A man with cold agglutinin disease (CAD) had significant red blood cell clumping in his coronary arteries during a procedure to correct an abnormal heart rhythm, blocking blood supply to the heart, according to a recent case report.
Researchers called this “a novel and unusual manifestation” of CAD that caused the man’s heart and lungs to fail suddenly, a complication known as cardiopulmonary decompensation. The man was put on bypass to support his heart and lungs, and the blood clots were removed.
“While the disease’s clinical pearls have been well described, there is little consensus on the … significance and management” of CAD during and after a surgical procedure, the researchers wrote in “Cold Agglutinins Causing Intracoronary Hemagglutination During Premature Ventricular Contraction Ablation Requiring Thrombectomy and Venoarterial Extracorporeal Membrane Oxygenation,” published in the Journal of Cardiothoracic and Vascular Anesthesia.
CAD is a form of autoimmune hemolytic anemia (AIHA) that features the presence of self-reactive antibodies — cold agglutinins — that bind to red blood cells at low temperatures, causing them to clump together and ultimately die.
As these clumps move through the bloodstream, they can block blood flow, forcing the heart to work harder to pump blood throughout the body. This can cause high blood pressure (hypertension) and an irregular heartbeat (arrhythmia).
A new CAD complication
Researchers in Richmond, Virginia described the case of a 63-year-old white man who had cardiopulmonary decompensation during a procedure to fix premature ventricular contractions, a problem marked by extra heartbeats that begin in one of the heart’s lower chambers and disrupt its rhythm.
The man’s health issues included severe obesity, hypertension, diabetes, and obstructive sleep apnea, which occurs when breathing frequently stops while sleeping. He also had a history of lupus, heart disease, low thyroid function, and both warm and cold AIHA. He was in the hospital for an elective procedure to ablate premature ventricular contractions, which accounted for 11% of his heartbeats.
Blood testing before the procedure revealed the presence of cold agglutinins at room temperature, but the patient “displayed no clinical signs or symptoms of anemia,” the researchers wrote.
During the procedure, an angiography was performed to map the coronary arteries because the ablation point was suspected to be near those arteries. Angiography uses a special contrast dye and X-rays to look for a blockage or stenosis (abnormal narrowing) inside blood vessels.
A left coronary angiography “showed no significant stenosis in the left main coronary artery and its branches.” Before the doctors moved to the right side, however, the man became agitated and reported severe pain in his back, chest, and shoulders.
He was placed under general anesthesia and began having heart-related issues 20 minutes later, including hypertension, arrhythmia, tachycardia (fast heart rate), and then cardiac arrest, which occurs when the heart stops beating.
CPR was initiated, but the man’s condition didn’t improve. As a last resort, the doctors used extracorporeal membrane oxygenation (ECMO), a type of bypass where a machine takes over heart and lung function.
Multiple blood clots were seen with a second coronary angiography and they were removed through an aspiration thrombectomy, restoring blood flow.
Cold contrast dye causes blood cell clumping
“We hypothesize that the cold contrast dye, which was kept at room temperature and introduced during this initial left coronary angiography, precipitated significant [red blood cell clumping] and thrombosis,” the researchers wrote.
The man’s condition gradually stabilized with the support of ECMO and he was transferred to the intensive care unit, where he received blood transfusions, temperature management, and medications. His heart function improved over time and he was discharged after 22 days with medications to manage premature ventricular contractions and prevent blood clots.
This case highlights the importance of quick decision-making and underscores the need to screen CAD patients to determine the presence of cold agglutinins and the temperatures at which they bind to red blood cells so a procedure is “maintained at temperatures exceeding that of the thermal amplitude,” the researchers wrote. “Careful vigilance for temperature monitoring, [red blood cell clumping], and resulting end organ damage is required to avoid life-threatening events.”