Researchers from the University of California at San Francisco (UCSF) reported the outcome of a retrospective study to determine the safety and efficacy of a procedure that is designed to relieve portal hypertension among patients with hemophilia.
Portal hypertension is high blood pressure in the portal venous system, the portal vein and its branches that serve the liver. If not treated, it can result in liver cirrhosis. Increased pressure in the portal system causes decreased blood flow, which diverts blood into surrounding blood vessels, resulting in enlarged veins, known as varices. Varices can develop on the esophagus and stomach, eventually causing them to weaken, triggering internal bleeding.
Portal hypertension can lead to a number of complications as the liver’s functions deteriorate, including dangerous gastrointestinal bleeding, vomiting of blood and painful fluid buildup in the abdomen. A procedure known as transjugular intrahepatic portosystemic shunt (TIPS) is usually needed to relieve the pressure.
TIPS involves inserting a catheter through the jugular vein and threading it through the superior and inferior vena cava to the hepatic vein. Using a needle and then a “guide wire,” a passage is established between the hepatic and portal veins. The insertion of a pair of tubes (or stents), followed by balloon dilation widens the passage, allowing the blood to flow more freely, relieving pressure.
The UCSF team studied four individuals with hemophilia A and cirrhosis who had undergone the TIPS procedure without complications. Through replacement therapy, their factor VIII levels were raised to approximately 50% before, during and for two days following the procedure to prevent bleeding. The investigators found that measured outcomes for the hemophilia patients were comparable to data from TIPS recipients without the disorder.
Although the study size was small, and further multi-center studies may be necessary, the investigators highlighted the importance of the findings, considering the significant number of hemophilia patients susceptible to cirrhosis, particularly those with hepatitis and HIV. Approximately two-thirds of hemophilia patients are co-infected with the two viruses.
“Complications of cirrhosis are now the leading cause of morbidity and mortality in this population,” said Joshua P. Beirne, MD, UCSF Department of Medicine, and his co-authors. “It is therefore possible that an increasing number of hemophiliacs will be referred for placement of transjugular intrahepatic portosystemic shunts in the future,”
The authors also suggested that if interventional radiologists, the physicians who typically place TIPS, have proof that the procedure is safe for hemophilia patients they might be more likely to perform it. The authors further recommend that an experienced hematologist always be consulted when TIPS is considered for a patient with a bleeding disorder.
“In summary, the results of this study suggest that TIPS can be successfully and safely placed in patients with hemophilia and cirrhosis in the setting of adequate factor replacement,” said Beirne and his co-authors.
The report, “Safety and Efficacy of TIPS in Patients with Hemophilia and Cirrhosis,” was published in the February 2007 issue of the Journal of Vascular and Interventional Radiology.
Source: Medicine & Law Weekly, April 20, 2007