|
Cardiology
Physicians
Centers of Excellence
Hypertrophic Cardiomyopathy Treatment Center
The Women’s Cardiovascular Health and Wellness Program
Atrial Fibrillation Treatment Center
Thoracic Aortic Vascular Treatment Center
Heart Failure Treatment Center
Premier Services
General Cardiology
Interventional Cardiology
Electrophysiology
Nuclear Cardiology
Echocardiography
Cardiac Rehabilitation
From the Desk of Kevin Marzo, MD-Chief Of Cardiology
Contact Us
Hypertrophic Cardiomyopathy Center
Srihari S. Naidu, MD, Director
Mee Fong Ng, Coordinator
259 First Street
Mineola, NY 11501
1-516-663-0333
1-866-WINTHROP
Click on links below to learn more
What is Hypertrophic Cardiomyopathy?
Who gets HCM?
What are the symptoms of HCM?
How can you treat HCM?
External Links
Testimonials and Press Releases
Hypertrophic Cardiomyopathy Team
Recent Events for Patients and Families
Recent Events for Professionals
|
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
- Letter from a Patient
- Renowned Cardiologist Dr. Srihari Naidu Joins Winthrop-University Hospital
- New Interventional Cardiology Procedure Now Available at Winthrop – Doctor Closes ASD/PFO Hole In Stroke Patient’s Heart
- Winthrop Cardiologist Uses New, Minimally Invasive Procedure – Alcohol Septal Ablation - to Treat Life-Threatening Heart Condition
- Winthrop Hosts the Area’s First Patient-Focused Hypertrophic Cardiomyopathy Symposium
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.
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.
Recent
Events for Patients and Families
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
|
