The day was sultry. The 61-year-old woman pushed her donkey into a trot. He loved riding around the ring on the beautiful white beast: at a walk, then a trot, then a gallop. Suddenly he noticed a tingling in his hands and feet that spread towards his torso. He recognized the feeling but forced himself to ignore it. It didn’t work. His strength seemed to fade, and before he could say or do anything, he felt himself slump forward. As the world turned grey, the donkey stopped. His arms, and then his body, slid down the animal’s sweaty neck and fell to the ground. Then everything went black.
He woke up looking at the snow-white belly of his donkey. He bent down and neighed softly, then brayed at the approaching instructor. Are you OK? The instructor shouted in alarm. Should I call an ambulance? He was fine, the woman assured the instructor. It was just that the heat could really bother her. He let me help him into the cool darkness of the stable. As she lay on the damp concrete floor, hot tears streamed down her face. I can’t continue like this, I think.
She fainted for the first time maybe four years ago. He was at the gym and was finishing a brisk walk on the treadmill when he felt a strange tingling sensation. Black dots swam before her eyes and she heard the thud 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 work as a field geologist allowed her to hike and climb regularly.
She went to her primary care provider, a nurse practitioner. She 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 was out of breath more easily than before. And yes, sometimes he got more tired than he expected. But no, he never had any pain or pressure in his chest. Mostly he felt good. And he didn’t faint often: a few times a year, especially in the summer, when he struggled in the heat. He had an exercise test and, when he was normal, a scan in which a radioactive dye showed how well his heart was beating. It was also normal. Also an echocardiogram.
So the woman’s NP focused on blood pressure and improvements in her diet. But even when his blood pressure seemed to be perfect, he occasionally found himself on the ground looking at worried faces.
A hard look at his heart
It was after the fall from the donkey that the woman decided she needed a fresh look at her problem. He made an appointment with Dr. David Ramos, a cardiologist at a Columbia New York-Presbyterian office in Monroe, N.Y., near his 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 increasing sensation of shortness of breath, her increasing tiredness during even mild exertion, her repeated fainting. One exam was unremarkable. Ramos examined his records. Fainting, known medically as syncope, is a fairly common problem caused by decreased blood flow to the brain. Forty percent of us faint at least once in our lives. And for most of us, it will be just once, triggered by a sudden change in blood pressure or heart rate, often in reaction to a medication or some kind of emotional or physical stress.
Repeated episodes of syncope suggest a heart problem. The woman’s NP and cardiologist had carefully examined 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 disrupt blood flow. There was nothing wrong with the structure of his heart, at least there hadn’t been a couple of years ago when he had his last echocardiogram. Ramos sent her to get another one.
This showed that the walls of the 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 as it works to pump blood into a system where pressures are higher. Thicker walls are stiffer, which in turn makes it difficult for the heart to relax and expand to fill completely 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 during exertion? Ramos prescribed drugs that would help slow her heart, give it more time to fill and reduce her blood pressure.
However he continued to faint, now more frequently. During the summer he may faint once or twice a month. He fainted on the steps of the Leaning Tower of Pisa. As she climbed the great stone stairs of New York’s Grand Central Terminal, she felt the tingling sensation that warned her that another episode was coming. He reached the top of the stairs and lay down on the stone floor. It seemed ridiculous to her, but she didn’t faint. Ramos increased the doses of the drugs, then added more. He continued to pass out or almost pass out regularly.
Small changes and a clear answer
After two years of changes to the woman’s medications without any improvement, Ramos repeated the echocardiogram. The thickening had gotten worse, 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 in front of the patient with a model of the heart and a pad of paper to help him show how this small change in the structure of her heart could be causing her episodes of syncope. The wall between the ventricles, known as the septum, plays a particularly important role in blood flow through the heart. When more blood is delivered to the left side of the heart due to greater demand – for example, during exercise in the heat – that wall must bulge outward, accommodating the greater volume. If it is thickened and stiff, it can’t do it. The thickening had also altered the way the ventricle’s valves opened, so that outward flow was now partially blocked with each beat.
The patient pointed out that she had been fainting for years; Why wasn’t his echocardiogram abnormal before? Ramos explained that HOCM affects how the heart pumps long before changes are visible. And the thickening of the septum becomes more exaggerated as time goes by.
HOCM is a family of genetic, mostly hereditary diseases that affect up to 1 in 500 people. While its most drastic manifestation occurs in young athletes who die unexpectedly, in most people the disease process begins later and progresses more slowly. Ultimately, most who have this condition need surgery to thin overgrown areas of the septum and repair the valves, Ramos told her. She wasn’t at that point, but she probably would get there.
This diagnosis changed the medications he would need to take. Some of those recommended for hypertension may make HOCM symptoms worse. And Ramos would need to monitor his heart with echocardiograms every year.
When Ramos described the disease, he called it by an acronym. He pronounced it HOE-like. Hearing this, the patient smiled. Ramos looked at her questioningly. “Where I come from,” he said, laughing, “hokum means nonsense, nonsense.”
The woman did well with the new medications, but just as Ramos predicted, the blockage in her heart worsened. She finally had surgery in 2021. She hasn’t fainted since. He exercises regularly, although not as hard as before. He no longer feels breathless on stairs or in hot weather. After his diagnosis, he looked into his family history. He had two uncles with heart problems; one died young and the other lived to be 70 but was homebound for much of his life. More recently, a great-grandson and 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 the name, HOCM is no nonsense.