Enjaymo prior to major surgery can prevent CAD complications: Case
Pretreatment effective for woman, 80, who underwent urgent aorta surgery
In people with cold agglutinin disease (CAD), pretreatment with Enjaymo (sutimlimab-jome) may limit disease-associated complications following urgent or planned surgeries in which a patient’s body temperature is considerably decreased.
That’s according to a new report detailing the case of an 80-year-old woman in Japan for whom Enjaymo treatment before an urgent surgery to the aorta, the body’s main artery — along with warming measures during surgery — successfully prevented CAD-related issues.
Noting that CAD “can lead to significant complications due to hemolysis [red blood cell destruction] during aortic surgery,” the researchers reported that, for this elderly woman, “the postoperative course was uneventful.” Indeed, “on postoperative day 18, the patient was discharged without any hemolysis-related deficits,” the team wrote.
The case study, “Partial arch replacement for type A aortic dissection with cold agglutinin disease after sutimlimab,” was published in the Journal of Cardiology Cases.
In the report’s “learning objective,” the researchers wrote that Enjaymo “is relatively easy to administer and may help avoid postoperative hemolytic complications.”
CAD is an autoimmune disorder marked by an overproduction of self-reactive antibodies known as cold agglutinins. These antibodies mistakenly attach to red blood cells at cold temperatures, causing the cells to clump, or aggregate, and be marked for destruction — a process known as hemolysis.
Planning for surgeries in people with CAD
Symptoms of CAD tend to worsen in cold environments. This increases the risk of complications during or after cardiovascular surgeries that utilize hypothermic circulatory arrest — a technique in which a person’s core body temperature is significantly lowered and blood circulation is temporarily stopped to facilitate complex procedures.
The researchers noted that, to date, “there are no established methods for the perioperative management of patients with preexisting cold agglutination disease undergoing cardiovascular surgery requiring hypothermic circulatory arrest.” Perioperative refers to the time period before, during, and after surgery.
Enjaymo, the only CAD-approved therapy, is designed to prevent hemolysis by selectively blocking the activity of the C1 protein. C1 plays a key role in the complement pathway, a part of the immune system that contributes to CAD-related hemolysis.
“However, there are no reports of its use in aortic surgery that required hypothermic circulatory arrest,” the researchers wrote.
Here, a research team from Kansai Rosai Hospital described the case of an elderly woman who sought treatment for sudden chest pain. She was diagnosed with CAD, and then underwent an uneventful aortic surgery after receiving pretreatment with Enjaymo.
Rituximab not covered by insurance in Japan
Pretreatment with rituximab — a therapy commonly used off-label in CAD that targets the immune cells that produce antibodies — can be an effective preventive strategy in a surgical setting, but it is not covered by insurance in Japan.
After the chest pain, the woman was diagnosed with an acute type A aortic dissection, or a serious tear in the aorta, the main artery that carries blood from the heart. She also had a bulge in the aorta.
While she was scheduled to undergo an urgent aortic surgery, presurgery blood tests showed low levels of hemoglobin, the protein in red blood cells that transports oxygen, and high levels of hemolysis markers.
These findings suggested hemolytic anemia, a feature of CAD in which red blood cells are destroyed faster than they can be made. A subsequent direct Coombs test, which checks for antibodies and/or complement proteins bound to red blood cells, came back positive, and her levels of cold agglutinins were also markedly high.
Based on all these results, the woman was diagnosed with CAD.
Because of the risk of cold-induced hemolysis during aortic surgery, and given that the woman showed no other life-threatening heart conditions, the physicians decided to delay the procedure to give time to administer Enjaymo.
Vaccination against meningococcal and pneumococcal infections needs to be completed or up to date at least two weeks before the first Enjaymo dose. The woman was given such vaccinations before treatment, which was started three days before the aortic surgery.
Enjaymo can’t be used for emergency surgery
The surgery was conducted 26 days after the onset of chest pain, per the report. To minimize the time spent with reduced blood flow during the procedure, a partial arch replacement was chosen. This technique involved opening the chest and using a cardiopulmonary bypass (CPB), where a machine takes over the job of the heart and lungs during surgery.
The cardiopulmonary bypass was discontinued when the woman’s body temperature dropped to about 28 C (about 82.4 F) to avoid further temperature reduction and associated hemolysis.
The surgery also involved selective cerebral perfusion, in which the blood flow is directed to the brain during certain phases of the operation to ensure it receives adequate blood and oxygen.
Blood products administered during surgery were prewarmed, and the operating room temperature was monitored and maintained at optimal values.
We administered [Enjaymo] treatment before surgery, in consultation with a hematologist [blood disease specialist], and partial arch replacement under mild hypothermia [low body temperature] with CAD was safely performed.
The total time of CPB was about 3.5 hours, and the heart was without a blood supply for nearly two hours.
The surgery was successful and the woman was discharged less than three weeks later.
“We administered [Enjaymo] treatment before surgery, in consultation with a hematologist [blood disease specialist], and partial arch replacement under mild hypothermia [low body temperature] with CAD was safely performed,” the researchers wrote, adding that, “to our knowledge, this is the first reported case of arch replacement with CAD after [Enjaymo] treatment.”
The team noted that the medication “cannot be used in emergency surgery because pneumococcal and meningococcal vaccines are required prior to administration.” But for “urgent or elective” surgeries, pretreatment with Enjaymo “is useful,” the team wrote.
Still, the researchers urged coordinated care in such cases.
“We recommend the use of a multidisciplinary approach, involving [heart] surgeons, anesthetists, [CPB specialists], hematologists, and other allied healthcare professionals to significantly reduce postoperative complications,” the team concluded.
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