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Hypertrophic Cardiomyopathy Service

Hypertrophic cardiomyopathy (often called HCM) is a condition associated with the abnormal thickening of a portion or the entire heart muscle. It often affects young individuals and may not cause many symptoms but could result in sudden death.

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Persons with HCM have unique needs and require specialized care over a wide range of medical expertise targeted towards relieving symptoms, preventing complications, and exploring surgical options.

The recognition of the need for a specialized center to provide these services in a patient friendly, well-coordinated environment has led to the launch of the Johns Hopkins Hypertrophic Cardiomyopathy Clinic.

Mission

The overall goal of this clinic is to offer patients with HCM and their families access to high quality clinical care supported by state of the art technology and research with a view to alleviate symptoms, prolong life and enhance the understanding of HCM, in a single center setting.

We hope to achieve this goal by building a team dedicated to HCM care consisting of master clinicians, experts in multi-modality imaging, skilled interventional and surgical staff, specialists in genetic medicine and innovative researchers.

The keystone to this program remains cutting edge, individualized patient diagnosis and treatment provided by the faculty in conjunction with our nurse practitioners. Our patients range from those who are asymptomatic to those in desperate need of cardiac transplantation.

Johns Hopkins offers a new innovative procedure known as Surgical Ventricular Restoration (SVR). SVR is a surgical procedure to treat congestive heart failure caused by myocardial infarction. Following a heart attack, scar or an aneurysm may develop resulting in an enlarged rounded heart that may lead to congestive heart failure (CHF). The goal of the SVR is to restore the heart to a more normal size and shape, therefore improving function. We have been on the leading edge in the refinement of this novel procedure.

The clinic staff hopes to improve the quality of life in HCM patients, provide education about the disease and to support research into better diagnosis techniques and treatments for HCM.

Specific facilities available through the JHHCC include access to a clinic staffed by specialists in:

All patients and relatives are also offered voluntary enrollment in a variety of ongoing research projects.

Ambulatory Clinic:

  • Evaluation of new and returning HCM patients
  • Coordinate testing, risk assessment and initiation of medical therapy
  • Assist patients and their referring physicians make decisions regarding interventional and surgical options for HCM
  • Screen family members of HCM patients

Echocardiography/Non-invasive Hemodynamics:

  • Standard transthoracic echocardiography
  • Transesophageal echocardiography
  • Exercise echocardiography
  • Detailed hemodynamic assessment at rest and during provocation (Valsalva, Amyl Nitrite or exercise)
  • Tissue Doppler and strain echocardiography to evaluate regional and global myocardial mechanics

Magnetic Resonance Imaging and Spectroscopy:

  • Detailed morphologic and functional imaging via standard, tagged and contrast enhanced MR imaging
  • Evaluation of novel disease markers by MR spectroscopy

Computed Tomography:

  • Assessment of coronary artery anatomy and cardiac morphology

Electrophysiology/Sudden Death Risk Assessment:

  • Evaluate risk for sudden cardiac death and need for defibrillator implantation
  • Implantation of defibrillators and pacemakers

Hemodynamics/Cardiac Mechanics:

  • Invasive hemodynamic evaluation

Interventional Cardiology:

  • Diagnostic coronary angiography
  • Percutaneous alcohol septal ablation for obstructive HCM

Cardiac Surgery:

  • Surgical myectomy for obstructive HCM
  • Valve repair

Medical Genetics:

  • Genotyping
  • Genetic counseling

Pediatrics:

  • Evaluation of children with known or suspected HCM
 

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