Cardiomyopathy

A big-hearted problem

Leonard Simmons suffered from cardiomyopathy – an enlarged heart. The condition can be fatal, but Simmons recovered after weeks of rest.

“It, started with a cough,” recalls Leonard Siminons, a fixture in Now Orleans city government for years. Simmons grow up in the French Quarter, graduated from Tulane University and now enjoys retirement, lie is a softer spoken and less hyperactive version of his exercise-guru brother Richard kSimmons.

“After a few weeks of coughing and a little lever, I saw my physician, who suspected bronchitis. He prescribed sonic antibiotics, and after a day or two I felt well enough to attend a meeting in Lafayette. Then a week later, I was feeling bad again. I became short of breath doing some yard work. That night I could not lie flat and breathe. I slept sitting up.

“The next day I had a chest X-ray. A few hours later, my doctor called with the results. I had an enlarged heart with fluid in my lungs. lie came to my house, picked me up and took me to the, hospital.”

In an effort to pump more blood, a. failing heart often increasis in size. But being bigger is not better. Having a big heart can be dangerous to your health.

There are more than 200 medical conditions that can cause eardiomyopathy or cardiac enlargement. The most common cause of heart failure in Louisiana is coronary artery disease. And some eardiomyopathy – ischemic eardiomyopathy, the most common kind – begins with blocked coronary arteries.

But the term primary cardiomyopathy refers to a large group of diseases whose initial problem actually begins in the heart muscle colls, rather than stemming from corenary artery obstructions, untreated hypertension and damaged heart valves that lead to congestive heart failure.

Of persons with congestive heart failure, perhaps one in 10 has primary cardiomyopathy. Like Gaul, these cardiomyopathies are divided into three types -dilated, hypertrophie and restrictive.

DILATED CARDIOMYOPATHY

As the heart expands in size, the muscular wall becomes thinner and weaker. Pumping is less efficient. Impaired circulation leads to a common condition caused by many heart problems – congestive heart failure. The initial symptom is often shortness ofbreath or a cough that persists.

In Simmons’ situation, an angiogram showed normal coronary arteries and excellent cardiac output. However, his left ventricle was dilated and enlarged. lie had a dilated eardiomyopathy, the most common of the three types.

Simmons recovered after seven weeks of rest and no exorcise. This occurred 10 years ago.

“I have had no subsequent problems, but I do try to watch my diet,” he says.

“And he will never have a broken heart as long UK he is married to me.” chimes in his wile, Cathy.

VIRUSES

Simmons’ doctors diagnosed his illness as a viral cardiomyopathy. Viruses an; often the. culprit when a person with no underlying coronary or valvular heart disease develops cardiac: symptoms alter some sort of antecedent febrile illness or upper respiratory tract infection.

It is a rare condition, and most of the research on viral cardiomyopathies comes from animal studies. More than 40 years ago Dr. George Burch, chair of the Department of Medicine at Tulane, linked certain viruses to card iomyopathy.

Recent technology has detected coxsackievirus and adenovirus In biopsies from people with suspected viral myocarditis. These were the samt? viruses Burch studied in mice.

Treatment is symptomatic and includes a, close watch for irregular heart rhythms. The good news Is that people with viral myocarditis, such as Simmons, usually recover with a simple restriction of heart-strcssful physical activity for several months. The enlarged heart can return to normal or near normal size.

The patient who does not eventually improve may need a cardiac muscle biopsy to look for other problems, which might call for different specific therapy.

Another virus that can cause cardiomyopathy is HIV. The actual role of the virus Is unclear, and secondary factors including certain medications and related opportunistic infections may play a role.

ALCOHOL AND ENLARGED HEARTS

In the United Stilles, the leading cause of primary cardiomyopathy is alcohol consumption. Several studies have compared heart size among heavy drinkers, social drinkers and teetotalers.

The cardiac enlargement in alcoholic cardiomyopathy begins slowly and without symptoms. The left ventricle gradually dilates and becomes larger. An echocardiogram can show these changes belbre UIP, drinker becomes symptomatic.

As alcohol-poisoned heart-muscle cells die or become less functional, cardiac output falls, and the left ventricle fries to compensate for loss of pump pressure by increasing in size. With continued drinking over years, the left ventricle dilates and the wall thins.

The typical patient with alcoholic cardiomyopathy has consumed 90 to 200 grams of alcohol daily for 15 or more years. If you assume that a drink contains 12 grams of alcohol, this works out to eight to 21 drinks a day, an amount more, common around Mardi Gras but hardly the intake of most readers of this magazine.

OTHER CAUSES

At least 75 other specific heart muscle diseases can cause dilated cardiomyopathy. These include certain drugs, genetics and immune-system shenanigans.

Several drills, including cocaine, are cardiotoxic. Chronic and long-term cocaine abuse can cause a cardiomyopalhy, which can reverse when cocaine use stops. Occasional cocaine use is unlikely to c.iiuHe a cardiomyojiathy. However, even a single use can cause lethal cardiac arrhythmias.

Sometimes the individual cardiac muscle cells become pumped up just like the arms of a weight, lifter. The heart chambers shrink in size and hold less blood. Circulation suffers. This kind of hypertrophie cardiomyopathy can be caused by a genetically determined abnormal protein, which causes enlargement of individual cardiac muscle cells. Multiple family members might have the same enlarged and deformed hearts.

Restrictive cardiomyopafhv is the rarest of the three types. It is usually related to some serious systemic illness. For example, some patients with cancer produce amyloid, an abnormal protein thiit chokes out the normal heart muscle cells. The heart muscle becomes stiff, impairing circulation.

Most patients with dilated cardiomyopathies such as Simmons do well, but it is difficult to predict which patient will have jmigressive problems. A small number of patients bacome more and more symptomatic, and some eventually need heart transplants.

Treating cardiomyopathy

* Symptoms – Cough, fatigue and weakness are nonspecific symptoms. Palpitations and unexplained shortness of breath are more specific symptoms. Chest pain can occur.

* Diagnosis – First an echocardiogram measures the size of the four cardiac chambers and the thickness of their walls. In rare situations, a biopsy of the cardiac muscle is needed to make a specific diagnosis.

Dr. John Walsh: Cardiomyopathy and the New Orleans Connection

“At one time the wards at Charity Hospital always had some beds filled with women who developed a cardiomyopathy during the last month of their pregnancy or within five months of delivery.

“[Tulane cardiac researcher] Dr. George Burch became interested in this condition called peripartum cardiomyopathy in the 1950s, as no conventional therapy seemed to help. Burch had an idea that longtime bed rest would help heal their ailing hearts.

“We hospitalized dozens of women and kept them in bed for months and months in a special unit at the old United States Public Health Hospital. Their heart sizes reduced; most improved. This was the first treatment shown to benefit those with cardiomyopathy. At the time there were glimmers of suspicions that malnutrition and alcohol were co-factors, but the recognition of a unique alcoholic cardiomyopathy came a few years later.

“Africa also had some unusual cardiomyopathies. Based on our work here in New Orleans, we secured a grant to go to Uganda. After we began publishing our findings, their minister of health became upset. He did not think it was good for their economy to admit that Ugandans were susceptible to heart disease. Since we were almost through our five-year grant, we stopped early and pulled out.”

-Dr. John Walsh, retired chancellor of Tulane Medical Center, speaking of experiences during his cardiology fellowship

BY BROBSON LUTZ, M.D.

Copyright New Orleans Publishing Group Inc. Feb 2005