The day was very hot. The 61-year-old woman urged her donkey to trot. She loved riding around the ring on the beautiful white beast – at a walk, then at a trot, then at a canter. Suddenly, she noticed a tingling sensation in her hands and feet, extending towards her torso. She recognized this feeling but wanted to ignore it. It didn’t work. Her strength seemed to fade, and before she could say or do anything, she felt herself falling forward. When the world turned grey, the donkey stopped. She slid her arms, then her body, down the animal’s sweaty neck and onto the ground. Then everything went black.
She woke up looking at the snow-white belly of her donkey. He leaned down and stroked it softly, then brayed at the approaching trainer. Are you well? The coach called out in alarm. Should I call an ambulance? The woman assured the trainer that she was fine. It was the hot weather that could really get to her. She allowed herself to be helped into the cold darkness of the barn. As she lay on the wet concrete floor, warm tears made their way down her face. I can’t go on like this, I thought.
She fainted for the first time maybe four years ago. She was at the gym, finishing a brisk walk on the treadmill, when she felt a strange tingling sensation. Black dots swam before her eyes, and she heard her head hit the wall of windows in front of her. Then black took over. I woke up surrounded by worried faces. It was weird, she was an active person. Her job as a field geologist has her regularly hiking and climbing.
She went to her primary care provider, a nurse practitioner. The NP said she had high blood pressure, but was otherwise healthy. The woman was sent to a local cardiologist. Yes, she told that doctor, she was getting out of breath more easily than she had before. And yes, sometimes she felt more tired than she expected. But no, she never felt chest pain or pressure. Mostly I felt fine. She did not faint often: a few times a year, mostly in the summer when she was exerting herself in the heat. She underwent an exercise stress test, and when that was normal, a scan was performed using a radioactive dye that showed how effectively her heart was beating. It was normal too. So was the echocardiogram.
So the woman’s NP focused on her blood pressure and improving her diet. But even when her blood pressure seemed perfect, she would occasionally find herself on the floor looking up at concerned faces.
A closer look at her heart
After falling off the donkey, the woman decided she needed a new look at her problem. She made an appointment with Dr. David Ramos, a cardiologist at Columbia New York Presbyterian’s office in Monroe, New York, near her home in the village of Pyrmont. It took months before she could enter. But when she finally met Ramos, he listened carefully as she described her worsening sense of shortness of breath, the increasing fatigue she felt even during mild exertion, and the frequent fainting. The exam was normal. Ramos reviewed her records. Fainting, medically known as syncope, is a common problem caused by decreased blood flow to the brain. Forty percent of us will faint at least once in our lives. For most of us, it will only be a one-time occurrence, due to a sudden change in blood pressure or heart rate, often in response to a medication or some type of emotional or physical stress.
Repeated episodes of fainting indicate a heart problem. The woman’s doctor and cardiologist examined her heart intensely 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 there wasn’t any on the echocardiogram two years ago. Ramos sent her for another.
This image showed that the walls of the left ventricle, the part of the heart that pumps blood into the circulatory system, were thicker than normal. This can happen in people with high blood pressure: the heart becomes stronger by pumping blood to a system where the pressure is higher. Thick walls are stiffer, making it harder for the heart to relax and expand until it is completely filled with blood. Is it possible that the woman’s heart was not able to pump enough blood to meet the needs of her body and mind when she exerted herself? Ramos prescribed medications that would help slow her heart, give it more time to fill, and lower her blood pressure.
However, she continued to faint, frequently now. During the summer, she may faint once or twice a month. She fainted on the steps of the Tower of Pisa. As she walked up the grand stone stairs at Grand Central Station in New York, she felt the tingling sensation that warned her of another incident. I reached the top of the stairs and lay down on the stone floor. It sounded ridiculous, but she didn’t faint. Ramos increased the doses of her medications and then added more doses. She continued to faint or nearly faint regularly.
Small changes and a clear answer
After two years of adjusting the woman’s medications with no improvement, Ramos had a repeat echocardiogram. The thickness has worsened, but only in the wall separating the left and right ventricles. It was a small change, but it was enough for Ramos to realize 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 explain how such a small change in the structure of her heart could cause her fainting spells. The wall between the ventricles, known as the septum, plays a uniquely important role in blood flow through the heart. When more blood is delivered to the left side of the heart due to increased demand — for example, during exercise in the heat — this wall needs to protrude outward, to accommodate the larger volume. If it’s thick and hard, it can’t do that. The thickening also changed how the ventricular valves opened, so that outflow became partially obstructed with each beat.
The patient indicated that she had been suffering from fainting for years. Why wasn’t her echocardiogram abnormal before? HOCM affects the way the heart pumps long before changes become apparent, Ramos explained. The thickness of the septum becomes more exaggerated over time.
HOCM is a family of genetic disorders, most of which are inherited and which affect up to one in 500 people. While the most serious presentation occurs in young athletes who die unexpectedly, in most people the disease process begins later and progresses more slowly. Eventually, most people with this condition need surgery to relieve enlarged areas of the septum and repair the valves, Ramos told her. Not at that stage but likely getting there.
This diagnosis changed the medications she had to take. Some of those recommended for high blood pressure can make HOCM symptoms worse. Ramos will need to have her heart monitored with an echocardiogram every year.
As Ramos described the disease, he referred to it by abbreviation. He pronounced it HOE-come. Upon hearing this, the patient smiled. Ramos looked at her questioningly. “Where I come from, judging means bullshit and nonsense,” she said with a laugh.
The woman did well on the new medications, but as Ramos predicted, the blockage in her heart worsened. She finally underwent surgery in 2021. She has not lost consciousness since then. She exercises regularly, but not as vigorously as she used to. No longer get upset on the stairs or when it’s hot. Since her diagnosis, she has investigated her family history. She had two uncles with heart problems. One died young, the other lived into his seventies but remained a resident of the house most of his life. Recently, a nephew and cousin were diagnosed with HOCM, and the patient continues to encourage others to get tested for the disease.
It turns out, she told me, that despite its name, HOCM is not nonsense at all.