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A large number of blood vessels are found within the digestive nerve tract of the human torso . Heyde Syndrome is a condition characterised by abnormal doings of these ancestry vessels lead to bleeding within the intestine . These vascular freakishness are either inborn in nature or are associated with underlie systemic disorders such as Heyde Syndrome . The risk of developing abnormalities with theblood vesselincreases with aging and in the comportment of other cardiovascular disorders .
What is Heyde Syndrome?
Heyde Syndrome is a rare condition characterise by a trinity of aortic stenosis , get coagulopathy and anaemia lower-ranking to GI bleeding take to angiodysplasia i.e vascular malformation in the gut . It was discovered by Dr. Edward C. Heyde in the year 1958 and was thereafter named after him . The exact etiology of Heyde Syndrome is still unclear ; however , the most recognized hypothesis states that it is cause by the unnatural activity of Von Willebrand factor while crossing the stenotic valve , which in twist increase the risk of infection of intestinal haemorrhage .
Von Willebrand broker ( vWF ) is a blood glycoprotein that aids in blood clotting and pedigree coagulation . vWF lack or dysfunction increases the risk of bleeding , preponderantly in orbit with high blood flow .
Signs and Symptoms of Heyde Syndrome
Heyde Syndrome is primarily characterised by gastrointestinal hemorrhage . This presents itself as :
Heyde Syndrome is closely associated with anemia . continuing going of blood over a period of time can direct to fairness , fatigue , deficiency of energy and constant notion of debilitation . The above mentioned symptoms in improver to presence of aortal stricture refer to Heyde Syndrome .
Epidemiology of Heyde Syndrome
Heyde Syndrome is a rarified stipulation discover among 3 % of the population with aortic stenosis . Heyde Syndrome is more common among the aged universe than the young age group , as the occurrence of aortic stenosis and angiodysplasia increases with age .
Prognosis of Heyde Syndrome
The prognosis of Heyde Syndrome is generally bright and good . If notice early on the symptoms of Heyde Syndrome can be reversed . Once the inherent cause of hemorrhage is detected , the haemorrhage can be contain . In most of the case , the symptom of Heyde Syndrome have totally resolved with aortic valve switch . However , if left untreated , Heyde Syndrome can lead to life - threatening spot in view of extravagant bleeding .
Causes and Pathophysiology of Heyde Syndrome
The exact correlativity between aortal stricture and stomachal tract hemorrhage has still not been confirmed . A expectant number of studies have been convey out and it was offer that Heyde Syndrome is possibly get by mild form of Von Willebrand disease leading to coagulation mental defectiveness . It has been comment that coagulation abnormalcy improved with aortic valve refilling . A sketch carried out by Vincentelli et . al . proclaims that this clotting abnormality is cause by abnormal equipment failure of vVF molecule by the enzyme holler ADAMTS13 in view of excessive stress around the aortic valve . This hinders blood coagulation in the gastrointestinal tract direct to abnormal bleeding .
Complications of Heyde Syndrome
The complications of Heyde Syndrome include :
The comportment of underlie aortal stenosis can compromise the cardiac output which can leave to living - threatening situation in anemic patients .
Risk Factors of Heyde Syndrome
The risk of Heyde Syndrome increase with aging and is seen as receive cheeseparing relation with traditional cardiovascular risk factor .
Diagnosis of Heyde Syndrome
The first tone in diagnosis of Heyde Syndrome would be incur a exhaustive case history of the patient . All inherent disorders have to be note . Faecal occult test generally appears electropositive . A physical examination is not sufficient for correct diagnosing . The following investigative studies are have a bun in the oven for early spotting and direction :
Treatment of Heyde Syndrome
Heyde ’s disease is a very rare condition associated with gastrointestinal bleeding , curdling disorder , aortic stenosis and anaemia . It generally affect the older population with implicit in cardiac issues . From pathophysiological stand point it is make by unnatural performance of the Von Willebrand agent which causes coagulation disfunction . This in spell causes gastrointestinal lesions and excessive bleeding from the GI pathway . Clinical features are associated with blood in faecal matter , benighted coloured faeces , blood stained emesis and anaemia . If impart untreated it can lead to chronic stemma passing and even demise .
Heyde Syndrome is a relatively rare condition and a lot of it is still unknown . A orotund number of studies are being carried out to determine the precise cause of the term . It is a reversible circumstance , if diagnosed at the right time . aortal valve refilling has proved to be successful in bring home the bacon arrant stableness and at present is the most plebeian choice of treatment .
acknowledgment :
Vincentelli , A. , Susen , S. , Le Tourneau , T. , Six , I. , Fabre , O. , Juthier , F. , … & Deklunder , G. ( 2003 ) . Acquired von Willebrand syndrome in aortic stricture . New England Journal of Medicine , 349(4 ) , 343 - 349 .
Kravtsov , D. V. , Greaves , M. , & Coller , B. S. ( 2012 ) . Pathological von Willebrand element – cohere proteolysis in thrombotic thrombocytopenic purpura and the hemolytic – uremic syndrome . Current Opinion in Hematology , 19(5 ) , 459 - 466 .
