Hassan Shabazz with his mother Marion.
|Failure of the Heart...|
His former basketball buddies keep a watchful eye on him now, calling his mother, an emergency medical services dispatcher, or taking him directly to the hospital, when his symptoms return. They know the signs of trouble. His breathing becomes labored. A walk across a room leaves him exhausted. Climbing the two flights of steps to his apartment might take half an hour. His legs swell. And he has to sleep sitting upright in a chair. He feels like he is dying.
People often feel like that when their hearts are failing. His physician, Marc Klapholz, director of the heart failure program at UMDNJ-University Hospital, says the young man had no history, or previous signs, of cardiac disease. But without warning, his heart stopped doing its job.
Heart failure means that the organ can't pump enough blood to meet the body's needs. Usually it's not contracting well. Sometimes, it can't relax sufficiently. There are often many symptoms, but the most common are shortness of breath and fatigue when exercising.
"The technical term for Hassan's condition is idiopathic cardiomyopathy," says Klapholz, who is also an assistant professor of medicine at UMDNJ-New Jersey Medical School. "What that means is his pump was damaged for no reason that we could detect. One possibility is the muscle was attacked by a virus."
Heart failure is not just a disease of old men. While the incidence increases dramatically after age 45, and it is more common in men than women, anyone can be prone at any time. It is estimated that five to six million Americans have failing hearts, and that 400,000 new cases are diagnosed each year. Depending on the severity of the condition, mortality averages anywhere from 15 to 50 percent in the first year following diagnosis.
In addition, the disabling symptoms mean that many individuals can't work or go about their everyday activities, and that 5,000 are sick enough to be placed on an urgent heart transplant waiting list. About 2,500 of these will die if a new organ is not found within a year; and only about 2,000 hearts are available for transplant in that time period.
"We cannot fix the pump yet," says the cardiologist, "so we try to decrease the resistance against which the heart has to work, and correct the metabolic derangements that occur as a result of the impaired pump. If that doesn't do the job, we need to replace the heart."
The incidence of heart failure is on the rise. Of the total number of cases, about 60 percent result from recurrent heart attacks - a consequence of coronary artery disease. About 30 percent are damaged, either by hypertension alone, or viral infections. Most of the remaining 10 percent are deemed idiopathic, meaning no cause can be found. A small percent are the result of a condition known as peripartum cardiomyopathy that occurs around the time of delivery in 1 in 4,000 pregnancies, often resulting in permanent damage to the heart.
"There are 43 million people in this country with hypertension," says Klapholz. "Half get no treatment. One quarter are treated but not controlled. Only one-quarter are treated and controlled. Hypertension is really a major player in heart failure."
The cardiologist points to the Framingham studies which followed 10,000 individuals in Framingham, MA, over a period of 20 to 25 years. Hypertension was clearly identified as a major factor in heart failure in the majority of cases.
He calls heart failure a cascade of events that becomes a vicious cycle. While it was long thought to be solely a mechanical problem, it is now understood to be far more complex.
"The pump failure is the initial inciting event. Then a host of neurohormones become activated and go crazy, leading to marked disruptions in their function," he explains. "This increases the work of the already weakened heart."
In addition, other substances called cytokines are activated, causing further destruction. Finally, even certain genes are triggered, and appear to cause additional heart cell destruction.
Even so, Klapholz says, well-managed patients should not need to spend much time in a hospital. Unfortunately, many do. The national rate for readmission - following diagnosis of the condition - is 35 percent each year.
Many do well on conventional drug therapy. The art, he says, is in combining drugs and knowing how to work with dosages, and being able to tell when a patient's condition worsens even slightly. This allows for early intervention, which is really the single most important factor in preventing readmission to the hospital.
Marc Klapholz, MD, director of the heart failure program, UMDNJ-University Hospital
There are also many new drugs in various stages of development. Many patients, like Shabazz, are entered into clinical trials of several not-yet-approved medications, which are generally available only through university-affiliated health centers. They are very closely monitored during the trials. A new drug - if it appears to be valuable - is sometimes made available to study participants immediately upon completion of the study, long before others with the condition can get it.
Klapholz says changes in eating and cooking habits, and exercise, are also vital aspects of the regimens. Salt is dangerous for those whose hearts are failing because the patient is already retaining sodium. Salt retention causes water retention, which leads to edema and ‘water' in the lungs.
"This causes shortness of breath and an inability to perform daily activities. Ingesting additional salt only worsens the process," he states.
This often means major changes - both at home and out. No Burger King, says the cardiologist. No quick stops at KFC. No fast foods at all. "You have to learn the salt content of every food you eat, even cornflakes and your favorite bread," he states.
The average pickle has 1,000 mg of sodium, a Burger King chicken sandwich has 1440, a McDonald's quarter pounder with cheese has 1160. A Wendy's bacon and cheese baked potato has 1430. "The Amer-ican diet is loaded with salt," he continues. "Most Americans average 6,000 mg of sodium every day, but heart failure patients are restricted to 1,800 mg. They learn to cook with no salt. At first, it's very difficult, but after two to three months, most people don't miss it at all."
Sandifer was nearing the end of her pregnancy when her heart failed. Klapholz says that of those affected by this condition, 15 percent make a full recovery, 35 percent a partial recovery and the remaining 50 percent have irreversible damage.
Newark resident Barbara Sandifer, 34, knows what it means to overhaul one's life. She was doing just fine, thank you, with a very good job as a public health representative for the New Jersey State Department of Health. She traveled throughout New Jersey's southern counties to inform and advise those whose HIV tests came out positive and to locate their contacts. Hers was not a 9 to 5 job - night hours and weekend work were frequent.
Sandifer was nearing the end of her pregnancy in 1995 when her heart failed. Klapholz says that of those affected by this condition, 15 percent make a full recovery, 35 percent a partial recovery and the remaining 50 percent have irreversible damage.
Sandifer's heart did not recover. She was placed on a respirator until her son, Yusef, was born. Following the birth, the previously energetic woman could barely walk across a room. "I couldn't breathe, I couldn't walk without feeling completely fatigued. I was very disabled," she says.
But Sandifer had never been the type to take things sitting down.
"I couldn't do my job to my standards, so I had to resign," she says. "But I wasn't ready to fail."
She says her doctor brought her back to life. Klapholz calls Sandifer "quite a fighter." She is presently on a regimen of conventional therapies - angiotensin converting enzyme (ACE) inhibitors, angiotensin II receptor antagonists, diuretics to lessen edema, and nutritional changes - along with a recently approved drug, Carvedilol.
ACE inhibitors block the formation of angiotensin II, a neurohormone that reaches high levels when the heart fails. The drug relieves symptoms by reducing resistance to blood flow and prevents salt and water retention. This class of drugs has been commercially available since 1987 and is still a mainstay of treatment. Klapholz says ACE inhibitors bring down out-of-control levels of the neurohormone, and improve survival.
There are various other approved drugs in the arsenal, which decrease mortality as well as improving symptoms and functioning. But none are miracle cures. Digoxin, Klapholz says, lessens symptoms. Drugs that block the receptors to angiotensin II, or a combination of the drugs Hydralazine with isosorbide dinitrate (given to patients who can not tolerate ACE inhibitors), increase exercise capacity and decrease mortality.
A large-scale, national trial - in which Klapholz was one of the principal investigators - recently showed the effectiveness of the third generation beta blocker Carvedilol. This was a major breakthrough since beta blockers were traditionally avoided in the treatment of heart failure because they tend to decrease pump function in normal hearts. However, somewhat paradoxically, this beta blocker improves cardiac function in the diseased heart and actually leads to improved survival. The cardiologist says Carvedilol is not presently recommended for severe heart failure because the data is lacking on its effect, but a study is currently underway to evaluate its value for these patients as well.
There are several other promising new therapies currently undergoing clinical trials nationwide. Seven such investigational drug trials are being conducted at University Hospital.
Klapholz says among those being tested are therapies that raise levels of the body's own counter-regulatory hormones, which go into overdrive when the heart fails. He is studying a class of drugs that prolongs the effectiveness of atrial natriuretic factor (ANF) - a counter-regulatory neurohormone that promotes salt and water excretion, and vasodilation.
Equally important are studies of a drug that blocks the receptors to endothelin, a neurohormone that is activated inappropriately in heart failure patients. Endothelin is the most potent vasoconstrictor, increasing the resistance against which the heart has to pump.
Aerobic exercise - walking, cycling, using the treadmill and Stairmaster - is generally prescribed for all heart failure patients. Physical therapists with expertise in cardiac rehabilitation work with patients to maximize their physical abilities without causing harm. Aerobic exercise promotes improved blood flow and greater exercise capacity.
After nine months of drug therapy and lifestyle changes, Sandifer has returned to work. She has taken a new job with the North Jersey Community Research Initiative, which requires less travel and no night or weekend work. Klapholz says she was in "pretty dire straits" when he first met her, but that her condition now is quite good.
Although half of all heart failure patients are alive five years after their diagnosis, the risk of death from the continued deterioration of the heart, or as a consequence of an unpredictable irregular heartbeat, still remains quite high.
Of course, when all else fails, transplant is the final possibility. But with so few hearts to go around, there are heart failure patients confined to hospitals, dependent on continuous intravenous drips, or mechanical devices.
For those lucky enough to get a new heart, the constant need for immunosuppressive drugs means the patient's defenses are cut way down and infections are always a looming problem. About 30 percent of patients have a serious infection within the first year. Also, the costs of a transplant and the associated care are enormous, running more than $250,000 for the surgery and six months of follow-up care.
But Klapholz says an important new invention may allow many patients to live and function independently who might otherwise have died waiting for a heart. Called a left ventricular assist device (LVAD), the three-inch-long mechanical implant actually helps the heart pump blood. Heart Mate - one of several of these types of devices - was recently approved by the FDA as a bridge to transplant. Studies are currently underway to look at the LVAD as a stand-alone device (in place of a transplant). While the current models are pneumatic (with several small hoses running out of the chest), an electrical implant, powered by a battery pack worn on the shoulder, is expected to be approved by the FDA shortly. Klapholz thinks that the LVAD, which is becoming more miniaturized and with a smaller power source, will eventually be used in place of transplantation for many patients. "For some patients, the LVAD has actually improved heart function," he says. "If the device can reverse damage, it may allow the heart, after a period of resting, to work well again on its own," states the cardiologist. So science promises something only poetry held out to us before - that a seriously broken heart might, in time, be mended.
Fall 1998 Table of Contents