The day was stormy. The 61-year-old woman coaxed her donkey into a trot. She loved riding around the ring with the beautiful white beast — at a walk, then a trot, then a canter. Suddenly she noticed a tingling sensation in her arms and legs spreading up her torso. He recognized the sensation but willed himself to ignore it. It didn’t work. Her strength seemed to melt away and before she could say or do anything, she felt herself fall forward. As the world turned gray, the donkey stopped. Her hands, and then her body, slid down the animal’s sweaty neck and onto the ground. Then everything went black.
She woke up staring at the white belly of her ass. He leaned down and spoke softly, then looked at the approaching trainer. are you ok The teacher shouted in alarm. Should I call an ambulance? It was fine, the woman assured the trainer. It’s just that the hot weather could really get to her. She let herself be helped into the cool dark of the barn. As she lay on the wet concrete floor, warm tears streamed down her face. I can’t go on like this she thought.
He passed out for the first time maybe four years earlier. He was at the gym, finishing a brisk walk on the treadmill, when he felt the strange tingling sensation. Black dots swam before her eyes and she heard the thump of her head hitting the wall of windows in front of her. Then black took over. She woke up surrounded by worried faces. It was strange — he was an active person. Her job as a field geologist kept her hiking and climbing regularly.
She went to her primary care provider, a nurse practitioner. He had high blood pressure, the NP said, but was otherwise healthy. The woman was referred to a local cardiologist. Yes, she told the doctor, she got short of breath more easily than she used to. And yes, sometimes he was more tired than he expected. But no, he never had chest pain or pressure. Mostly it felt good. And she didn’t faint often: a few times a year, mostly in summer, when she exercised herself in the heat. She had a stress test and, when that was normal, a scan in which a radioactive dye showed how efficiently her heart was beating. It was also normal. So does an echocardiogram.
Thus, the woman’s NP focused on her blood pressure and improving her diet. But even when her blood pressure seemed perfect, every now and then she found herself on the ground looking at worried faces.
A hard look into her heart
It was after falling off the donkey that the woman decided she needed a new look at her problem. She made an appointment with Dr. David Ramos, a cardiologist at a Columbia New York Presbyterian office in Monroe, New York, near her home in the village of Piermont. It took months to get in. But when she finally met Ramos, he listened thoughtfully as she described her worsening sense of breathlessness, her increasing fatigue even during mild exertion, her repeated blackouts. An examination was unremarkable. Ramos reviewed her records. Fainting, medically known as syncope, is a fairly common problem caused by reduced blood flow to the brain. Forty percent of us will pass out at least once in our lives. And for most of us, it will be only once, caused by a sudden change in either blood pressure or heart rate, often in response to a medication or some kind of emotional or physical stress.
Repeated episodes of syncope indicate a heart problem. The woman’s NP and cardiologist had looked hard at her heart and found nothing. There was no evidence of coronary artery disease or heart failure. There was no sign of an abnormal rhythm that could interrupt blood flow. There was nothing wrong with the structure of her heart — at least it hadn’t been a few years earlier when she had her last echocardiogram. Ramos sent her for another.
This showed that the walls of her left ventricle, the part of the heart that pumps blood into circulation, were thicker than normal. This can happen in people with high blood pressure: The heart becomes more muscular from the work of pumping blood into a system where the pressures are higher. Thicker walls are more rigid, which in turn makes it harder for the heart to relax and expand to fill fully with blood. Could it be that the woman’s heart was unable to pump enough blood to meet the demands of her body and brain when she exercised herself? Ramos prescribed her medication that would help slow her heart, give it more time to fill, and lower her blood pressure.
However, she continued to pass out, now more frequently. During the summer, it may faint once or twice a month. He passed out on the steps of the Leaning Tower of Pisa. Rushing up the long stone stairs at New York’s Grand Central Terminal, she felt the tingling that warned her that another episode was coming. He reached the top of the stairs and laid down on the stone floor. It was ridiculous, but he didn’t pass out. Ramos increased the doses of her medication, then added more. He kept fainting or almost fainting regularly.
Small changes and clear answer
After two years of adjusting the woman’s medications without improvement, Ramos repeated the echocardiogram. The thickening had worsened, but only in the wall separating the left and right ventricles. It was a small change, but enough for Ramos to recognize the problem. The woman had a disorder called hypertrophic obstructive cardiomyopathy (HOCM).
Ramos sat across from the patient with a model of the heart and a piece of paper to help him show how this small change in the structure of her heart could cause her to have syncopal episodes. The wall between the ventricles, known as the diaphragm, plays a unique role in the flow of blood through the heart. When more blood is pumped to the left side of the heart due to increased demand—say, while exercising in the heat—this wall must expand outward, accommodating the larger volume. If it’s thick and stiff, it can’t do it. The thickening had also changed the way the ventricular valves opened so that outflow was now partially blocked with each beat.
The patient noted that she had been fainting for years. why wasn’t her echocardiogram abnormal before? Ramos explained that HOCM affects how the heart pumps long before changes are visible. And the thickening of the diaphragm becomes more exaggerated as time goes on.
HOCM is a family of genetic, mostly inherited disorders that affect up to one in 500 people. While its most dramatic onset occurs in young athletes who die unexpectedly, in most people the disease process begins later and progresses more slowly. Eventually most of those with the condition need surgery to thin the overgrown areas of the diaphragm and repair the valves, Ramos told her. He wasn’t at that point, but he would probably get there.
This diagnosis changed the medications he had to take. Some of those recommended for high blood pressure could worsen HOCM symptoms. And Ramos would have to monitor her heart with echocardiograms every year.
As Ramos described the disease, he referred to it by an acronym. He pronounced it HOE-come. Hearing this, the patient smiled. Ramos looked at her questioningly. “Where I come from,” he said with a laugh, “hokum means nonsense, balloon.”
The woman did well on the new drugs, but just as Ramos predicted, the blockage in her heart worsened. He finally had surgery in 2021. He hasn’t passed out since. He exercises regularly, though not as hard as he used to. No more curling up on the stairs or when it’s hot. Since her diagnosis, she has researched her family history. He had two uncles with heart problems. One died young and the other lived into his 70s but was confined to the house for most of his life. More recently, a nephew and a cousin were diagnosed with HOCM, and the patient continues to encourage others to get tested for the disease.
It turns out, he told me, that despite its name, HOCM is not nonsense after all.
