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Winthrop University Hospital

Services of the HCM Center

Cardiac Imaging & Non-Invasive Testing

Noninvasive cardiac imaging is key to detecting HCM. In addition, imaging assists in understanding the pathophysiology in a given patient and selecting — as well as guiding — therapy. Today’s powerful noninvasive cardiac imaging techniques provide detailed views of the heart’s anatomy with unprecedented clarity and speed. Winthrop’s cardiac imaging experts use these sophisticated technologies to help diagnose the disease — especially when outside studies are equivocal — leading to timely and appropriate treatment.

Transthoracic Echocardiography (TTE)

Echocardiography is one of the most important tests used to detect HCM. Under the direction of Jerald L. Cohen, MD, Winthrop’s Echocardiography Laboratory employs noninvasive ultrasound technology to produce accurate assessments of the size of the heart, pattern of ventricular hypertrophy, contractile function — including diastolic dysfunction — evidence for diastolic dysfunction at rest or with provocation, and associated mitral regurgitation and pulmonary hypertension. Abnormal diastolic performance, as assessed by tissue-Doppler echocardiography, is typically seen in patients even when diastolic dysfunction is not present, and is a cause of disabling symptoms in some patients. Additionally, 3D-Echo is now utilized to more fully characterize subtle anatomic abnormalities, especially when cardiac MRI is not possible.

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 supra-valvular or sub-valvular obstructiontransesophageal echocardiogram is vital to ensure that the patient is properly treated. According to George Gubernikoff, MD, Director of Winthrop’s Center for Aortic Diseases, TEE can identify LVOT obstruction versus other forms of obstruction, significantly changing the patient’s treatment options. Patients whose obstruction is due to abnormal papillary muscles or membranes, for example, require very precise surgical correction, guided specifically by the findings of TEE.

Cardiovascular MRI (Cardiac MRI)

Coupling the basic principles of magnetic resonance imaging (MRI) with electrocardiography gating and rapid imaging sequences, cardiac MRI can assess key functional and structural features of the cardiovascular system. According to Juan Gaztanaga, MD, Winthrop’s Director of Cardiac MRI/CT, the technology — which displays the complete anatomy of the heart via high-resolution images — is the gold standard for evaluating diseases such as HCM. In addition, the gadolinium (contrast) enhanced images can detect intramyocardial scarring, a marker of significant disease, as well as the potential for arrhythmias that cause sudden cardiac death. Cardiac MRI is also exceptionally important when visualizing rare forms of the disease, such as apical HCM, or to elucidate the presence or absence of thrombus in patients with end-stage “burnt-out” HCM.

Holter Monitoring

When HCM patients present with palpitations, a Holter monitor may be utilized to assess for arrhythmias during a 24-hour period or longer. If arrhythmias are found, specific medications or electrophysiology ablations may be used to control them. In addition, Holter monitoring is important to the initial and annual assessment of a patient’s risk for sudden cardiac death.

Stress Testing

Since patients with HCM often have symptoms of heart failure, as well as angina, stress testing is useful to determine the patient’s level of disability (how far they can walk before symptoms develop). Additionally, stress testing may help find atherosclerosis in some patients. It is also used as part of the annual sudden cardiac death risk stratification protocol to determine which patients may benefit from internal cardiovertor defibrillator placement.

Electrophysiology

Winthrop’s electrophysiology team, which works in the Hospital’s state-of-the-art Electrophysiology Laboratory and Pacemaker/Arrhythmia Center, is led by Todd Cohen, MD, Director of Electrophysiology, and Associate Director Joseph Germano, DO. They specialize in diagnosing and treating heart-rhythm irregularities. At the forefront of the field for decades, the team has pioneered several ground-breaking procedures on Long Island, including the implantation of the smallest internal cardioverter defibrillator (ICD) and the first MRI-safe ICD.

Electrophysiology (EP) Studies & Pacemakers

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. Patients in the HCM Center undergo periodic observation with Holter and event monitors to watch for dangerous arrhythmias, and physicians may utilize complex, yet minimally-invasive, EP studies to induce such arrhythmias in order to guide treatment. In particular, patients with atrial fibrillation and other complex supraventricular tachycardias may benefit from radiofrequency ablation techniques to prevent recurrence. In addition, some patients may benefit from permanent pacemaker implantation.

Internal Cardioverter Defibrillators (ICDs)

Used to treat very rapid heart rhythms that would otherwise cause the patient to collapse or die, 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 in the HCM Center undergo a yearly assessment of their risk for sudden death, triggering ICD implantation in those at highest risk.

Genetic Testing/Counseling

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.

Winthrop’s 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, can lead to identification of at-risk family members, who are currently asymptomatic and may have normal echocardiograms. Those who test positive for the familial mutation receive regular echocardiograms and counseling from genetic counselor Monika Zak, MS, who helps patients understand the implications of their test results. Those who test negative may avoid further testing for this disease for both themselves and their heirs.

Treatment

Fortunately, most HCM symptoms respond to lifestyle changes and appropriate medical therapy. However, in some cases, medication can cause side effects or do not fully address the symptoms. At Winthrop, the patient’s cardiologist helps find a regimen that agrees with the patient, while minimizing symptoms. For those who continue to have severe symptoms despite medication, other options are available. The range of treatment choices include:

Lifestyle Modification

HCM symptoms can be improved with hydration, which means drinking three-to-five tall glasses of water daily and avoiding products that can cause dehydration, such as alcohol, caffeine and tea. Additionally, patients should avoid strenuous or competitive aerobic exercise, which may trigger abnormal heart rhythms. However, regular, non-competitive (recreational) activities are encouraged for general health.

Medical & Pharmacologic Therapy

A variety of medications are available to HCM patients. The most commonly used — beta blockers — help the heart relax, minimize obstruction to blood flow and alleviate mitral regurgitation, thereby reducing symptom severity. Other medications, such as calcium-channel blockers and disopyramide, may be used in certain patients. Finally, for patients with certain heart-rhythm disorders and palpitations, anti-arrhythmic medications, such as amiodarone, and blood thinners, such as Coumadin or Dabigatran, may be options.

Permanent Pacemaker Implantation

For some patients the placement of a permanent pacemaker may improve symptoms. This device allows precise contraction sequence of the heart’s chambers and may minimize LVOT obstruction, as well as improve the heart’s efficiency. In some patients, pacemakers may allow the individual to tolerate higher doses of medications, which in turn help to control symptoms. Permanent pacemakers seem to work well in older patients (those over age 65).

Internal Cardioverter Defibrillator (ICD) Implantation

In patients deemed at high risk for sudden cardiac death, an ICD may be offered. The 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 heart rhythm and determines whether or not there is a lethal arrhythmia. Such arrhythmias are shocked back to normal immediately, thereby saving the patient’s life.

Alcohol Septal Ablation (ASA)

For patients with severe symptoms, alcohol septal ablation (ASA) may be used to decrease the amount of LVOT obstruction within the heart.

Alcohol septal ablation (ASA) is a less invasive alternative to surgical myectomy. Offered at only a few hospitals nationwide, it is used for patients who qualify anatomically and clinically. At Winthrop, ASA is conducted in the Cardiac Catheterization Laboratory by Srihari S. Naidu, MD, Director of the Lab and the Hospital’s HCM Center.

ASA involves injecting a small amount of alcohol directly into the bulge of the abnormally thickened septum that is narrowing the ventricle. The alcohol destroys the excessive cells on contact, debulking the septum and widening the outflow tract over the ensuing three-to-six months. It leads to a reduction in symptoms comparable to surgical myectomy. Older patients and those with other medical problems are particularly well suited for ASA.

Winthrop’s HCM Center, which performs the most alcohol septal ablations annually in New York State, New Jersey or Connecticut, is recognized as a national leader in the technique, with its staff teaching other physicians throughout the country.

Surgical Myectomy & Complex Valve Repair

Long the gold standard for treatment of HCM, surgical myectomy entails removing a portion of the septum that is obstructing blood flow from the left ventricle. It is performed only at specialized centers of excellence by cardiothoracic surgeons experienced and skilled in the delicate operation.

The thickened muscle is approached via the aortic valve without incising the heart muscle. Once the thickness of the septum is reduced, the left ventricle enlarges, 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 structures have been removed, and the mitral and aortic valves are working properly, predicting long-term success.

Surgical myectomy is particularly useful in younger patients and for those with complex anatomic pathology.

At Winthrop, cardiothoracic surgeons Scott Schubach, MD, John Goncalves, MD, and William Kokotos, MD, are known for their skill in open-heart surgery as well as surgical myectomy.

Pediatric Specialized Care

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.

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.

At Winthrop, pediatric cardiologists Carlos Montoya, MD, Donna Better, MD and Tasneem Hoque, MD, prescribe beta blockers and calcium-channel blockers when indicated, and recommend the placement of an ICD for secondary prevention after an episode of resuscitated sudden death, or for patients with multiple risk factors. surgical myectomy is generally reserved for children who have failed medical therapy and remain severely symptomatic. alcohol septal ablation (ASA) is not performed on children.

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