Heyde's syndrome

As Heyde 's syndrome is a disease ( syndrome) is called, the combination of an acquired aortic stenosis and bleeding from malformed blood vessels ( angiodysplasia ) consists of the ascending part of the colon. Sometimes occurring through the bleeding anemia is considered part of the syndrome. It was named after the American internists Edward C. Heyde, who described the syndrome in 1958.

Epidemiology

The exact prevalence of the syndrome is unknown. In one study, an aortic valve stenosis in 2003 at 73 angiodysplasia patients by echocardiography in 31 percent noted. In the control group, the stenosis was only 14 percent of those examined.

Symptoms

Bleeding in the gastrointestinal tract express themselves in bloody vomiting, black stool or blood deposits in the chair. Severe aortic stenosis manifests itself in shortness of breath (dyspnea ), chest tightness (angina) and paroxysmal loss of consciousness ( syncope). However Milder forms can also exist without discomfort.

Causes

The causal link between the stenosis by calcification and degeneration of the aortic valve, bleeding from malformed blood vessels in the region of the ascending colon and cecum of has not been elucidated. It is assumed that a disturbance in the regulation of blood coagulation by reducing the von Willebrand factor, but for which there is so far no evidence.

Diagnostics

In a colonoscopy due to bleeding occurred, the blood vessel malformations ( angiodysplasia ) can be detected. A stenosis of the aortic valve can be confirmed echocardiographically. The usual case of a von Willebrand syndrome diagnostic procedures can be normal due to the low changes.

Therapy

The Symptomatic treatment includes blood transfusion, a gift of desmopressin and factor VIII preparations. In case reports have described that the replacement of the affected heart valve leads to annealing of the intestinal bleeding. Even a partial removal of the colon ( colectomy ) has been successfully applied as a means of treatment of intestinal bleeding.