Patient stories

YNHH patient stories:
Defining heart failure

By Jan Taylor

Soon after he learned to speak, Laurence Urdang picked up French and German. At age 9, he began studying Latin; at 11, he took up Greek. He studied English literature and linguistics at Columbia, and when Laurence finished his formal education, he became a lexicographer--a writer and compiler of dictionaries. In 1966, Urdang edited the first edition of the 260,000 entry Random House Dictionary of the English Language.

Today, this veritable workhorse of a man has more than 125 reference books to his credit and is hard at work on projects for clients worldwide, spending half the year in Old Lyme, Conn., and half the year in Aylesbury, England.

His medical problems began when , with projects spilling off three desks, Urdang visited a number of doctors complaining about a stomach problem. "After a while they started looking at me as if I was a hypochondriac," he said. He felt progressively worse, and eventually he was unable to lie down without feeling as if he would smother.

"I would sit up all night in a chair, but never having been sick before in my life, I just thought tomorrow it will go away."

But it didn't go away. As Urdang was later to discover, he had experienced a heart attack and was living with congestive heart failure. After about a month of extreme shortness of breath, he decided to see a pulmonologist in New London, who ordered a series of breathing tests and an echocardiogram. When the report came back a day or two later, he referred Urdang to John Elefteriades, MD, chief of cardiothoracic surgery at Yale-New Haven Hospital. Urdang also saw cardiologist Dr. Alan Radoff of Hamden, who admitted him to Yale-New Haven for an angiogram, an X-ray image of the heart and blood vessels.

"When I woke up that afternoon," remembered Urdang, "Dr. Radoff said, 'You might need a new valve, you're certainly going to need a bypass, but you must have this surgery as quickly as possible.' He didn't think I would survive more than three days without surgery." The pumping strength of his heart was measured at 15 percent; normal is 65 percent.

"I figured grandchildren shouldn't be traumatized by dying grandfathers," so Urdang decided to have the surgery. His surgical option existed primarily because he was at Yale-New Haven, one of few places where surgeons do bypasses on patients with extremely weak hearts.

After a triple bypass was performed, Urdang woke up in intensive care with a nurse under each arm trying to wake him from the anesthetic. Into his right ear, the nurse kept saying, "Mr. Urdang, wake up. Say something, Mr. Urdang."

"What do you want me to say?" he replied.

"Say anything, but say a sentence," she said.

"That was a sentence," he answered.

"Well, say something else," she said.

And then, with his best Irish brogue, he said, "I bet you're Irish!"

"Right, he's all right," nurse Tricia Buckley, a native of Cork City, Ireland, answered.

And he was all right. Urdang was hospitalized for 11 days. The time between his diagnosis and surgery was only a week. After the bypass surgery, Urdang's heart pumping strength increased by 50 percent.

"What terrified me most was the thought that I would have to give up certain things, but as it turned out, I was eating pretty healthy stuff before. I can't eat things like calf's liver anymore--because it's solid cholesterol--and I was pretty fond of that." His cholesterol levels have dropped from 189 to 134.

Walking on a treadmill every other day, Urdang plans to swim for exercise while he works half the year in England. A week's trip to Barbados for recuperation was canceled. He has work to do. Four months after nearly dying, he's off to England to continue work on a second edition of his Oxford Thesaurus and a historical nautical dictionary.

"What do I have to recuperate from?" he asked. "I feel fine."

Clinically
It's small, about the size of a person's fist. Relatively simple in function, its primary purpose is to pump...24 hours a day, 70 to 80 times a minute, propelling about five quarts of blood through the body. Blood sustains life because it carries essential oxygen to the body's cells. But it's more often the heart, not the blood, that has captured our imagination. The heart represents not only a fabulous pump made of muscle, but the very essence of a human being. For the heart to fail is no small matter.

More hearts are failing than ever before
Heart failure is the only cardiac problem on the increase. Coronary artery disease, high blood pressure and stroke are all in decline. But not heart failure. More than 400,000 Americans are newly diagnosed with failing hearts each year, joining what amounts to more than three million total patients. By year's end, 200,000 can be expected to die with heart failure--almost half within two years of diagnosis.

Ironically, increasing numbers of failing hearts can be blamed on better medicine. Today a person is more likely to survive a heart attack and therefore live with a damaged and perhaps dysfunctional heart. Better medications, procedures to bypass clogged arteries, heart valve replacement or repair, pacemakers and improved diagnostic imaging procedures for early diagnosis, added to an aging population, combine to mean that heart failure now costs Americans more than any other medical problem.

What is heart failure?
A diagnosis of heart failure is drawn from a "constellation" of symptoms and signs, explained Tarik Ramahi, M.D., director of the Yale Heart Failure and Transplant Cardiology Program. These symptoms arise from the heart's inability to pump blood forward on demand. In short order, blood begins to congest, or back up, and pressure within the heart rises. This puts a strain on the circulatory system, and not enough oxygen-rich blood reaches the body's cells. Symptoms of this congestion include shortness of breath, swelling, fatigue and decreased exercise tolerance.

"One would think defining heart failure is easy, but it's not," said Dr. Ramahi. "Heart failure does not have a single cause; it can be caused by several underlying problems." He explained, "Prior heart attacks, inflammation in the heart, problems with the heart's valves, muscle-pumping problems, or heavy alcohol consumption for a long period of time can all lead to heart failure."

When signs of heart failure appear, doctors usually order an echocardiogram, an ultrasound image of the heart. From this moving image, doctors watch the left ventricle--the heart chamber that works hardest to pump blood to the rest of the body and is almost always the reason for heart failure--to see if it is able to contract well enough. If not, the patient is said to have left ventricular dysfunction.

In the past two decades, improved medications have reduced heart failure symptoms and improved survival. Even those who have surgery still benefit from medical therapy prescribed before and after surgery.

What are the surgical options for heart failure?
Until recently, options for treatment of heart failure were rather limited--medication for most, transplantation for the worst cases. Of the three million Americans with heart failure, the majority have not been eligible for surgical procedures. That's a situation Dr. John Elefteriades and other cardiothoracic surgeons at Yale-New Haven would like to change. They have been looking at surgical procedures not previously considered for those with severe heart failure and some new procedures not even dreamed of five years ago.

Coronary Artery Bypass Graft (CABG)
The coronary artery bypass graft has been around for more than 30 years, but "it used to be taught that you couldn't do a bypass if the left ventricle was very weak from prior heart attacks," recalled Dr. Elefteriades, chief of cardiothoracic surgery. "It was thought the risk of operation was too high and the patient wouldn't benefit anyway because the damage had been done." Not so, they found.

After a heart attack, the muscle at the site of the attack is dead but the muscle in the periphery is still alive, although it lacks blood flow and therefore cannot contract well. When surgeons bypass the blockage with a blood vessel borrowed from elsewhere in the body, the restored blood flow reanimates hibernating muscle.

Yale-New Haven surgeons lead the nation in performing bypass operations on patients whose hearts have very low pumping strength. The bypass improves heart pumping strength by about 30 percent and increases long-term survival. In addition to living longer, patients feel better, reporting their angina has disappeared.

Aneurysmectomy
When a large area of heart muscle dies, as often happens with a heart attack, the dead muscle can stretch out and become thin forming an aneurysm. In some cases, the aneurysm can become as large as the heart itself. Yale-New Haven surgeons have found that by removing the aneurysm--actually cutting away the dead muscle--the function of the failing ventricle is improved.

It's like a facelift for the heart. Sagging muscle is removed and when sewn back together, the smaller heart is a better pump. Aneurysmectomy patients feel better and their hearts have improved pumping strength.

"By bringing down its size, the heart just wakes up," said Dr. Elefteriades. "The procedure is like replacing a 20-pound barbell with a five pound barbell," said Dr. Elefteriades. "You're asking the heart to do a lot less work."

Although aneurysmectomy was first described in 1958 by famous heart surgeon Dr. Denton Cooley, it was not until 1993 that its effectiveness was proven when doctors at Yale-New Haven used diagnostic and cardiology imaging techniques to study the procedure.

Reduction ventriculoplasty
Surgeons are only beginning to evaluate another procedure based on the same principle--the larger the heart, the less effectively it works. When an 18-year-old is hospitalized with a failed heart, it's rarely the result of coronary artery disease, but rather a viral infection. This infection leads to dilated cardiomyopathy, or an enlarged heart.

Doctors at Yale-New Haven worked with a surgeon in Brazil who has had success in cutting out a portion of the muscle wall of the stressed heart to reduce its size. Yale-New Haven surgeons traveled to Brazil to gain experience with the procedure, called reduction ventriculoplasty. Results in 140 Brazilian patients with advanced heart failure are good...the pumping strength of the heart is improved.

"If its effectiveness is borne out in clinical trials at academic centers like Yale-New Haven," added Dr. Elefteriades, "it could be a revolution in the treatment of heart failure."

Cardiac transplant
No longer experimental, heart transplantation is one of the best answers today for a failing heart, but because of a limited supply of donor hearts, transplantation is available to only a small minority of patients. Generally heart transplants go to patients under age 65; most heart failure patients are older than 65. Although nationwide 2,000 patients receive a new heart each year, there are 3,000 on the waiting list. To expand the availability of transplants, Graeme Hammond, MD, professor of cardiothoracic surgery, is involved in groundbreaking research investigating whether organs from other animals could be used--called xenotransplantation.

The artificial heart
In addition, Yale-New Haven is one of a few centers nationwide investigating the use of a portable artificial heart--the Novacor. Novacor doesn't replace the whole heart, just the left ventricle. The artificial heart, once attached to a console the size of a desk, now attaches to a controller the size and weight of a mini-cassette recorder and uses an external battery. Today the Novacor is used as a bridge to transplant, but in Europe these devices have become a permanent answer for the failing heart. Dr. Elefteriades predicts that this portable artificial heart will eventually be approved as a permanent treatment in the U.S.

Dynamic cardiomyoplasty
While some procedures remove the problematic heart muscle or reawaken hibernating muscle, dynamic cardiomyoplasty takes a more creative approach. This new procedure, now in clinical trials at Yale-New Haven, is based on research demonstrating that under electrical stimulation, skeletal muscle can mimic cardiac muscle--no small feat considering cardiac muscle works every minute of every hour of every day, and even the strongest skeletal muscles could not do the same.

In this procedure, the surgeon removes the large latissimus dorsi muscle from the patient's back and wraps it around the heart. A specially designed pacemaker stimulates the "lat" muscle to contract in rhythm with the ventricle so that it will perform continuous "internal cardiac massage." After surgery, the muscle is allowed to heal for two weeks. Then in the next 10 weeks, on an outpatient basis, the muscle is gradually paced with electrical stimuli, taught to contract as though it were a heart muscle. Twelve weeks after surgery, the skeletal muscle is able to contract 24-hours a day, usually assisting every other heartbeat.

Hardly missed from the person's back, the "lat" improves heart function and reduces symptoms of heart failure in its new location. Perhaps its most important contribution is to "girdle" the heart and prevent the left ventricle from increasing in size. Along similar lines, research at Yale is examining whether skeletal muscle can be used to form entirely new pumping chambers.

When surgery is no option
Laurence Urdang was one of a small minority of heart failure patients eligible for surgery. Eighty percent of patients with heart failure are over 65, and therefore not usually eligible for transplantation, still the definitive treatment for severe heart failure. About half suffer heart failure from causes other than coronary artery disease, eliminating the option of bypass surgery.

The portable heart, dynamic cardiomyoplasty, and the other procedures mentioned above are in limited use while under clinical investigation. A complete artificial heart is years away. Coronary bypass and aneurysmectomy, while safe and effective, are appropriate for only certain heart failure patients. And at any rate, these procedures do not completely reverse left ventricular dysfunction, so medical management is still required.

"Although surgical procedures have significantly expanded treatment options for the failing left ventricle," wrote Dr. Ramahi recently, "chronic heart failure from left ventricular dysfunction remains a predominately medically treated disease."

At one time, medications would simply relieve immediate symptoms, but now they have been found to actually improve survival.

Yale-New Haven's heart failure program looks for the underlying cause for a failing heart and tries to identify any cause which can be reversed. The emphasis is on optimizing medical care so that every patient is put on the best medication regimen currently available. The cornerstone of therapy in patients with heart failure is a class of medications called ACE inhibitors. Used in the appropriate doses, they have been shown to decrease admissions to the hospital and improve survival. But although proven to work, they are vastly underutilized.

"Less than 50 percent of those eligible for treatment with ACE inhibitors receive such therapy," Dr. Ramahi said, "and many receiving the therapy do not receive the standard doses. It's a problem we'd like to correct."

In addition to medications of proven benefit, the Heart Failure and Transplant Cardiology Program participates in FDA-approved clinical trials of new medications. "These medications are not used in clinical trials until they've been shown to improve exercise tolerance, symptoms and heart function in earlier studies," said Dr. Ramahi. "People who have a poor prognosis stand to benefit from these protocols."

"The search for new medications for heart failure is intensive and ongoing," Dr. Ramahi added, "and is likely to offer a better future for patients who are not eligible for surgery." From both medical and surgical perspectives, there's new hope for the failing heart.

Next story: Am I Having a Heart Attack?

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Last revised: May 22, 2007 (dh)

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