Welcome to the Hypertrophic Cardiomyopathy (HCM) Program at Westchester Medical Center, the flagship of the WMCHealth Network. Our program is a fully comprehensive, patient-centered experience, and the only one based in Westchester County with a full service satellite office in Long Island. Under the leadership of Srihari S. Naidu, MD, our program is devoted to treating patients and families from the tri-state area and beyond, offering expert, specialized services that address all facets of the disease. The two primary tenets of our program are its one-stop-shop approach to patient care, without the need to refer patients elsewhere for any service, and its goal of providing patients and referring physicians with 24/7 access through two dedicated HCM coordinators, a team of expert HCM physicians, and dedicated HCM email addresses and phone numbers.
Dr. Naidu initially established the program in 2004 around his reputation for performing the minimally invasive alcohol septal ablation (ASA), an alternative to surgical myectomy in patients with obstruction to left ventricular outflow. The program is now known for serving a large volume of patients from throughout the United States, with patients also traveling from other countries, and for its ability to perform all services from genetics to pediatrics, alcohol septal ablation to surgery, echocardiography to cardiac MRI, and electrophysiology testing to heart transplantation by physicians expert in and dedicated to the care of patients with HCM.
Dr. Naidu is an internationally-known expert in the treatment of HCM, and is involved in national clinical trials of new drugs, publishes the international textbook Hypertrophic Cardiomyopathy, and co-runs the live alcohol septal ablation proctoring course annually in Detroit.
As a co-author on the 2011 American Heart Association Guidelines on the Diagnosis and Management of HCM, he writes and lectures extensively to contribute knowledge to the medical community, and runs annual patient and physician oriented conferences to educate those living with or treating this disease.
The WMC HCM Program is one of a handful nationally with all the necessary diagnostic and therapeutic options available for both adults and children, all under one roof. Coordinated by regularly-scheduled HCM Program meetings, the following services comprise a team of experts that help comprehensively manage each individual patient and family.
To contact the program for a consultation at one of our two offices, or for referrals or other clinical issues, please use our dedicated HCM email address and phone number, available 24/7:
Email: HCMProgram@WMChealth.org
Call or Text: 914.598.7651. We look forward to working with you.
Office Locations
Westchester Medical Center
Ambulatory Care Pavilion
100 Woods Road
Valhalla, NY 10595
877 Stewart Avenue
Suite 12
Garden City, NY 11530
Hypertrophic cardiomyopathy (HCM) is an
inherited disease that may appear without an
obvious family history, making the diagnosis
often difficult – patients may have lived with
the disease for years and decades prior to a
diagnosis being made. Although previously
thought of as rare, we now know it affects 1
in 500 individuals across all age groups, both
genders and every race, and is transmitted to
half of all family members. As such, one half
of all offspring of a known HCM patient will
inherit the gene, although when and how the
disease presents is highly variable, even
within a single family.
Characterized by myocardial disarray that creates
physical and electrical abnormalities in the
heart muscle, HCM involves excessive thickening
of the inter-ventricular septum or other
areas, increased left ventricular stiffness, as
well as left ventricular outflow tract obstruction
and mitral valve regurgitation in the majority of
symptomatic patients. Most threatening, however,
is the risk of sudden cardiac death, making
proper diagnosis and treatment important in
patients and affected family members. In general,
younger patients are predominantly at risk
of sudden death, while older patients typically
suffer from progressive heart failure symptoms,
which impact both quality and quantity of life.
The clinical spectrum of disease presentation
is diverse, ranging from asymptomatic individuals
to those with disabling symptoms of heart
failure, exercise intolerance, shortness of breath,
chest pain and lightheadedness or syncope.
HCM is also the most common cause of sudden
cardiac death in individuals under 30 who
appear otherwise healthy, especially athletes.
A conclusive diagnosis, which may include
genetic testing and advanced imaging, is vital
to the prognosis and determination of treatment
options.
Because of the difficulties in diagnosis, which
often requires advanced imaging and invasive
testing in some, and the variety of complex
management options, the American Heart Association
recommends that all patients and families
with this disease be seen at dedicated HCM
centers, with the full complement of services
needed to optimally treat these patients.
While diagnosis traditionally involves an echocardiogram,
the subtleties of the disease manifestations
may be difficult to discern, requiring
comprehensive focused echocardiographic
evaluations using multiple views. When echocardiogram
is inconclusive or suspicious for severe
thickening or apical variant, cardiac MRI may be
required. Other diagnostic testing may include
periodic Holter monitoring and stress testing, as
well as transesophageal echocardiography and
internal loop recorders in some.
Although there is no cure, most symptomatic
patients can be managed with appropriate
education and lifestyle modification; medical
therapy, including beta-blockers, calcium
antagonists, anti-arrhythmic drugs and anticoagulants;
pacemaker therapy; and the implantation
of an internal cardioverter defibrillator to
prevent sudden cardiac death. Diet, nutrition
and psychiatric services are oftentimes needed
to help manage the disease’s effects on other
aspects of physical and mental health. Children
with HCM require dedicated pediatric services,
including advanced imaging, electrophysiology
devices and surgery in some.
When symptoms persist despite the above
measures, surgical myectomy or catheter-based
alcohol septal ablation is used to reduce the
size of the thickened septum and enlarge the
ventricular outflow tract, eliminating obstruction
and mitral regurgitation while improving
diastolic function. And for the rare patient who is
not a candidate for these advanced therapies, or
whose symptoms persist or progress despite all
other options, heart transplantation may remain
the only viable solution.
Most important, HCM is a family disease, and
it is not enough to simply treat the patients. All
blood relatives must be evaluated by genetic
testing or echocardiogram – the latter at routine
intervals, regardless of initial results – and all
children should be evaluated by pediatric cardiologists
specializing in the disease. Education
and longitudinal care, where strong doctor-patient
relationships are formed, help families
understand and cope with the disease.
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HCM Services
Initial HCM Consultation
Initial office consultations, as well as follow-up evaluations, are performed at both the Westchester and Long Island offices, and patients are all seen within 1-2 weeks of initially contacting the program whenever possible. According to Srihari S. Naidu, MD, Director of the HCM Program, office consultations serve to educate the patient and family on HCM in general, and then hone in on the way HCM is affecting each individual patient – indeed, HCM is different in each person, and understanding the unique aspects of a patient’s disease is key to choosing therapies that work. This will involve echocardiography in all patients, usually on the same day as the initial visit, as well as cardiac MRI in most patients. Once the disease is better understood, lifestyle modifications, diet, exercise, and medications are utilized and discussed to manage the disease and the patient’s symptoms. Other testing, such as Holter monitors, stress tests and both external and internal loop records, may be needed to help understand symptoms or to further stratify risk of sudden cardiac arrest. Finally, a discussion of genetics, gene testing and family counseling rounds out the consultation.
In general, the first visit will focus on understanding a patient’s unique presentation, while subsequent visits will assess sudden cardiac death risk and the extent of family involvement. At office consultations, Dr. Naidu is joined by at least one HCM Coordinator. Together, they will get to know each patient, so that a strong connection is formed between the patient and the HCM program.
Patients Traveling from Afar
The HCM Program takes pride in coordinating care for patients visiting
from distant states and foreign countries. After an initial phone or email
communication with Dr. Naidu, our coordinators will work with the patient
and referring physician on insurance authorization, obtaining outside
records, travel arrangements, and sequential rapid testing on the day of
the visit, including electrophysiologic monitoring, stress testing, echocardiography
and cardiac MRI, followed by the comprehensive office consultation.
According to Mallory O’Shea, LPN, HCM Coordinator, patients will
be able to take all records back with them at the end of the day, including
the consult note. Follow-up arrangements between their local cardiologist
and the HCM program will be arranged so that the plan can be carried out.
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Transthoracic Echocardiography (Echo)
Echocardiography is one of the most important tests used to detect HCM.
Under the direction of Tanya Dutta, MD, the Echocardiography laboratory
employs non-invasive ultrasound technology to produce accurate assessments
of the size of the heart, pattern of ventricular hypertrophy, contractile
function – including diastolic dysfunction - evidence for outflow tract
obstruction at rest or with provocation, and associated mitral regurgitation.
Abnormal diastolic performance, as assessed by tissue-Doppler echocardiography,
is typically seen in patients even when outflow tract obstruction
is not present and is a cause of disabling symptoms in some patients.
In addition, 3D-Echo and contrast (Definity) is now utilized to more fully
characterize subtle anatomic abnormalities and follow wall thickness over
time, especially when cardiac MRI is not possible. Working with Angelica
Poniros, RCS, Clinical Manager of Noninvasive Services, we have developed
a dedicated HCM protocol to provide consistent, high-quality and
accurate measurements of HCM in our patients.
Transesophageal Echocardiography
(TEE)
In patients with complex sub-valvular anatomy
– including possible disease of the mitral valve,
papillary muscles, or chordal apparatus – and
in patients with possible supra-valvular or
sub-valvular obstruction, transesophageal echocardiogram
is vital to ensure that the patient is
properly treated. According to Tanya Dutta, MD,
TEE can separate left ventricular outflow tract
obstruction due to asymmetric hypertrophy from
other forms of obstruction, resulting in significant
changes to the patient’s treatment options.
TEE also provides thorough evaluation of the
aortic and mitral valves, helping determine the
appropriate treatment for patients with HCM
needing invasive therapies.
Cardiovascular Magnetic Resonance
Imaging (Cardiac MRI)
Coupling the basic principles of magnetic resonance
imaging with electrocardiography gating
and rapid sequences, cardiac MRI can assess
key functional and structural features of the
cardiovascular system. Under the direction of
Anthon Fuisz, MD, Director of cardiac MRI, the
technology – which displays the complete anatomy
of the heart via high-resolution images – is
the “gold standard” for evaluating diseases with
regional heterogeneity, such as HCM. In addition,
the gadolinium (contrast) enhanced images can
detect intramyocardial scarring, a marker of
significant disease, and the potential for arrhythmias
that cause sudden cardiac death. Cardiac
MRI is also exceptionally important when
visualizing rare forms of the disease, such as
an apical HCM, or to elucidate the presence or
absence of thrombus in patients with end-stage
“burnt out” HCM. Finally, MRI can often help
better understand cardiac function, as well as
rule out other mimickers of HCM – “look-alikes”
that require different treatments.
Exercise Stress Treadmill Test (ETT)
Functional status is often objectively tested
using a standard treadmill test. According to
Noemi Mercado, PA, who supervises these
procedures, ETT is used to both determine a
patient’s functional status and to determine
their risk of sudden cardiac arrest on an annual
basis. Serial ETTs can be used to evaluate for
worsening function, and can guide the timing
of invasive therapies such as alcohol septal
ablation or surgical myectomy. In some patients,
the ETT is combined with echocardiography
to evaluate for the presence and severity of
left ventricular outflow tract obstruction, and
to determine response to invasive therapies or
medications.
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Electrophysiology (EP) Studies
Sudden cardiac death is the most devastating consequence of HCM.
Therefore, it is vital to determine whether an individual with HCM is at risk
for dangerous ventricular tachycardia or fibrillation. According to Sei Iwai,
MD, Director of Electrophysiology, patients in the HCM Center undergo
periodic observation with Holter and Event Monitors to watch for dangerous
arrhythmias, or long-term internal loop recorders that last 3 years. Although
not needed in most patients, some HCM patients benefit from electrophysiologic
studies to evaluate for ventricular tachycardia, supraventricular
arrhythmias or conduction disease.
Atrial Fibrillation Management
Patients with atrial fibrillation often develop worsening heart failure
symptoms and palpitations, and are at increased risk of stroke. In addition
to blood thinners, patients may benefit from complex atrial fibrillation
ablation, a minimally-invasive procedure. Patients at high risk of both
stroke and bleeding may benefit from implantation of a left atrial appendage
occlusion device. According to Sei Iwai, MD,
Co-Director of the LAA Closure Program, such devices avoid the need
for long-term anticoagulation.
Pacemakers
Pacemakers may be required when medications cause low heart rates, or when
surgical myectomy or alcohol septal ablation cause conduction disease. In addition,
older patients may benefit from pacemaker placement prior to contemplating
more invasive procedures, as pacemakers have been shown to reduce outflow
track gradients by themselves in a portion of patients. More recent advances
include the leadless pacemaker, which may avoid long term infection risk.
Internal Cardioverter Defibrillators (ICDs)
Used to treat very rapid heart rhythms that would otherwise cause the
patient to pass out or arrest, ICDs are tiny devices, implanted permanently
under the chest wall. The ICD monitors the heart’s rhythm, senses potentially
lethal arrhythmias and either paces the rhythm back to normal or,
if that fails, delivers a mild electric shock to restore normal heart rhythm.
Patients with HCM undergo a yearly assessment of their risk for sudden
death, triggering ICD implantation in those at highest risk. According to
Jason Jacobson, MD, Director of Complex Arrhythmia Ablation, subcutaneous
ICDs are also available and may be an option in young patients who
wish to avoid long term potential complications of ICDs.
Ventricular Tachycardia (VT)
Ablation
Given the association with sudden cardiac death, some patients may
be candidates for complex ventricular tachycardia ablation. According
to Dr. Jacobson, patients with HCM may have severe arrhythmias despite
medications, or that cause repeated ICD shocks, prompting ablation.
In these cases, patients may benefit from ventricular assist devices
to make the procedure safer.
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Genetic Studies
Genetic studies have identified HCM as a
disease of the sarcomere, caused by mutations
in any of 17 different genes. To date, over 500
individual mutations have been identified. Identification
of genes for HCM has made preclinical
diagnosis possible in families with a known
mutation. Our extensive genetic testing and
counseling services are available to symptomatic
HCM patients and asymptomatic individuals
with a known family history. Genetic testing,
which typically takes between two-to-six weeks
through commercial companies, can lead to
identification of at-risk-family members, who
are currently asymptomatic and may have normal
echocardiograms. Directed by David Kronn,
MD, Geneticist, and Nandini Chandy, CGC, HCM
Genetics Counselor, if a gene test is positive, the
test can be used to determine if others in the
family are at risk or not at risk of ever developing
the disease, effectively reassuring half
the family. Pedigree analysis and whole exome
sequencing is also possible, according to John
Fallon, MD, Chief of Pathology with expertise in
cardiac pathology and cardiogenetics.
Mental Health Services
Patients with HCM may suffer from anxiety
or depression, a normal result of living with a
chronic disease that may affect their lifestyle or
risk of sudden death. Dedicated mental health
services, including assessment and treatment,
are available through assigned mental health
experts with interest in HCM. Stephen Ferrando,
MD, Chairman of Psychiatry, joins quarterly
meetings and provides mental health consultations
as needed or requested by patients.
Diet and Nutrition Services
Diet and nutrition services are important to
maintaining an ideal weight, so as to improve
a patient’s quality of life and ability to live with
their HCM. In addition, as HCM symptoms are
susceptible to changes in fluid status and salt,
diet services aid in reducing a patient’s risk of
developing congestive heart failure or dizziness,
or even syncope.
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Cardiac Catheterization
For patients with significant symptoms of heart
failure, chest pain or dizziness, especially when
medications fail to adequately control symptoms,
cardiac catheterization can aid in the
assessment of heart failure and to rule out other
diseases that may cause similar symptoms.
Srihari S. Naidu, MD, Director of the HCM
Program, performs cardiac catheterization
to evaluate both the lungs and the heart, and
oftentimes uses the information to change
management while the patient is still on the
table. In some cases, cardiac catheterization
will lead to alcohol septal ablation or surgical
myectomy, but oftentimes it leads to modification
of medications to improve symptoms.
Septal Myectomy
Septal myectomy is a complex surgical procedure
used when medication cannot relieve
symptoms in patients with obstructive HCM. Our
cardiothoracic surgeons Steven Lansman, MD,
David Spielvogel, MD and Ramin Malekan, MD,
use the technique to remove the portion of the
septum obstructing the blood flow from the left
ventricle. The thickened muscle is approached
via the aortic valve. Once the thickness of the
septum is reduced, the left ventricle outflow
tract widens, filling pressure is reduced, and
blood flow improves, dramatically alleviating
symptoms. If mitral valve regurgitation persists
due to intrinsic abnormality, the valve is
repaired at the same time. Before leaving the
operating room, a transesophageal echocardiogram
confirms that all obstructing myocardium
structures have been removed, and the mitral
and aortic valves are working properly, predicting
long-term success. Long the gold standard
for treatment, surgical myectomy is particularly
useful in younger patients and those with severe
thickening.
Alcohol Septal Ablation (ASA)
Alcohol septal ablation (ASA), a less invasive
alternative to septal myectomy for patients who
qualify anatomically and clinically, is offered only
at a few hospitals nationwide. ASA is conducted
in the Cardiac Catheterization Laboratory by Dr.
Naidu. ASA involves injection of a small amount
of alcohol directly into the bulge of the abnormally
thickened septum and widening the outflow
tract over the ensuing three-to-six months,
leading to reduction in symptoms comparable to
surgical myectomy. Dr. Naidu is the most experienced
alcohol septal ablation operator in NY, NJ
or CT, and is widely regarded as a leader in the
technique. Alcohol septal ablation is preferred in
older patients, those at higher risk for surgical
myectomy, and patients with a strong desire
to avoid open heart surgery after a balanced
discussion.
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HCM in children and adolescents is a significant cardiac condition with
serious concomitant health issues and a sometimes profound risk for premature
death, especially among infants and competitive athletes. Frequently,
children with HCM are asymptomatic, remaining undiagnosed for many
years. For those suspected of suffering from the disease, due to family
history or clinical symptoms, early diagnosis is critical. Diagnostic studies
used for pediatric patients include echocardiograms, ECGs, cardiopulmonary
testing and genetic screening. Symptomatic children typically present
with chest pain and dyspnea. Lethargy, syncope, arrhythmias and palpitations
are also common. Our pediatric cardiologist Christa Miliaresis, MD,
works out of the Maria Fareri Children’s Hospital, physically connected to
Westchester Heart and Vascular, and has extensive experience in sudden
death in athletes and screening for cardiac conditions in pediatric patients.
She prescribes beta blockers and calcium channel blockers when indicated,
and recommends the placement of an ICD for secondary prevention
after an episode of resuscitated sudden death or for patients with multiple
risk factors. She is joined by Drs. Irfan Warsy, Supriya Jain, and
Marise D’Souza, with expertise in electrophysiology, cardiac MRI and
echocardiography in the pediatric population. Surgical myectomy is
generally reserved for children who have failed medical therapy and
remain severely symptomatic. Alcohol septal ablation is generally not
performed on children.
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Although rare, patients who fail
to respond to surgical myectomy
or alcohol septal ablation, those
who are not candidates for
invasive therapies, those with
non-obstructive HCM, and those
with “burnt-out” end-stage HCM
may benefit from heart transplantation.
Alan Gass, MD, Medical
Director of Heart Failure and
Cardiac Transplantation, is an
expert in the management of
severe heart failure. He evaluates
patients both before and after
heart transplantation. As one of
4 heart transplant programs
around New York City, Westchester’s
program is one of the busiest
programs in the region. Cardiopulmonary
exercise testing is used to
stratify the heart failure state in
each patient. In some patients,
ventricular assist devices, either
temporary or permanent may be
required to stabilize the patient
prior to heart transplantation.
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Patient interaction may be helpful
in sharing stories and, in particular,
ways of coping with this chronic
disease. According to Rosemary
Conlon, RN, Patient Liaison, patients
of all ages participate in the
patient discussion group, and help
coordinate patient interaction and
networking. Patients participate
in the annual HCM symposium
as panelists, and help coordinate
community events and fundraisers.
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