Man with VWD type 1 undergoes heart procedure without major bleeding

A man in his 80s with von Willebrand disease (VWD) type 1 underwent a heart procedure to treat atrial fibrillation, an irregular heartbeat that increases the risk of stroke and heart failure. The procedure was completed without major bleeding or blood clot-related complications after he received treatment to help prevent excessive bleeding.

The procedure, called pulsed field ablation, successfully treated the abnormal heart rhythm. A single dose of recombinant (engineered) von Willebrand factor (VWF), a protein present at lower-than-normal levels in people with VWD type 1, increased the activity of the protein before the procedure. One year later, the patient’s heart rhythm remained normal, with no reported bleeding or blood clot-related complications.

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The report, “Successful Pulsed Field Ablation for Paroxysmal Atrial Fibrillation in a Patient with Type 1 von Willebrand Disease under Perioperative Recombinant Human von Willebrand Factor Supplementation,” was published in HeartRhythm Case Reports.

VWD is the most common inherited bleeding disorder. Type 1 is the most common form and occurs when the body has lower-than-normal levels of VWF.

Catheter ablation can be used when medications do not adequately control atrial fibrillation. During the procedure, doctors guide thin tubes called catheters through blood vessels into the heart. They use the catheters to treat small areas of heart tissue that produce or carry abnormal electrical signals. Traditional ablation methods use heat or extreme cold to treat these areas. Because the procedure involves accessing blood vessels and using blood-thinning medications to prevent clots, doctors must carefully manage bleeding risks in people with VWD.

Pulsed field ablation is a newer type of catheter ablation that uses very short, high-voltage electrical pulses instead of heat or freezing to treat targeted areas of heart tissue.

In the report, doctors in Japan described what they believe is the first case of pulsed field ablation performed with recombinant VWF treatment in a patient with VWD type 1.

Doctors manage bleeding risk before heart procedure

The patient, a man in his 80s, had episodes of atrial fibrillation that had not responded adequately to medication. His VWF activity had been as low as 20%, compared with a normal range of 50%–150%. Levels of activity for factor VIII, another protein that helps blood clot and is stabilized by VWF, were also below normal. Despite these abnormalities, he had no history of major spontaneous bleeding.

Although he did not meet the usual criteria for a lower dose, doctors prescribed 30 mg daily of the blood thinner edoxaban after considering his age and bleeding risk. About 12 hours before the procedure, he received a single infusion of vonicog alfa, a lab-made form of VWF. His VWF activity increased to 61% on the morning of the procedure. He received no additional VWF or factor VIII treatment.

During pulsed field ablation, doctors successfully blocked abnormal electrical signals from all four pulmonary veins, where signals that trigger atrial fibrillation often originate. The procedure was completed without bleeding or blood clot-related complications.

The patient was discharged from the hospital two days later. Three months after the procedure, doctors stopped edoxaban following a discussion and individualized assessment of his bleeding and blood clot risks. At the 12-month follow-up, his heart rhythm remained normal without medications to control the abnormal rhythm. No bleeding or blood clot-related complications were reported.

The researchers cautioned that this was a single case involving a patient with relatively mild bleeding problems despite low VWF activity. The findings should not be assumed to apply to people with more severe forms of VWD. They also noted that there is not enough evidence to determine whether pulsed field ablation carries a lower bleeding risk than traditional ablation methods.

“This case demonstrates the feasibility of recombinant human [VWF] as 1 option for perioperative hemostatic management [bleeding around the time of surgery] in a carefully selected patient with type 1 vWD undergoing [atrial fibrillation] ablation,” the team concluded.