"There is nothing to fear except the persistent refusal to find out the truth." - Dorothy Thompson
One of the chapters in my recent book, "Courageous Confrontations," describes my experience with a patient named Emma Jorgenson. Shortly after sitting down in my consultation room, she said, "I hope you can help me, Doctor. Those other doctors keep saying that my symptoms are all in my head."
"What seems to be bothering you, Emma?"
"Bothering me? My problems aren't just bothering me, they're killing me. If you don't do something to help me, I am going to die. I just know it."
"What kind of symptoms are you having?"
Heaving a huge sigh, Emma shifted uncomfortably in her chair. "I don't know where to begin," she said. "Whenever I try to explain my symptoms to a doctor, he just rolls his eyes, and tells me it's nothing to worry about." Emma hunched over, and began to well up.
I reached for the box of tissues on my desk and handed them to Emma. "Let's figure out how we can help you. How about starting at the beginning?" I said. "What was your first symptom?"
"One night, about three years ago, I woke up with a pounding sensation in my chest. My heart was racing so fast, I thought it was going to jump out of my chest. Then I began to get nauseous and dizzy. I called Dr. Cahill, my family doctor who's also my gynecologist, and when I went in to see her the next day, she found a tumor in my tummy. She said I needed to have an operation to remove it."
"What did she find?"
"A cyst on my ovary. It was nothing serious, but after the operation, the pounding and the dizziness became more frequent, so she sent me to a neurologist."
"Why a neurologist?"
"I'd read an article that said the three most common symptoms of brain tumors were headaches, nausea and dizziness, so I asked her to send me to a specialist. He did a bunch of scans and electrical tests, and said everything was okay. He prescribed a tranquilizer, but I knew that wasn't going to solve my problem."
"Did the tranquilizer help?"
"A little at first, but then my symptoms got worse. When the pounding started, in addition to becoming dizzy and nauseous, my hands would begin to tingle and become numb. After a while, the numbness spread to my face. The whole area around my mouth would lose all feeling, except for a wired tingling sensation. I was sure I going to have a stroke. That's when Dr. Cahill referred me to an ENT doctor."
"An Ears, Nose and Throat doctor?"
"That's right. She thought my dizziness might be due to an inner ear problem. He examined me and said he wasn't sure what was going on, but that I needed an operation to get to the bottom of it. But I was too scared. Besides, I still hadn't recovered from the ovary surgery, and my wounds weren't healing right, so my gynecologist said that I could wait before having another operation."
Emma's story made me wince. She had unwittingly fallen into the maze of modern medicine. Each specialist viewed her symptoms through the prism of his own specialty, ordered the inevitable battery of tests, and treated her with a pill or a procedure without having a diagnosis. Medications are the fifth leading cause of preventable death in the United States.
"Why did she send you to me?"
"I told her I didn't think I had an inner ear problem, and that it had to be some kind of a heart condition. After all, how could an inner ear problem cause chest pains and shortness of breath?"
"Chest pain and shortness of breath? You didn't say anything about that."
I explained that there are several causes for chest discomfort, and each has a telltale set of characteristics. For example, in patients with pleurisy, an inflammation of the lining of the lungs, pain occurs with deep breaths. With an inflammation of the sac around the heart, called pericarditis, the pain increases when a patient lies down, and improves when they sit up and lean forward. In patients with blocked coronary arteries, the discomfort occurs during physical activity, like walking or climbing stairs. A bulging or tear in the aorta, the main artery in the body, also has characteristic features.
All these possibilities and more needed to be carefully explored by delving into the nuances of Emma's chest pain, as well as her palpitations and shortness of breath. Emma's description of her chest pains did not conform to any of the common causes of chest discomfort, but it was important not to overlook other serious possibilities. In patients with pleuritis a rubbing sound can be heard with a stethoscope over the lungs during a deep inhalation. Pericarditis sounds like sandpaper being rubbed in synchrony with the heartbeat. Cardiac birth defects, diseases of the heart muscle, and valve abnormalities all provide telltale murmurs and other characteristic clues on the physical exam.
Despite Emma's rapid pulse, her blood pressure was normal and her lungs sounded clear. On the cardiac exam, her heart impulse was normal, but when I placed the stethoscope under her left breast, the diagnosis immediately became obvious.
When Emma's heart contracted, a series of loud clicking sounds were audible. My patient had mitral valve prolapse.
The heart is divided into two sides, each having four chambers. The upper two are called atria, and the lower two are the ventricles. The right atrium and ventricle receives oxygen-depleted blood from the body and sends it to the lungs where its oxygen content is replenished. The left atrium then receives the rejuvenated blood, passing it along to the left ventricle. The powerful left ventricular chamber then pumps its contents back to the body.
The atria and ventricles are separated from each other by valves. The tricuspid valve is located on the right side of the heart, while the mitral valve resides on the left. When the mitral valve opens, blood exits the left atrium, travels through the valve, and enters the left ventricle. As the left ventricle begins to contract, the valve closes, preventing blood from moving backwards into the left atrium.
The mitral valve consists of two leaflets, or flaps, each in the shape of a parachute. Normally, both leaflets close in unison, but in patients with mitral valve prolapse, either the valve leaflets are too large, the chords that attach them to the heart are too long, or the connective tissue in the structure are more elastic than normal. In any case, one or both of them balloons, or flops-prolapsing into the left atrium. The characteristic click heard when listening to the heart is caused by the sound of the valve leaflet prolapsing into the atrial chamber, much like a parachute in the wind.
Mitral valve prolapse is a relatively common condition, occurring in two and a half to five percent of people in the United States. It is particularly prevalent in pre-menopausal women between the ages of fourteen and forty. There has been a considerable amount of speculation about how the valve abnormalities occur, but recent research has shown that there is a genetic predisposition for the syndrome. Between twenty and fifty percent of the relatives of mitral valve prolapse patients also have the syndrome.
Echocardiograms are a valuable means of evaluating patients with suspected mitral valve prolapse. The test confirms the diagnosis by demonstrating the prolapsing valve leaflets. In addition, the presence and severity of any blood leaking backwards across the valve from the ventricle to the atrium can also be detected. In Emma's case, I did not hear the telltale murmur suggesting the presence of a leak.
Patients with mitral valve prolapse often have symptoms that mimic serious illnesses like heart attacks and cardiac rhythm abnormalities, but in the vast majority of women, the condition is neither dangerous nor life threatening. Most of the close relatives of patients with mitral valve prolapse who demonstrate a floppy valve on echocardiography are completely free of symptoms.
The reason for the chest pains, palpitations, or shortness of breath that occur in some patients with mitral valve prolapse has never been understood. For want of a more scientific explanation, it has been hypothesized that, their nervous systems are programmed to respond excessively to stress. For unknown reasons, they are triggered to react to unthreatening circumstances as though they were dangerous. This imbalance is called dysautonomia.
After putting my stethoscope in the pocket of my lab coat, I patted Emma gently on the shoulder. "I have wonderful news! Your symptoms are being caused by a benign condition called mitral valve prolapse." I assumed that Emma would be relieved. Instead, the diagnosis increased her anxiety to the point where she became a shut-in.
While the symptoms of mitral valve prolapse are divers and can be frighteningly severe, it is important to emphasize that for the overwhelming majority of individuals with the condition, it is neither dangerous nor life-threatening. Studies have found that increase levels of circulating adrenalin like substances account for the symptoms of chest pains, palpitations, shortness of breath, anxiety and panic attacks that plague people with the problem.
The outlook for the great majority of people with mitral prolapse is excellent. My experience has been that those with debilitating symptoms gradually do feel better over time. They come to realize that their symptoms will not result in a heart attack or sudden death, and the symptoms themselves then become less incapacitating. As one of my patients said, "I just put the pains in my handbag and went about my business."
For most patients, the only treatment necessary is the use of prophylactic antibiotics before various types of surgery and dental work, but this is used only in those who have a leaking valve. Medication, particularly a group of drugs called "beta blockers" can be useful to control debilitating symptoms. Exercise, a healthy diet and relaxation techniques have all been useful in controlling symptoms.
And as for Emma, she had a transformative experience that changed her life. No longer immobilized by fear, she developed a remarkable new equanimity and a new appreciation of life.
It also gave her a new life purpose. Emma became a patient care counselor and an invaluable member of our health care team, using her experience as a resource to counsel our fear-ridden patients.