It was the EKG in the emergency department that provided Wallac with the final clues needed to make a diagnosis. EKG measures the electricity generated by the heart to effectively contract muscles. The thick, muscular heart creates larger than normal, exaggerated EKG traces. The more muscles you have, the louder the signal. However, this man’s heart produced a smaller signal than usual. Low electricity may indicate low muscle. Was this man’s heart enlarged by something other than muscle?
There are diseases that invade the heart muscle and look larger but weaker. Such illnesses can explain all the symptoms of men — thick-looking walls, lung overflows, strange EKG, shortness of breath, and even hemoptysis. “I think you may be doing something serious,” Wallac told the patient. Cardiac MRI can give them an answer. The patient underwent the test a few days later. He hadn’t been out of the scanner for more than 20 minutes when his phone rang. It was a wallac. The image tells the story: The man had a disease known as amyloidosis.
Amyloidosis is the ultimate result of many disease processes that ultimately cause zigzag fibers to accumulate in different parts of the body. Cardiac amyloidosis may be the result of a cancer known as multiple myeloma. In this cancer, a type of white blood cell called plasma cells can make abnormal fibers that break down to form the characteristic serrated fibers of amyloidosis. These jagged fibers can also be the result of aging. In this version of the disease, a carrier protein known as transtiletin is broken down, resulting in abnormal but characteristic irregular folds of amyloidosis. In both diseases, these serrated fibers travel through the body and invade and accumulate in the muscles (often the heart muscle).
Blood and urine tests quickly revealed that his illness was not due to myeloma. It was a relief. Patients with cardiac amyloidosis due to multiple myeloma have a poor prognosis. They often die within a year of being diagnosed. Myocardial biopsy proved that it was a form of amyloidosis associated with aging. This type of amyloidosis is also progressive, but much slower. The patient was referred to a cardiothoracic surgeon at Columbia University. Sooner or later he needed a heart transplant.
Three years have passed before Wallach heard from the patient again. He wrote to let Wallac know that he had a heart transplant and was on track. He wrote to thank you: “You saved my life.”
I asked Wallac how he could make this diagnosis when no other doctor had diagnosed it. He called it Aunt Tilly’s signature. “If I explained Aunt Tilly to you and sent you into the crowd to find her, you would probably fail, but if you’ve seen Aunt Tilly,” — he fingers. I snapped — “No problem. You will find her soon. Recognition is everything.”
Lisa Sanders, MD, is a contributor to this magazine. Her latest book is “Diagnosis: Solving the Most Troublesome Medical Mysteries.” If you have a resolved case to share with Dr. Sanders, please write her to Lisa. Sandersmd @ gmail.com.
He was vomiting blood.But his lungs looked okay
Source link He was vomiting blood.But his lungs looked okay