Hypertrophic Cardiomyopathy Treatment Center

1. What is Hypertrophic Cardiomyopathy?

Hypertrophic Cardiomyopathy (HCM) is a genetic disease, occurring in 1 in 500 individuals. Although more common than initially thought, it is hard to diagnose and may be quite subtle, and therefore most patients are unaware of any problem until they develop symptoms. Making it even more difficult to diagnose is the fact that symptoms may develop early, in the teenage years, or much later in life. And, despite being a genetic disease, many patients may not be aware of a family history of the disease. Unfortunately, there is no reliable genetic test that can make the diagnosis in all cases, leaving physicians to rely on their own specialized expertise and experience with the disease process. For this reason, diagnosis and management of HCM is increasingly being performed by specialized Centers of Excellence.

The basic problem in this disease is an abnormality of the heart muscle fibers themselves. The muscle is rendered inefficient, and as a result becomes larger and larger in an effort to compensate. The effect is a markedly thickened heart muscle. In some cases, the thickening is disproportionate, affecting the wall between the two ventricles (septum) more than the free walls. Such patients are prone to obstruction of blood flow out of the heart (left ventricular outflow tract obstruction or LVOT obstruction) and a leaking heart valve (mitral regurgitation or MR) and more severe symptoms. These latter patients are termed Hypertrophic Obstructive Cardiomyopathy (HOCM), to differentiate them from patients without LVOT obstruction.

2. Who gets HCM?

HCM is a genetic disease, transmitted predominantly in an autosomal dominant fashion. This means that if a parent has

the disease, then 50% of the children will carry the gene as well. However, even if a child gets the same gene, there is still no way of telling whether the child will develop thickening or symptoms. Thus, it may appear that the disease skips generations at times, when in fact it has not. Such children should be screened with an echocardiogram every 5 years to evaluate for the development of thickening. And, any symptoms of palpitations or lightheadedness, shortness of breath or chest pain, should be taken seriously.

3. What are the symptoms of HCM?

Patients with HCM may develop a variety of symptoms, including lightheadedness, passing out, shortness of breath, chest pain or palpitations (racing heart). Symptoms may occur on exertion, or even when the patient is not doing anything physical.

a. Lightheadedness or Passing Out

Patients with HCM are at increased risk of sudden death, at a rate of 1-2% per year. Because of the muscle abnormality, the heart is irritable and may trigger abnormal fast heart rhythms, which drop the blood pressure to dangerous levels that don’t support life. Lightheadedness or passing out is particularly worrisome, and should be evaluated thoroughly at a specialized center. Lightheadedness and passing out may also be related to LVOT obstruction. In these cases, medical therapy and other procedures may improve the symptoms.

b. Shortness of Breath

Patients with HCM can develop heart failure; that is, a backup of fluid in the lungs and legs. This occurs because the heart cannot pump out the requisite amount of blood. Moreover, some patients with HCM develop a leaky valve (mitral regurgitation or MR), compounding the shortness of breath. Finally, those with severe LVOT obstruction are particularly affected, and may not be able to walk even a few feet without shortness of breath.

c. Chest Pain

Because the muscle thickens, it requires more blood. Therefore, even if patients don’t have artery blockages (atherosclerosis) they may still get typical heart attack type chest pain (angina). Moreover, patients with HCM are not immune to typical coronary disease, and can develop heart blockages like others of similar age, especially if they have the typical risk factors of smoking, diabetes, high blood pressure, and high cholesterol.

d. Palpitations

HCM can cause a variety of arrhythmias, from simple “extra beats” to atrial or ventricular tachycardias and fibrillation. Those with palpitations may benefit from further testing.

4. How can you treat HCM?

Fortunately, most symptoms respond to lifestyle changes and appropriate medical therapy. However, in some cases, the doses are high, and may result in side effects at times. At Winthrop, your cardiologist will help find a regimen that agrees with you, while minimizing symptoms. For those who continue to have severe symptoms despite medication, there are other options, including pacemaker placement, alcohol septal ablation or surgical myectomy.

a. Lifestyle Modification

HCM symptoms can be improved by staying hydrated. This means drinking 3-5 tall glasses of water every day, and avoiding products that can cause dehydration, such as alcohol, caffeine and tea. In addition, patients should avoid strenuous aerobic exercise, which may trigger abnormal rhythms.

b. Medications

A variety of medications are available for HCM. The most commonly utilized are a class called beta-blockers. These medications help the heart relax, minimize obstruction to blood flow, and alleviate mitral regurgitation, thereby decreasing symptoms. Other medications, such as calcium-channel blockers and disopyramide may be utilized in certain patients. Finally, for patients with certain rhythm disorders and palpitations, anti-arrhythmic medications such as amiodarone and blood thinners such as coumadin may be options.

c. Permanent Pacemaker

In some patients, placement of a permanent pacemaker may improve symptoms. This device allows precise contraction sequence of the various chambers of the heart, and may minimize the outflow obstruction as well as improve the efficiency of the heart. In studies, permanent pacemaker seems to work particularly well in older patients.

d. Alcohol Septal Ablation

For those with severe symptoms, alcohol septal ablation may be performed to decrease the amount of obstruction (LVOT obstruction) within the heart. This procedure, performed by an interventional cardiologist, was first described over 10 years ago and is performed in the cardiac catheterization laboratory. Under mild sedation, a small amount of alcohol is injected into the small area of heart muscle causing the obstruction. Almost immediately, there is a reduction in obstruction in properly-selected patients, and continued improvement of symptoms over the ensuing 2 years, at which point it plateaus. Over 90% of patients show significant improvement in symptoms. Patients stay in the hospital 3-4 days for monitoring. The main risks of the procedure include a possibility of permanent pacemaker, and rare occurrences of stroke, heart attack or death. Older patients and those with other medical problems are particularly well-suited for this procedure.

e. Surgical Myectomy

Performed for over 40 years, surgical myectomy is a type of open-heart surgery whereby the thickened septum is removed physically in order to improve the obstruction to blood flow out of the heart (LVOT obstruction). As opposed to alcohol septal ablation, surgical myectomy is particularly well-suited for younger patients, and those with valve disease or other disease (such as coronary atherosclerosis), in whom surgery is required for valve replacement, repair or coronary artery bypass surgery. Appropriately selected patients have a dramatic improvement in symptoms that persists for many years, similar to alcohol septal ablation. Patients typically stay in the hospital for 5 days, and require rehabilitation for another 1-2 weeks. The main risks include stroke, heart attack, permanent pacemaker and death, in addition to risks of infection and bleeding associated with bypass and mechanical ventilation. However, as most patients with HCM are young and otherwise healthy, these risks are typically much less than one would expect in patients who undergo coronary bypass surgery (CABG).

f. Implantable Defibrillator (ICD)

In patients deemed to be at higher risk for sudden death, an ICD may be offered. This device is placed under the left shoulder and is similar to a permanent pacemaker. In fact, the ICD can function as a pacemaker, but also functions as a mini “shock box” that constantly monitors your heart rhythm and determines whether there is a lethal arrhythmia. Such arrhythmias are shocked back to normal immediately, thereby saving the patient’s life. In order to determine your need for an ICD, your Winthrop cardiologist may schedule certain tests, such as a Holter monitor or exercise stress test (treadmill test).

5. External Links

a. Hypertrophic Cardiomyopathy Association (HCMA)

6. Testimonials and Press Releases

7. Hypertrophic Cardiomyopathy Team

Srihari S. Naidu, MD, FACC, FSCAI

Director, Hypertrophic Cardiomyopathy Center
Director, Cardiac Catheterization Laboratory
Dr. Naidu specializes in initial consultation, diagnosis, management and long-term follow-up. In most instances, this will be performed alongside your general cardiologist, who will be kept in the loop regarding your care. Dr. Naidu also specializes in alcohol septal ablation for patients who qualify for the procedure. Dr. Naidu is a noted expert in HCM, lecturing across the country on the topic. He also runs the HCM Symposium, in joint-sponsorship with the Hypertrophic Cardiomyopathy Association (HCMA).

Todd Cohen, MD and Joseph Germano, DO

Director and Associate Director, Electrophysiology
Drs. Cohen and Germano specialize in complex arrhythmia management and consultation, including electrophysiologic studies and the placement of permanent pacemakers or implantable defibrillators (ICD). Dr. Cohen is Editor-in-Chief of EP Lab Digest and is the Section Editor for Electrophysiology of the Journal of Invasive Cardiology. His textbook, Practical Electrophysiology, was published in 2006.

Todd Kerwin, MD

Director of Nuclear Cardiology
Dr. Kerwin is a specialist in non-invasive cardiology and clinical cardiology, with noted expertise in the echocardiographic evaluation of patients with Hypertrophic Cardiomyopathy. In addition to patient consultation, he specializes in the echo assessments of invasive techniques such as alcohol septal ablation or surgical myectomy.

Scott Schubach, MD, William Kokotos, MD and John Goncalves, MD

Chief of Cardiothoracic Surgery, Director and Director of Aortic
Drs. Schubach, Kokotos and Goncalves comprise the surgical team evaluating patients for surgical myectomy. Dr. Schubach is well-known on Long Island as a leader in cardiothoracic surgery.

Russell Schiff, MD

Director, Pediatric Cardiology
Dr. Russell specializes in the assessment of sudden death risk in young athletes. He is a noted expert in this topic, and provides consultation for young patients with HCM.

Upcoming Events for Physicians

“Percutaneous Treatment of Structural Heart Disease Symposium”

Saturday, September 13th, 2008
Garden City Hotel, Garden City, Long Island
Registration: Seating is limited, RSVP to Anne Maloney at 516-663-2476.
Admission is Free to Physicians
Breakfast and Lunch Provided

CME Activity: 6 hours CME credit awarded (pending).

Speakers:

Jeffrey Moses, MD
Columbia University
Topic: Percutaneous Aortic Valve Replacement

Ziyad Hijazi , MD
Rush Medical Center
Topic: Aortic Coarctation and Other Adult Congenital Intervention

Howard C. Herrmann, MD
University of Pennsylvania
Topic: Percutaneous Mitral Valve Repair

Srihari S. Naidu, MD
Winthrop University Hospital
Topic: Alcohol Septal Ablation for HCM

Carlos Ruiz, MD
Lenox Hill Hospital
Topic: ASD/PFO Closure and Paravalvular Leaks

Recent Events for Patients and Families

Hypertrophic Cardiomyopathy Association National Meeting

May 31, 2008
Hyatt Morristown, Morristown, NJ

Alcohol Septal Ablation: When is it the right answer?

Speaker: Srihari S. Naidu, MD

Hypertrophic Cardiomyopathy Symposium 2007

Saturday, November 10, 2007
Carltun on the Park, Eisenhower Park, Long Island, NY

“What is HCM and where are we now?”

Barry Maron, MD
Director, HCM Center, Minneapolis Heart Institute

“Approaches to Treatment”

Srihari S. Naidu, MD
Director, HCM Center, Winthrop-University Hospital

“Management of Arrhythmias”

Joseph Germano DO,
Associate Director, Electrophysiology, Winthrop-University Hospital

“Role of the HCMA”

Lisa Salberg
President, Hypertrophic Cardiomyopathy Association (HCMA)

Sponsored by Winthrop-University Hospital and the Hypertrophic Cardiomyopathy Association (HCMA)

Recent Events for Professionals

Advances in Cardiac and Endovascular Therapies (ACE Conference)

Sunday, October 7, 2007
Grand Hyatt Hotel, New York, NY

“Alcohol Ablation for Hypertrophic Cardiomyopathy”

Srihari S. Naidu, MD
Director, HCM Center, Winthrop University Hospital

Sponsor: Lenox Hill Hospital

Abbott Vascular Interventional Fellows Course

Saturday, September 8, 2007
Conrad Hilton Hotel, Miami, FL

“HOCM: Hemodynamics and Ablation Techniques”

Srihari S. Naidu, MD, Director, HCM Center, Winthrop University Hospital

Sponsored by Abbott Vascular

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