Heart Disease UK

From Richard N. Fogoros, M.D.
A common congenital heart disorder
Hypertrophic cardiomyopathy (HCM), originally felt to be rare, is now known as a common congenital heart disease that causes several varieties of heart problems – not the least of which is sudden death.

What is HCM and what causes it?
HCM is a form of heart disease in which the muscular walls of the ventricles become abnormally thickened. The thickening of the heart muscle causes the muscle itself to function abnormally. The thickening can also cause the ventricles to become distorted, which can interfere with the function of the aortic valve and the mitral valve.
HCM is caused by a genetic abnormality that produces a striking disorder in the growth of the heart muscle fibers.
However, in almost half the patients with HCM, the genetic problem is not inherited at all, but occurs as a spontaneous mutation – in which case parents and siblings of the patient will not be at risk for this condition (but children of the patient can be.)

What problems does HCM cause?
There are four kinds of cardiac problems caused by HCM:
1) HCM can cause diastolic dysfunction. "Diastolic dysfunction" refers to the fact that thickened ventricles become stiff, making it more difficult for the ventricles to fill with blood. This stiffness causes the blood to "back up" into the lungs, causing shortness of breath – usually with exertion. The diastolic dysfunction also makes it more difficult for patients with HCM to tolerate arrhythmias, especially atrial fibrillation.
2) HCM can cause systolic dysfunction. "Systolic dysfunction" means that the heart's pumping action is not normal - that is, when the heart beats, an unsufficient volume of blood is ejected. In HCM, systolic dysfunction is usually caused by abnormal functioning of the mitral or aortic valves, which, in turn, is caused by distortion of the ventricles resulting from the abnormal thickening of muscle.
3) HCM can cause dilated cardiomyopathy. This condition leads to heart attack, and is caused by an eventual “burning out” of the thickened heart muscle. Dilated cardiomyopathy occurs late in the course of the disease.
4) Finally, HCM can cause sudden death. The sudden death in HCM is usually due to ventricular tachycardia or ventricular fibrillation. While many of these sudden deaths occur during vigorous exertion, it can also occur during minimal exertion or at rest, with no warning whatsoever. The risk of sudden death has been estimated being as high as 5% per year in patients in their teens and 20s, though it drops off somewhat after that.

How is HCM diagnosed?
In general, the echocardiogram is the best method of diagnosing HCM. The echocardiogram allows accurate measurement of the thickness of the ventricular walls, and can detect abnormal heart valve function as well. The electrocardiogram (ECG) also can give important clues as to the presence of HCM.
Both an ECG and echocardiogram should be performed in close relatives of a patient diagnosed with HCM, and an echocardiogram should be performed in any person in whom the ECG or the physical examination suggests ventricular hypertrophy.

How is HCM treated?
HCM cannot be cured, but it can be managed. Beta blockers and calcium blockers can help reduce the "stiffness" in the thickened heart muscle. In some patients - especially those who have significant heart valve dysfunction - surgery to remove portions of the thickened heart muscle is necessary. Atrial fibrillation, if it occurs, often causes severe symptoms and needs to be managed more aggressively in patients with HCM than in the general population.

How can sudden death be prevented?
Sudden death in HCM is often seen in younger patients – often before symptoms have occurred, or even before a diagnosis has been made. While sudden death is always a devastating problem, it is particularly so when it occurs in young people.
Many methods have been tried for reducing the risk of sudden death in patients with HCM - including avoiding exercise, using beta blockers and calcium blockers, and using antiarrhythmic drugs - these methods unfortunately met with mixed results. In recent years it has become apparent that in patients whose risk of sudden death appears high, an implantable defibrillator should be used. The implantable defibrillator is a pacemaker-like device that is implanted under the skin, monitors the heart rhythm continuously, and automatically delivers a shock to the heart to restore a normal rhythm should a dangerous ventricular arrhythmia occur. While it sometimes seems a drastic step, it is much less drastic than allowing a young individual to die suddenly.

By DrRich
EECP may help in heart failure
Enhanced External Counterpulsation (EECP) is a procedure that has proven beneficial in patients with angina. Some have long speculated that the cardiovascular effects induced by EECP might also be useful for patients with heart attack.

What is EECP?
EECP is a mechanical procedure in which long inflatable cuffs (like blood pressure cuffs) are wrapped around both of the patient’s legs. While the patient lies on a bed, the leg cuffs are inflated and deflated with each heartbeat. This is accomplished by means of a computer, which triggers off the patient’s ECG so that the cuffs deflate just as each heartbeat begins, and inflate just as each heartbeat ends. When the cuffs inflate they do so in a sequential fashion, so that the blood in the legs is “milked” upwards, toward the heart.

EECP has two potentially beneficial actions on the heart. First, the milking action of the leg cuffs increases the blood flow to the coronary arteries. (The coronary arteries, unlike other arteries in the body, receive their blood flow after each heartbeat instead of during each heartbeat. EECP, effectively, “pumps” blood into the coronary arteries.) Second, by its deflating action just as the heart begins to beat, EECP creates something like a sudden vacuum in the arteries, which reduces the work of the heart muscle in pumping blood into the arteries.

EECP in heart failure
In a study, 26 patients with stable congestive heart attack were enrolled to receive a standard, 35 session course of EECP. 19 patients completed the EECP sessions and were followed for 6 months afterward. These patients showed, on average, a significant improvement in their functional capacity and quality of life. The authors point out, as well, that the EECP was well-tolerated in these patients.
Since there were no control subjects in this small study, no firm conclusions can be drawn about how useful EECP might be in treating heart failure. But the study was impressive enough to launch a larger, randomized clinical trial that should provide more definitive data on how well EECP might benefit patients with heart failure. The PEECH trial has already begun.
Despite the fact that the potential benefits of EECP in heart failure are still being evaluated, the FDA was sufficiently convinced of these benefits that it cleared the makers of the EECP system (Vasomedical) to begin promoting EECP for heart failure.
Most cardiologists have not embraced the use of EECP for heart disease, quite justifiably citing the need for larger clinical trials. However, since cardiologists don't like EECP even when it is of proven benefit, patients with heart failure who are interested in this treatment option should watch for results of the PEECH trial, and if they prove positive, should take the initiative in bringing up the option of EECP to their doctors.

From Richard N. Fogoros, M.D.
Causes, symptoms, diagnosis, and prognosis

What is cardiomyopathy? What is heart failure?
Cardiomyopathy is disease of the heart muscle.
In most cases, cardiomyopathy causes the heart muscle to become weak. Various medical disorders cause various types of cardiomyopathy, but all types of cardiomyopathy ultimately do the same thing – they reduce the efficient functioning of the heart muscle, and diminish the ability of the heart to meet the needs of the body. When the heart can no longer pump enough blood to meet the needs of the body, heart failure is said to be present.

What are the types of cardiomyopathy?
There are three major types of cardiomyopathy – dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. The vast majority of patients who develop cardiomyopathy have the dilated form. So, after briefly describing hypertrophic and restrictive cardiomyopathies, we will concentrate on dilated cardiomyopathy for the remainder of this article.
Hypertrophic cardiomyopathy is a genetic disorder that causes a chaotic growth of heart muscle cells within the ventricles. The disordered, thickened heart muscle can lead to problems pumping sufficient blood to the body’s organs, and can cause potentially fatal cardiac arrhythmias. Restrictive cardiomyopathy is a very rare condition in which the heart muscle is infiltrated, and made stiff, by abnormal cells, protein, or scar tissue. The stiffening of the ventricles restricts the return of blood to the heart, causing the blood to “dam up” into the body’s organs. The most common cause of restrictive cardiomyopathy is amyloidosis, a disease in which protein-like substance is deposited within the body’s tissues. Other causes include sarcoidosis and hemochromatosis.
In dilated cardiomyopathy, previously normal heart muscle becomes damaged, leading to a generalized weakening of the walls of the cardiac chambers. To compensate for the weakening of their muscular walls, the cardiac chambers dilate. The weakening and the dilation of the heart muscle eventually lead to heart failure.
What causes dilated cardiomyopathy?
Because almost anything that damages cardiac muscle can lead to dilated cardiomyopathy, there are many causes.
The most common cause of cardiomyopathy in developed nations is coronary artery disease. Heart attacks cause death of heart muscle by obstruction of a coronary artery. While the damage is localized to the region of muscle supplied by that artery, within a few months the entire left ventricle dilates to compensate for the damage. With a small heart disease, the amount of ventricular dilation is minimal. But with a large heart attack or a series of smaller heart attacks, dilated cardiomyopathy becomes extensive, and heart failure ensues.
Another common cause of dilated cardiomyopathy is inflammation of the heart muscle, a condition called myocarditis. Myocarditis is most often caused by viral infections, but can also be caused by bacterial infections and by non-infectious causes such as lupus and other inflammatory diseases.
Alcohol is another cause of cardiomyopathy. In some patients (probably determined by genetic predisposition), alcohol acts as a powerful toxin to heart muscle, directly damaging cardiac cells. Alcoholic cardiomyopathy can be seen after as few of five years of excessive alcohol intake.

From Richard N. Fogoros, M.D.
Symptoms, diagnosis, and prognosis

Causes of dilated cardiomyopathy
Valvular heart disease, especially aortic regurgitation and mitral regurgitation, cause dilated cardiomyopathy. Indeed, the gradual enlargement of the cardiac chambers is an important sign that the time may be right for valve replacement or repair.
Nutritional abnormalities – especially a deficiency in vitamin B1 – can cause cardiomyopathy. Cardiomyopathy sometimes develops in women within a month of delivering a baby. This so-called peripartum cardiomyopathy is the result of a myocarditis that occurs for unknown reasons, associated with childbirth.. There are also genetic forms of dilated cardiomyopathy.
This is why some families are clearly affected by an extremely high incidence of dilated cardiomyopathy.
Cardiac “overwork” is another cause of dilated cardiomyopathy. Any condition that causes the heart muscle to work at high loads for prolonged periods of time (weeks or months) can eventually cause cardiac dilation and weakening of the heart muscle. Such conditions include prolonged severe anemia, abnormal sustained tachycardias, chronic hyperthyroidism, and the overwork produced by leaky heart valves.

What are the symptoms of dilated cardiomyopathy?
The symptoms of cardiomyopathy are those of heart disease. These include shortness of breath and/or fatigue with exertion or when lying down, waking up at night gasping for air, and swelling in the lower legs.

How is cardiomyopathy diagnosed?
Diagnosing dilated cardiomyopathy depends on demonstrating enlargement of the cardiac chambers, especially the ventricular chambers. Such enlargement can be seen on chest X-ray, but can be more accurately assessed using an echocardiogram or a MUGA scan.
Once dilated cardiomyopathy is found, every effort should be made to identify a potentially reversible cause. Coronary artery disease and valvular heart disease need to be ruled out. Anemia, abnormal tachycardias, nutritional deficiencies, alcoholism, and thyroid disease also need to be ruled out. Sometimes, a cardiac biopsy is performed to rule out active myocarditis.

What are the clinical pattern and prognosis of dilated cardiomyopathy?
Since it is generally causes no symptoms until actual heart failure sets in, by the time cardiomyopathy is diagnosed, heart disease is usually already fairly advanced. Classically the clinical pattern of a patient with dilated cardiomyopathy is characterized by episodes of severe heart failure that lead to hospitalization, followed by relatively long periods of “baseline” symptoms. During this baseline period, patients often have symptoms only with exertion. As time goes by, the episodes of severe coronary heart disease come more and more frequently, and the “baseline” periods are characterized by a gradually worsening level of symptoms. In the year or so prior to death, frequent hospitalizations are common, and it is usually apparent to both patient and doctor that a steady, unrelenting deterioration is under way.

From Richard N. Fogoros, M.D.,

Treating dilated cardiomyopathy and heart failure
The first rule of treating cardiomyopathy and heart disease is to aggressively seek out and treat any reversible underlying cause. Treatment for coronary artery disease or valvular disease needs to be optimized. Thyroid disease, anemia, or nutritional deficiencies need to be reversed. Abnormal tachycardias need to be aggressively controlled.
While attempting to identify and stabilize the underlying cause, treatment should be instituted to minimize the symptoms of heart failure and to optimize the efficiency of the failing heart. The mainstay of treatment has been and remains medication, but transplant surgery has saved thousands of lives, and new medical devices are being developed to treat cardiomyopathy and heart disease.

Drugs
Digitalis. Digitalis agents improve the force of heart muscle contraction by increasing the amount of calcium inside cardiac cells.
If digoxin helps this condition, its effect seems to be marginal. Also, a recent study suggests that digoxin might actually be detrimental in women who have heart failure.
Diuretics. Diuretics, or “water pills,” increase the elimination of sodium through the kidneys. Eliminating sodium reduces the fluid retention that occurs in heart failure, helps rid the lungs of the excess fluids that cause shortness of breath, and helps reduce leg swelling. Most patients who have experienced an episode of heart failure are placed on daily therapy with diuretics.
ACE inhibitors. ACE inhibitors act to dilate blood vessels in the body. This is extremely important, because in heart failure the body responds by constricting blood vessels, and the constriction of blood vessels greatly increases the work of the heart. ACE inhibitors have proven to be the most effective in improving both the symptoms and the outcome of patients with heart failure. Virtually every patient with heart failure should be on an ACE inhibitor.

Antiarrhythmic therapy.
Unfortunately, patients with moderate to severe dilated cardiomyopathy have a significantly increased risk of sudden death from ventricular arrhythmias. Also unfortunately, antiarrhythmic drugs have never been shown to reduce that risk. The implantable defibrillator has been shown to significantly reduce mortality in certain subsets of patients with cardiomyopathy, particularly in those with prior heart attacks. A trial has shown that patients with prior heart attacks whose ejection fractions are less than 30% have a significantly improved survival when they receive implantable defibrillators.

Cardiac resynchronization therapy (CRT).
CRT is a new form of treatment for some patients with dilated cardiomyopathy. CRT is a form of cardiac pacing that stimulates both ventricles (right and left) simultaneously. The purpose of CRT is to coordinate the contraction of the ventricles, which improves the efficiency of the heart, and increases the amount of blood pumped with each heart beat. Any patient with dilated cardiomyopathy and a complete or partial bundle branch block should be considered for CRT.

From Richard N. Fogoros, M.D.,

Getting what you need from your doctor
Anyone diagnosed with heart disease should expect the following:
1)Insist on a thorough search for a definitive underlying cause. Especially important is to rule out undiagnosed coronary artery disease and valvular heart disease.
But you need to make sure that your doctor aggressively focuses on looking for all reversible causes of cardiomyopathy, before further heart damage is done.
2)Insist on being placed on ACE inhibitors and beta blockers. These two drug therapies for cardiomyopathy are relatively recent, and in the case of beta blockers are counterintuitive and against what many doctors have been taught for years. But these therapies are now well-documented not only to improve symptoms, but also to reduce mortality with dilated cardiomyopathy.
3)Insist on being carefully instructed on how to monitor yourself to reduce the frequency of the periodic exacerbations of heart failure seen with dilated cardiomyopathy. It is now felt that by looking for telltale changes in vital signs and weight, the exacerbations of heart disease that lead to periodic hospitalization can often be “headed off” by timely medication adjustments.
4)Insist that your doctor specifically address the issue of life-threatening arrhythmias. While usage of the implantable defibrillator is limited by the FDA, appropriate indications for using this life-saving device in patients with cardiac problem are changing frequently. Since a large proportion of deaths in patients with dilated cardiomyopathy are sudden and due to arrhythmias, this is an important issue that needs to be periodically revisited.
5)Ask your doctor if you are a candidate for CRT. This new therapy is possibly the most important recent advance in treating cardiac failure, and many doctors are still completely unaware of it.

From Richard N. Fogoros, M.D.,
A useful treatment for angina your cardiologist doesn't want to hear about.
Recent data documenting the effectiveness of Enhanced External Counterpulsation (EECP) for the treatment of angina has failed to bring this apparently effective procedure into the mainstream of cardiology practice. In this article, DrRich discusses what EECP is, how it works, and why cardiologists are avoiding this safe, noninvasive treatment like the plague.

What is EECP?
EECP is a mechanical procedure in which long inflatable cuffs are wrapped around both of the patient’s legs. While the patient lies on a bed, the leg cuffs are inflated and deflated with each heartbeat. This is accomplished by means of a computer, which triggers off the patient’s ECG so that the cuffs deflate just as each heartbeat begins, and inflate just as each heartbeat ends. When the cuffs inflate they do so in a sequential fashion, so that the blood in the legs is “milked” upwards, toward the heart.
EECP has two potentially beneficial actions on the heart. First, the milking action of the leg cuffs increases the blood flow to the coronary arteries. Second, by its deflating action just as the heart begins to beat, EECP creates something like a sudden vacuum in the arteries, which reduces the work of the heart muscle in pumping blood into the arteries. Both of these actions have long been known to reduce cardiac ischemia (the lack of oxygen to the heart muscle) in patients with coronary artery disease. Indeed, an invasive procedure that does the same thing, intra-aortic counterpulsation (IACP, in which a balloon-tipped catheter is positioned in the aorta, which then inflates and deflates in time with the heartbeat), has been in widespread use in intensive care units for decades, and its effectiveness in stabilizing extremely unstable patients is well known.
While a primitive form of external counterpulsation has also been around for a long time, it has not been very effective until recently. Thanks to new computer technology that allows the perfect timing of the inflation and deflation of the cuffs, and produces the milking action, modern EECP has been greatly enhanced.
EECP is administered as a series of outpatient treatments. Patients receive 5 one-hour sessions per week, for 7 weeks (for a total of 35 sessions). The 35 one-hour sessions are aimed at provoking long lasting beneficial changes in the circulatory system.

How effective is it?
EECP now appears to be quite effective in treating chronic stable angina. A randomized trial with EECP, showed that EECP significantly improved both the symptoms of angina (a subjective measurement) and exercise tolerance (a more objective measurement) in patients with coronary artery disease. EECP also significantly improved “quality of life” measures, as compared to placebo therapy.
More recent data show that this improvement in symptoms following a course of EECP seems to persist for up to five years.
Furthermore, there is also preliminary data suggesting that EECP may be useful for treating unstable angina, as adjunctive therapy after revascularization (i.e., with angioplasty, stent, and/or bypass surgery), and even as first-line (instead of last resort) therapy for more routine forms of angina.
Finally, clinical trials have suggested that EECP may be useful in improving symptoms in patients with heart failure.

From Richard N. Fogoros, M.D.
How EECP works, and who it may help
Who is likely to benefit from EECP?
Based on what is already known, EECP should be considered in anybody who still has angina despite maximal medical therapy and prior revascularization. No cardiologist could argue logically against this. And, frankly, if a patient insisted on trying EECP prior to agreeing to purely elective revascularization for chronic stable angina, the cardiologist might not like it, but would be hard pressed to give anything beyond a purely emotional reason as to why this should not be tried.

Why does EECP work?
The mechanism for the sustained benefits seen with EECP still amount to speculation. Everyone can agree that there are good reasons for EECP (just as for IACP) to benefit the heart while the therapy is actually taking place. But as to why the benefit of EECP persists even after the therapy is finished, no one can say for sure.
There are preliminary data suggesting that EECP can help induce the formation of collateral vessels in the coronary artery tree, by stimulating the release of nitric oxide and other growth factors in within the coronary arteries.
There is also evidence that EECP may act as a form of “passive” exercise, leading to the same sorts of persistent beneficial changes in the autonomic nervous system that are seen with real exercise.

Can EECP be harmful?
EECP can be somewhat uncomfortable, but is not painful. In fact, it is apparently very well tolerated by the large majority of patients.
But not everyone can have it. People probably should not have EECP if they have certain types of valvular heart disease (especially aortic insufficiency), or if they have had a recent cardiac catheterization, an irregular heart rhythm, severe hypertension, significant blockages in the leg arteries, or a history of deep venous thrombosis (blood clots in the legs). For anyone else, however, the procedure appears to be quite safe.

From Richard N. Fogoros, M.D.
Why cardiologists don't like it - and what you should do about it
Despite its increasingly apparent potential usefulness, EECP is hardly taking the cardiology world by storm. In fact, it seems that for most cardiologists EECP is not even on the list of potential treatments for coronary artery disease. Why is that?
There are several possible reasons. Let us dispense with the most obvious first, namely, that EECP doesn’t pay well. A series of 35 treatments costs $5000 to $6000 dollars. That’s not chicken feed, but keep in mind that we’re talking about 35 hours of therapy over 7 weeks, which involves not only the doctor’s time but also the time of office staff, nursing personnel, etc., etc. Still not a terrible return, but when you consider that a cardiologist can often bill that much by spending a morning in the lab.
Then there’s the fact that EECP remains somewhat intellectually unsatisfying. To your average cardiologist, there’s no reason at all that anyone should have thought it would work in the first place – that temporarily providing counterpulsation would have lasting effects. And the fact that it apparently does work is merely blind luck, and leaves investigators scrambling ridiculously to explain why it does. This is a less than satisfying way to advance science.
In addition, to most cardiologists, EECP is logistically difficult. To accommodate patients for EECP, they would not only have to purchase expensive equipment, but also would have to radically change the organization of their offices, their office staff, and their space.
Finally, and most importantly, EECP has nothing in common with what cardiologists do. Cardiologists study and treat the heart, for goodness sake. They stress it, image it, measure it, pace it, shock it, stent it, ablate it, revascularize it, and bathe it in drugs. What they do takes years of specialized training and expertise, millions of dollars of high-tech equipment, and tremendous manual dexterity, and it brings them significant prestige, even within the medical community.
This is the real reason the average cardiologist is completely ignoring EECP, as if it doesn’t even exist. They simply can’t believe anyone really expects them to do this.
In any case, you may need to raise your cardiologist’s consciousness. If you have coronary artery disease that has proved difficult to treat, then you need to bring EECP up yourself.
Once enough patients show themselves to be aware of this new therapy and to be expecting it, suddenly EECP will no longer be beneath cardiologists, and they’ll eagerly find a way to incorporate it into their practices.

How can you receive EECP?
If you are a candidate for EECP and wish to pursue it, start with your doctor. If your doctor discourages you from pursuing EECP, make sure he/she gives you a good reason for discouraging it. Good reasons would include: you don’t have the sort of coronary artery disease or angina that would benefit from EECP; your coronary artery disease is of the type that requires revascularization; or you have one of the contraindications for having EECP.

From Richard N. Fogoros, M.D
The results of two large clinical trials using drug-coated stents were presented at the Transcatheter Cardiovascular Therapeutics 2002 scientific sessions on Washington D.C. Both confirmed and extended results from earlier trials using drug-coated stents. That is, with drug-coated stents the risk of restenosis (the largest remaining problem with the use of stents in coronary arteries) appears to be dramatically reduced.
The first of the two trials, the SIRIUS trial, examined the use of the sirolimus-coated stent, from Cordis and Johnson & Johnson. Previous trials with the sirolimus-coated stent suggested a remarkable reduction in restenosis compared to using "bare" metal stents.
However, the earlier trials were largely limited to patients whose coronary artery blockages were considered nearly ideal for the use of stents. In the SIRIUS trial, in contrast, patients were intentionally enrolled whose blockages were considered high-risk. Despite this higher risk population of patients, the SIRIUS trial showed a pronounced reduction in the rate of restenosis among patients receiving the sirolimus-coated stents. Patients receiving the drug-coated stent had a 91% reduction in restenosis within the stent itself. The main endpoint of the study, however, was not restenosis but "target vessel failure" defined as cardiac death, heart attack, or the need for revascularization within 9 months of stent placement. The drug-coated stents reduced target vessel failure from 21% to 8.6%.
In the second trial, TAXUS II, results with a paclitaxel-coated stent from Boston Scientific were presented. Overall results were comparable to those achieved with the sirolimus-coated stents. (In TAXUX II, most patients did not have high-risk coronary artery blockages. Ongoing trials with the paclitaxel-coated stents are enrolling high-risk patients.)
In summary, at least two types of drug-coated stents continue to yield remarkable decreases in the rate of restenosis when compared to standard, bare-metal stents. It is increasingly likely that, once these stents are approved by the FDA for use in the United States, they will revolutionize the treatment of coronary artery disease.
While cardiologists are extremely anxious to get their hands on this remarkable technology, the cost of these new stents will stress the budgets of many hospitals and create new stress for insurers.