Ch 96 hepatic vascular anomalies Flashcards
(121 cards)
List the tributaries of the portal vein from caudal to cranialList the tributaries of the portal vein from caudal to cranial
- Mesenteric veins (drain the intestines and form the cranial mesenteric vein)
- Caudal mesenteric vein
- Splenic vein (with left gastric vein)
- Gastroduodenal vein (dogs)
Portal Vein
- portal vein divides into right and left portal branches
- right portal vein: caudate and the right lateral liver lobe
- left portal vein: left medial and lateral and quadrate
- central portal vein(from left branch) to the right medial lobe
In cats the portal vein divides into right, central, and left branches.
Hepatic Artery
- right lateral, right medial, and left branches. The presence of additional branches varies
- In another 50% of dogs the right medial branch arises from the left hepatic artery and supplies only the right medial lobe
How many hepatic veins do dogs usually have?
Which is the largest?
5-8 hepatic veins
The left hepatic vein is the largest and most cranial
drain into the abdominal portion of the caudal vena cava
Embryology
The abdominal venous system originates from umbilical, vitelline, and cardinal veins during embryonic development.
Vitelline veins:
- Carry blood from the yolk sac to the embryonic heart.
- They form a venous plexus > hepatic sinusoids.
- right vitelline vein > caudal vena cava
- left vitelline vein atrophies.
- anastomoses between vitelline veins form the portal vein.
Umbilical veins:
- Carry blood from the allantois to the sinus venosus.
- left umbilical vein channel for placental blood to the liver,
- most blood bypasses the liver directlt to heart via the ductus venosus, a shunt connecting it to the right vitelline vein.
Cardinal veins:
- Three pairs (caudal, subcardinal, and supracardinal) contribute to caudal vena cava.
- supracardinal veins > azygos and hemiazygos veins.
What embryonic vessels give rise to:
- The hepatic sinusoids
- Hepatic portion of the vena cava
- Portain vein
The vitelline vessels
What is the ductus venosus?
A venous shunt between the left umbilical vein and the cranial segment of the right vitelline vein (which become the hepatic vena cava)
Portocaval and Portoazygos Shunts
- Developmental errors > abnormal connections between the cardinal and vitelline systems = extrahepatic portocaval and portoazygos shunts
- Extrahepatic and right or central intrahepatic portocaval shunts may result from persistent connections
- Theory; toy breeds shunts results from concurrent portal microvascular underdevelopment that increases intrahepatic portal resistance, forcing vestigial anomalous vessels to remain open
Patent Ductus Venosus
- ductus venosus is a fetal blood vessel that connects the umbilical vein to the portal sinus, allowing blood to bypass the liver.
- it originates opposite the umbilical vein and ends at the left hepatic vein.
Closure after Birth:
- Normally functionally closes 2-6 days after birth and undergoes structural closure within 3 weeks.
- Delayed closure > Irish Wolfhounds, 65% patent at 4 days old, though all had closed by day 9.
- Hemodynamic factors, such as reduced umbilical venous flow, likely influence closure.
Mechanisms of Closure:
- Smooth muscle contraction, driven by endothelin, cytochrome P-450, thromboxane A₂, and prostaglandins, regulates closure.
- Corticosteroids and indomethacin can accelerate closure in infants.
Persistent Ductus Venosus:
- unknown cause
- from increased sinusoidal pressure due to congenital portal hypoplasia
- chemical imbalances affecting endothelin.
What are the three broad categories of hepatic vascular disease?
- Congenital PSS
- “primary hypoplasia of the portal vein” PVH (PVH with portal hypertension and PVH without portal hypertension. PVH without portal hypertension was previously termed microvascular dysplasia)
- Disturbances in portal outflow
PSS
Portosystemic shunts can either be congenital or acquired in nature
Congenital Portosystemic Shunts
- commonly occurs as a single intrahepatic or extrahepatic vessel that provides direct vascular communication between the portal venous supply and the systemic venous circulation (caudal vena cava or azygos veins)
- 66% to 75% of congenital single PSSs are extrahepatic.
- intrahepatic PSSs are found in larger breed dogs, most extrahepatic PSSs are seen in smaller breeds
- spleno-caval, left gastro-phrenic, left gastro-azygous and right gastric vein account for 94%
- portocaval, portoazygous, cranial mesenteric, caudal mesenteric to caudal vena
Acquired Portosystemic Shunts
- (20%) most commonly occur secondary to chronic portal hypertension; increased portal pressures result in opening of vestigial fetal blood vessels.
- also cirrhosis, PVH with portal hypertension , and arteriovenous malformations
- means for handling the increase in hydrostatic pressure present
- usually multiple, tortuous, and extrahepatic
- connect a portal tributary directly to the renal vein or the caudal vena cava near the kidneys,
Where do acquired shunts most commonly enter the systemic circulation?
What are some causes of acquired shunts?
At the renal vein or the vena cava near the renal vein
Causes:
- Hepatic fibrosis
- PVH with portal hypertension
- Hepatic AV malformations
What % of dogs and cats with PVH-MVD have a concurrent congenital PSS?
Dogs 58%
Cats 87%
What breeds are overrepresented for PVH-MVD?
Cairn terrier, Maltese, Yorkie
Portal Vein Hypoplasia
PVH with hypertension
- characterized by intraabdominal portal hypertension, a patent portal vein, and a lack of cirrhosis on liver biopsy.
- underlying cause unknown > speculated intrahepatic microscopic vascular malformation
Primary hypoplasia of the portal vein without portal hypertension (PVH-MVD)
- microscopic malformation of the hepatic vasculature
- characterized by small intrahepatic portal vessels, often associaed with some fibrosis
- occur as an isolated disease or with congenital PSS
Hepatic Arteriovenous Malformations
- multiple high-pressure arterial and low-pressure venous communications are present within the liver
- usually congenital in nature.
- Typically a branch of the hepatic artery communicates directly with the portal vein(tens to hundreds)
- high-pressure system that causes hepatofugal blood flow and arterialization of the portal vein.
- multiple extrahepatic shunts open to decompress the portal system.
Pathophysiology
Hepatic Encephalopathy
- occurs when more than 70% of liver function is lost
- pathogenesis largely unknown and quite complex
- altered liver cannot perform its role in metabolism or substance clearance, permitting toxic substances to enter the systemic circulation
- > 20 different compounds found in excess in the circulation when liver function is impaired, including ammonia, aromatic amino acids, endogenous benzodiazepines, gamma-aminobutyric acid (GABA), glutamine, short-chain fatty acids
- substances may
1. impede neuronal and astrocyte function, causing cell swelling,
1. inhibition of membrane pumps or ion channels
1. elevation in intracellular calcium concentrations,
1. depression of electrical activity,
1. and interference with oxidative metabolism
1. altered permeability of the blood-brain barrier
Clinical signs
- variable, most suggestive of neuroinhibition.
- Excitatory activity such as seizures, aggression, and hyperexcitability also occur
list compounds found in excess in the circulation when liver function is impaired (7)
more than 20 identified…
- ammonia (decreases ATP availability; increases neuronal and cellular excitability; increases glycolysis; can cause brain edema)
- aromatic amino acids (Decrease dopamine neurotransmitter synthesis; alter neuroreceptors)
- endogenous benzodiazepines (Neural inhibition through hyperpolarization)
- gamma-aminobutyric acid (GABA) (Neural inhibition by hyperpolarizing)
- glutamine (Alters BBB amino acid transport)
- short-chain fatty acids (Decrease microsomal Na+,K+-ATPase in brain)
- Bile acids (alter cell membrane permeability; make BBB more permeable to others)
BASEGAG
Ammonia
- trigger a sequence of metabolic events
- produced by gastrointestinal flora
- converted in the normal liver to urea and glutamine by the urea cycle.
- excitotoxic and associated with an increased release of glutamate (excitatory neurotransmitter).
- Overactivation of the glutamate receptors, (NMDA), has been implicated as one of the causes of hepatic encephalopathy–induced seizures
- chronic liver impairment in PSS, results in alterations in neuronal responsiveness and energy requirements
- Treatments that decrease ammonia concentrations, seem to reduce signs of hepatic encephalopathy.
- Ammonia concentrations do not always correlate with signs > suggesting that other suspected neurotoxins are also very important in the pathophysiology
Coagulation Disorders
- Coagulation abnormalities in patients with liver failure are multifactorial, depending on the interaction of the coagulation, anticoagulation, and fibrinolytic systems.
- Spontaneous hemorrhage is uncommon
- Suggested causes of coagulopathy in liver failure include decreased factor synthesis, increased factor utilization, increased fibrinolysis and tissue thromboplastin release, synthesis of abnormal coagulants (dysfibrinogenemia), decreased platelet function and numbers, vitamin K deficiency (particularly in bile duct obstruction), and increased production of anticoagulants
- Approximately 43% of the dogs with congenital PSS had hypercoagulable thromboelastography results
What breeds are predisposed to exhepatic PSS?
Yorkies, Norweigan Terrier, Havanese, Maltese, Dandie Dinmont Terrier, Pugs, Min Schnauzer
commonly seen in small- or toy-breed
Intrahepatic PSS is overrepresented in larger breed dogs
irish Wolfhounds, retrievers (Labrador, Golden), Australian Cattle Dogs, and Australian Shepherds
What genes have been detected to have an increased expression in intrahepatic and extrahepatic shunts?
Intra: WEE1
Extra: VCAM1
What breed is represented for PVH with hypertension accounting for 27% of cases?
Doberman
Cairn Terriers and yorkies for PVH-MVD