea, or polyphagia. Most dogs are less than 1 year of age at initial presentation. Diagnosis from a laboratory viewpoint will involve a consideration of the history, clinical findings, haematology, serum biochemistry and urinalysis. If the findings are suggestive of a congenital portosystemic shunt, the demonstration of elevated fasting or, more consistently, post-prandial serum bile acid concentrations, and subsequent histological examination of a liver biopsy will provide a definitive diagnosis.     

Ultrasonographic diagnosis of congenital portosystemic shunt in 14 cats

Twenty-four cats with clinical and, or, clinico-pathological signs compatible with portosystemic shunting were examined prospectively using two-dimensional grey-scale, duplex and colourflow Doppler ultrasonography. Diagnosis of congenital portosystemic shunt was subsequently confirmed in 14 cats using operative mesenteric portography and surgery. Of the 14 affected cats, nine were purebred; eight were male and six female. The mean age at the time of diagnosis was nine months (range four to 27 months). Ultrasonographic evidence of a small liver was present in seven cats (50 per cent); visibility of intrahepatic portal vessels was reduced in three (21 per cent). An anomalous blood vessel was identified ultrasonographically in each cat; in 10 cats (71 per cent) the vessel was observed to originate from the portal vein and drain into the caudal vena cava. Abnormally variable portal blood flow waspresent in eight of the 10 cats in which it was measured. At surgery, six shunts were intrahepatic and eight extrahepatic; the ultrasonographic diagnosis of intra- versus extra-hepatic shunt was correct in 13 cats (93 per cent). No anomalous blood vessels or abnormalities affecting the portal vein were detected ultrasonographically in any of the 10 cats that did not have congenital portosystemic shunting. Hence, the accuracy of ultrasonography for diagnosis of congenital portosystemic shunting in this series was 100 per cent.     

Persistent cobalamin deficiency causing failure to thrive in a juvenile beagle

A six-month-old beagle was presented with a three-month history of failure to gain weight, lethargy, intermittent vomiting and seizures. Hypoglycaemia, portosystemic shunt, lead intoxication, gastrointestinal diseases and hereditary metabolic disorders were considered. Laboratory test results of low serum cobalamin (Cbl) concentrations, anaemia, leucopenia and methylmalonic aciduria while the dog was receiving a balanced commercial canine diet were suggestive of a congenital selective Cbl malabsorption. Treatment with repeated injections of parenteral cyanocobalamin (CN-Cbl) at 50 microg/kg every two weeks corrected the Cbl-deficient state and reversed all the clinical abnormalities. Selective Cbl malabsorption has previously been described in giant schnauzers and border collies and represents a unique readily treatable hereditary metabolic disorder.     

Ultrasonographic findings in dogs with hyperammonemia: 90 cases (2000-2002)

OBJECTIVE: To determine ultrasonographic abnormalities in dogs with hyperammonemia. DESIGN: Retrospective study. ANIMALS: 90 client-owned dogs with hyperammonemia. PROCEDURE: Ultrasonography of the abdominal vessels and organs was performed in a systematic way. Dogs in which the ultrasonographic diagnosis was a congenital portosystemic shunt were included only if they underwent laparotomy or necropsy. Dogs in which the abdominal vasculature appeared normal and dogs in which the ultrasonographic diagnosis was acquired portosystemic shunts and portal hypertension were included only if liver biopsy specimens were submitted for histologic examination. RESULTS: Ultrasonography excluded portosystemic shunting in 11 dogs. Acquired portosystemic shunts were found in 17 dogs, of which 3 had arterioportal fistulae and 14 had other hepatic abnormalities. Congenital portosystemic shunts were found in 61 dogs, of which 19 had intrahepatic shunts and 42 had extrahepatic shunts. Intrahepatic shunts originated from the left portal branch in 14 dogs and the right portal branch in 5. Extrahepatic shunts originated from the splenic vein, the right gastric vein, or both and entered the caudal vena cava or the thorax. Ultrasonography revealed splenic-caval shunts in 24 dogs, right gastric-caval shunts in 9 dogs, splenic-azygos shunts in 8 dogs, and a right gastric-azygos shunt in 1 dog. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that ultrasonography is a reliable diagnostic method to noninvasively characterize the underlying disease in dogs with hyperammonemia. A dilated left testicular or ovarian vein was a reliable indicator of acquired portosystemic shunts.     

Use of a percutaneous atrial septal occluder device for complete acute occlusion of an intrahepatic portosystemic shunt in a dog

A 3-month-old sexually intact male German Shepherd Dog was evaluated because of signs of depression, ataxia, and collapse. Clinicopathologic abnormalities included low serum BUN and albumin concentrations and high serum liver enzyme activities and plasma ammonia and serum bile acids concentrations. Abdominal ultrasonography revealed an intrahepatic portosystemic shunt (PSS). The dog was anesthetized; via a transjugular approach, guidewires and catheters were directed with fluoroscopic guidance to locate the shunt and determine its anatomic features. Minimal changes in portal vein pressure during temporary shunt balloon occlusion enabled complete shunt attenuation, which was performed by use of a self-expanding septal occlusion device that is typically used for treatment of atrial septal defects in humans. Following initial misplacement of the device, the procedure was repeated successfully 2 months later and resulted in complete shunt occlusion. One year after this second procedure, the dog was clinically normal and serum bile acids concentration was within reference limits. In certain dogs with intrahepatic PSSs, treatment with minimally invasive interventional techniques involving fluoroscopy may reduce the morbidity and mortality rates associated with more invasive surgical procedures.     

The Diagnosis and Surgical Correction of Congenital Portosystemic Vascular Anomalies in Two Calves and Two Foals

Two calves and two foals presented with episodic clinical signs of diffuse central nervous system disease. Portosystemic anomalies were tentatively diagnosed based on the history, clinical signs, and increased serum concentrations of blood ammonia and total serum bile acids with normal concentrations of liver derived enzymes. One calf died before intraoperative contrast portography, whereas the other calf and both foals had marked clinical improvement after intensive medical therapy. Surgical correction was attempted in these three animals and was successful in one foal. A right paracostal celiotomy was superior to a ventral median approach for exposure to the portal vascular system and shunt access.     

Outcomes after extrahepatic portosystemic shunt ligation in 49 dogs

OBJECTIVE: To evaluate outcomes after attenuation of extrahepatic portosystemic shunts in dogs using surgical silk. DESIGN: Retrospective study. PROCEDURE: Case records were reviewed for degree of surgical attenuation, experience of the primary surgeon, perioperative mortality and problems related to persistent portosystemic shunting or shunt ligation. Presence of portosystemic shunting after surgery was evaluated by ammonia tolerance testing, measurement of postprandial serum bile acid, plasma urea and cholesterol concentrations and liver enzyme activity. The influence of age, postocclusion portal pressure, primary surgeon, degree of attenuation and postoperative biochemical findings on the occurrence of postoperative problems was assessed. RESULTS: The mortality rate was 2.1%. Shunt attenuation was complete in 34% and partial in 66% of dogs. Portal hypertension necessitating ligature removal was encountered in only one dog. Five dogs experienced neurological abnormalities (seizures or ataxia), possibly as a manifestation of 'postligation seizure syndrome'. Postoperative liver function was normal in 78% of dogs, including 70% with partial shunt attenuation. Experience of the surgeon was related positively to outcome after partial attenuation (P = 0.002). Postoperative biochemical evidence of abnormal liver function was the most sensitive predictor of recurrence of clinical signs referable to persistent portosystemic shunting. CONCLUSIONS: In the hands of an experienced surgeon, surgical attenuation of single extrahepatic shunts was safe and effective, even in animals with partial attenuation. Most dogs with biochemical evidence of persistent shunting suffer relapse of clinical signs within 18 months of surgery. Postligation neurological syndromes of variable intensity may be more common than previously thought.     

ULTRASONOGRAPHIC DIAGNOSIS OF CONGENITAL PORTOSYSTEMIC SHUNTS IN DOGS: RESULTS OF A PROSPECTIVE STUDY

The aims of this study were to determine if accurate diagnosis of congenital portosystemic shunt was possible using two dimensional, grey-scale ultrasonography, duplex-Doppler, and color-flow Doppler ultrasonography in combination, and to determine if dogs with congenital portosystemic shunts have increased or variable mean portal blood flow velocity. Eighty-two dogs with clinical and/or clinicopathologic signs compatible with portosystemic shunting were examined prospectively. Diagnosis of congenital portosystemic shunt was subsequently confirmed in 38 of these dogs using operative mesenteric portography: 14(37%) dogs had an intrahepatic shunt and 24(63%) had an extrahepatic shunt. Ultrasonography had a sensitivity of 95%, specificity of 98%, and accuracy of 94%. Ultrasonographic signs in dogs with congenital portosystemic shunts included small liver, reduced visibility of intrahepatic portal vessels, and anomalous blood vessel draining into the caudal vena cava. Correct determination of intra - versus extrahepatic shunt was made ultrasonographically in 35/38 (92%) dogs. Increased and/or variable portal blood flow velocity was present in 21/30 (70%) dogs with congenital portosystemic shunts. In one dog with an intrahepatic shunt the ultrasonographic diagnosis was based partly on finding increased mean portal blood flow velocity because the shunting vessel was not visible. Detection of the shunting vessel and placement of duplex-Doppler sample volumes were facilitated by use of color-flow Doppler. Two-dimensional, grey-scale ultrasonography alone is sufficient to detect most intrahepatic and extrahepatic shunts; sensitivity is increased by additional use of duplex-Doppler and color-flow Doppler. Increased and/or variable portal blood flow velocity occurs in the majority of dogs with congenital portosystemic shunts.     

ANATOMY OF EXTRAHEPATIC PORTOSYSTEMIC SHUNTS IN DOGS AS DETERMINED BY COMPUTED TOMOGRAPHY ANGIOGRAPHY

Congenital extrahepatic portosystemic shunts are anomalous vessels joining portal and systemic venous circulation. These shunts are often diagnosed sonographically, but computed tomography (CT) angiography produces high‐resolution images that give a more comprehensive overview of the abnormal portal anatomy. CT angiography was performed on 25 dogs subsequently proven to have an extrahepatic portosystemic shunt. The anatomy of each shunt and portal tributary vessels was assessed. Three‐dimensional images of each shunt type were created to aid understanding of shunt morphology. Maximal diameter of the extrahepatic portosystemic shunt and portal vein cranial and caudal to shunt origin was measured. Six general shunt types were identified: splenocaval, splenoazygos, splenophrenic, right gastric‐caval, right gastric‐caval with a caudal shunt loop, and right gastric‐azygos with a caudal shunt loop. Slight variations of tributary vessels were seen within some shunt classes, but were likely clinically insignificant. Two shunt types had large anastomosing loops whose identification would be important if surgical correction were attempted. A portal vein could not be identified cranial to the shunt origin in two dogs. In conclusion, CT angiography provides an excellent overview of extrahepatic portosystemic shunt anatomy, including small tributary vessels and loops. With minor variations, most canine extrahepatic portosystemic shunts will likely be one of six general morphologies.     

Portovenogram findings in cases of elevated bile acid concentrations following correction of portosystemic shunts.

The case records of 36 cats and dogs undergoing surgical correction of a single extrahepatic portosystemic shunt were reviewed. In 12 animals, the shunt was fully ligated during the first surgical procedure, while, in the remaining 24, the shunting vessel could only be partially ligated. Assessment of serum bile acid concentrations demonstrated complete shunt occlusion in 15 of these latter 24 animals (63 per cent) between one and six months postoperatively. Ten animals (28 per cent) had persistently high serum bile acid concentrations postoperatively. Portovenogram findings in these individuals revealed six that demonstrated shunting solely through the original vessel; In five of these, full shunt attenuation was achieved at second surgery. Further shunt manipulation was not possible in the sixth case due to extensive adhesion formation. In the remaining four animals with raised bile acid concentrations, the portovenogram demonstrated shunting through the original vessel as well as the development of multiple acquired shunts.     

Presentation,stabilisation and contrast-enhanced computed tomographic (CT) diagnosis of a left gastrocaval portosystemic shunt in a pony foal

A 2-week-old pony foal was presented for recurrence of neurological dysfunction, obtunded-comatose mentation and collapse. Signalment, history, haematological and clinical chemical measurements and response to treatment prompted a presumptive diagnosis of hepatic encephalopathy secondary to a portosystemic shunt. This case differs from previous reported cases of  congenital portosystemic shunts in horses due to the early onset of clinical signs, which usually occur at 2–6 months of age. In addition, in comparison with previously reported cases, this foal stabilised rapidly with minimal intervention. Contrast-enhanced computed tomography (CT) confirmed a left gastrocaval extrahepatic portosystemic shunt. Surgical intervention was not attempted due to financial constraints, so the foal was subjected to euthanasia, and on post-mortem examination, the anomalous vascular connection could clearly be identified.