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eRounds _ Spring 2014

Taking the Puzzle Out of Pericarditis: Using an Imaging Guided Approach

Pericarditis can be tricky to diagnose. Inflammation of the sac around the heart causes symptoms that often resemble other cardiac and pulmonary diseases. Imaging modalities from chest X-ray to MRI can help confirm a diagnosis. But accurate, evidence-based guidelines for pericardial disease have not been available. Until now.

A new expert consensus statement, "Multimodality Cardiovascular Imaging of Patients with Pericardial Disease," has been issued by the American Society of Echocardiography, in conjunction with other major cardiovascular imaging societies. Allan Klein, MD, Director of Cleveland Clinic’s Center for the Diagnosis and Treatment of Pericardial Diseases, chaired the writing group of 15 U.S. authors — which included four other Cleveland Clinic experts.

“There is a strong demand for evidence-based guidelines to help clinicians diagnose and stage pericardial disease,” says Dr. Klein. “Physicians in all settings need to understand its spectrum of presentation, as well as how to identify the presence of inflammation and calcification in order to provide timely, appropriate treatment.”

Dr. Klein explains that the guidelines are intended for use in primary care, emergency departments, and rheumatology and cardiology subspecialty practices. “We see a low awareness of the signs and symptoms of pericardial disease across the board,” he says. “The disease is often not identified on imaging. Aggressive conditions that are identified are often treated for an inappropriate duration.”

Pericarditis, says Dr. Klein, is “an equal-opportunity disease.” It affects patients of all ages, from teenagers to older adults, including many with systemic lupus erythematosus or recent open heart surgery. The etiology may be infective, auto-immune, post-myocardial infarction, autoreactive or idiopathic. It may be acute, recurrent or constrictive. Symptoms are often puzzling, and diagnosis may be complicated by the condition's appearance in combination with myocardial or valve disease. (As an example of the guidelines’ diagnostic insights, Dr. Klein offers this pearl to help identify constrictive pericarditis: “Have the patient stand or sit upright, then look for distended veins in the neck and an elevated jugular venous pulse. These are clear clinical signs.”)

Dr. Klein believes that undertreatment of acute or recurrent inflammatory pericarditis is the leading reason for incomplete disease resolution. “By the time I see patients, many have had up to five or 10 recurrences.”

While many cases can be treated appropriately outside a dedicated center for pericardial disease, immediate referral to an expert center is advised when a patient presents with advanced symptoms or doesn’t respond to therapy, in view of the condition’s high morbidity with many recurrences and mortality from advanced heart failure. Within one or two days of an outpatient visit to Cleveland Clinic’s Center for the Diagnosis and Treatment of Pericardial Diseases, a patient will be seen by multiple subspecialists and undergo blood and advanced imaging tests with pericardial protocols, heart catheterization (if needed), appropriate medical treatment and surgery, if necessary.

“Imaging-guided treatment of pericarditis is undergoing a renaissance,” Dr. Klein observes. “Our goal in developing these guidelines is that the disease become more widely recognized and appropriately treated so that patients can feel better and get back on their feet.”

To refer a patient to the Center for the Diagnosis and Treatment of Pericardial Diseases, call 855.REFER.123.

New Name, New Director Reflect Progress in Transplants

With its newly updated name, Cleveland Clinic’s Blood & Marrow Transplant Program (formerly the Bone Marrow Transplant Program) is conveying the medical progress underway and the development of advanced therapies for pediatric and adult blood disorders.

New! FACT accreditation

Cleveland Clinic’s pediatric and adult blood and marrow transplant programs have been newly awarded three-year Foundation for the Accreditation of Cellular Therapy (FACT) accreditation. FACT is the only accrediting organization that addresses all quality aspects of cellular therapy treatments: clinical care, donor management, cell collection, cell processing, cell storage and banking, cell transportation, cell administration, cell selection and cell release.

The name change “is reflective of the advances that have been made and where the field is going,” explains Navneet Majhail, MD, MS, a respected hematology/oncology clinician and researcher who was named Director of the Blood & Marrow Transplant (BMT) Program in 2013.

Those advances, Dr. Majhail says, include better infection, rejection and bleeding prevention and treatment; safer pretransplantation radiation and chemotherapy protocols; and the identification of new sources for hematopoietic stem cells, such as from half-matched haploidentical donors.

“All these have come together to help outcomes in transplantation,” says Dr. Majhail, who previously was Medical Director of Health Services Research at the National Marrow Donor Program and an adjunct associate professor with the University of Minnesota’s Division of Hematology, Oncology and Transplantation. “It’s a combination of better understanding of basic science, of the immunology of transplant, of better protocols and better supportive care, all coming together through clinical research trials and studies.”

Matching Difficulties Create Challenges

Ideally, patients in need of a hematopoietic stem cell transplant (HSCT) can be matched with a sibling who has the same human leukocyte antigen (HLA) tissue type. These transplants have the greatest chance for success. However, only about one-third of candidates for allogeneic HSCT have HLA-matched brothers or sisters.

Improvements in HLA typing have enabled the use of HLA-matched unrelated donors, assuming one can be found. The chances of identifying such a match vary significantly depending on the recipient’s racial and ethnic background. Almost a third of minority patients are not able to find a suitable unrelated donor.

Without an HLA-matched sibling or unrelated HLA-matched donor, a patient’s remaining transplant options until recently were limited to hematopoietic stem cells derived from umbilical cord blood or from a partially HLA-mismatched unrelated donor. Both procedures carry a high risk of transplant- related mortality.

Haploidentical Matching Broadens Donor Pool

Cleveland Clinic is one of a handful of institutions that have begun offering patients another alternative: HLA-haploidentical HSCT. Also known as a “half-matched” transplant, the procedure involves a donor who shares identity with the recipient for one HLA haplotype on chromosome 6 and is variably mismatched for HLA genes on the unshared haplotype.

Since each person inherits one HLA haplotype from each biological parent and passes along one haplotype to each biological child, any patient with a living parent or child has a potential HLA-haploidentical donor for HSCT. The major advantage of haploidentical HSCT is that it significantly broadens the donor pool and gives nearly all patients the chance to benefit from a transplant. It’s a step closer to the concept of a universal donor, says Rabi Hanna, MD, Director of Cleveland Clinic’s Pediatric Blood & Marrow Transplant Program. Moreover, haploidentical donors generally can be identified quickly, are highly motivated to donate for a family member, and can donate lymphocytes for infusion or other cellular therapies in case the recipient experiences post-transplant relapse.

In the past, haploidentical HSCT was considered too dangerous for all but the sickest patients because of a high incidence of severe graft-vs.-host disease (GVHD). But improvements in reduced-intensity conditioning regimens and GVHD prophylaxis with high-dose post-transplant cyclophosphamide have made haploidentical HSCT a feasible option for patients with high-risk malignancies and even for those with life-threatening nonmalignant disorders, Dr. Hanna says.

Additional Research Initiatives are Ongoing

Other efforts are underway at Cleveland Clinic to improve the HSCT process. They include:

  • Developing a transplant protocol that overcomes the HLA barrier, which would further broaden the number of patients who could benefit from a bone marrow transplant.
  • Searching for novel treatments to prevent GVHD and to reduce treatment-related toxicities.
  • Providing nonmyeloablative transplants, a less toxic option in which lower doses of chemotherapy and radiation therapy are given before stem cells are infused so as to suppress rather than eliminate the immune system.

To refer a patient to the Blood & Marrow Transplant Program, call 216.445.5600 or 800.223.2273 ext. 55600.

Diagnosis: Resistant Hypertension

A conversation with George Thomas, MD, Department of Nephrology and Hypertension, Glickman Urological & Kidney Institute

When Is Hypertension Considered Resistant?

When at least three blood pressure medications from different classes, including a diuretic, given at maximal doses have failed to control blood pressure to the patient’s goal. True resistance should be differentiated from false or “pseudo” resistance because so many factors can affect blood pressure.

What Are Some Risk Factors For Resistant Hypertension?

Older age and black race are nonmodifiable risk factors associated with resistant hypertension. Being overweight, excessive sodium consumption (> 1 teaspoon per day), and alcohol intake (> 2 drinks per day for men and > 1 drink per day for women) are modifiable risk factors. Secondary hypertension, which usually is treatable, can be caused by kidney disease, hormonal influences (such as pheochromocytoma or primary aldosteronism) and vascular disease (such as renal artery stenosis).

Causes of pseudoresistance include medications that can elevate blood pressure (such as NSAIDs and oral contraceptives), inappropriate blood pressure medication combinations or dosages, and incorrect blood pressure measurement at home or in the office.

How Do You Ensure Accurate Blood Pressure Readings?

We make sure the patient has not had caffeine, smoked or exercised for at least 30 minutes and is seated for at least five minutes in a quiet room with no distractions. We do readings with the cuff over the bare arm. The patient sits with the back supported, legs uncrossed and on the floor, and the arm supported at heart level.

Because of the potential for white-coat hypertension, we also have patients check their blood pressure at home or use a 24-hour ambulatory blood pressure monitor.

At-home measurements can be done with any validated device, preferably using an arm cuff. One of the recommended schedules is as follows: For one week prior to your clinic visit, check blood pressure in the morning (before medications) and in the evening (before going to bed). Take at least two blood pressure readings with each measurement. Bring the blood pressure machine and/or your logbook to your visit.

For the general population, an office reading of > 140/90 mm Hg on more than two visits, or an average home reading of > 135/85 mm Hg, is diagnostic of hypertension.

Are Blood Pressure Goals Different For Some Patients?

Using the 2014 evidence-based guidelines (JNC 8), the recommended goal blood pressure is < 140/90 mm Hg for adults age < 60 and for adults age ≥ 18 with chronic kidney disease or diabetes; the recommended goal is < 150/90 mm Hg for adults age ≥ 60.

Consider referral to a hypertension specialist when patients are not at goal despite multiple blood pressure medications, when you suspect secondary causes of hypertension or for consultation in managing complicated cases.

What Resources Does Cleveland Clinic Offer For Treating Resistant Hypertension?

Cleveland Clinic’s Department of Nephrology and Hypertension includes physicians who are certified as hypertension specialists by the American Society of Hypertension. Available cutting-edge diagnostic techniques include 24-hour ambulatory blood pressure monitoring, assessment of central pressures and hemodynamic testing to assess the cause of severe hypertension.

To refer a patient for resistant hypertension, call 855.REFER.123.

Using Cardiac Rehabilitation to Lower Mortality from Heart Disease

Multiple studies involving nearly 700,000 patients nationwide have confirmed that participation in cardiac rehabilitation after heart attack, open-heart surgery or percutaneous coronary intervention (PCI) reduces mortality 21 to 47 percent over a one- to six-year period. Yet only 18 percent of patients who qualify for cardiac rehab participate in any program — primarily because they are never referred. Other deterrents include lack of access and cost. Cleveland Clinic is working to eliminate these barriers and encourage participation.

Cleveland Clinic Cardiac Rehab Programs

Main Campus, Cleveland

Euclid Hospital, Euclid

Fairview Hospital, Cleveland

Hillcrest Hospital, Mayfield Heights

Marymount Hospital, Garfield Heights
216.587.8998, ext 8819

Medina Hospital, Medina

Sagamore Hills Medical Center, Sagamore Hills

Wooster Milltown Specialty and Surgery Center, Wooster

Cardiac Lifestyle Intervention Program (CLIP) Wellness Institute, Lyndhurst
877.331.WELL (9355)

“Cardiac rehab motivates and encourages patients to make and sustain lifestyle changes that reduce their risk of heart attack and cardiac death,” says Gordon Blackburn, PhD, Director of Cardiac Rehabilitation at Cleveland Clinic’s Sydell and Arnold Miller Family Heart & Vascular Institute.

“Although many physicians address these issues with each patient, a 10-minute office discussion has not been shown to produce the necessary motivation for change in most patients,” he says. “A team approach involving the patient, primary care physician, cardiologist and rehab staff has been demonstrated to motivate sustained change in a much larger percentage of patients.”

Two types of cardiac rehab are reimbursed by Medicare: a traditional program appropriate for all comers and an intensive program for the highly motivated patient. Cleveland Clinic offers both.

“Any patient who has stable angina or is discharged after coronary artery bypass surgery, heart valve surgery, PCI, myocardial infarction or heart transplant should be referred for cardiac rehab. Exceptions might include patients with a high mortality risk and those discharged to a long-term-care facility,” says Dr. Blackburn.

Overcoming Barriers

Although referrals to cardiac rehab are likely to increase in 2015, when Medicare ties referrals to physician reimbursement, there is no guarantee a patient will enroll in or complete the program. Cleveland Clinic is examining ways to improve patient interest and adherence.

Lack of access or transportation to a program and cost are commonly cited reasons for failure to participate. Cleveland Clinic has improved access by offering cardiac rehab programs throughout Northeast Ohio. Transportation from the Stephanie Tubbs Jones Health Center in East Cleveland to the main campus program is provided free of charge.

Making rehab more affordable is a goal. Currently, copays of $20 to $30 for each visit may be required. Cleveland Clinic is lobbying to establish a single copay for all forms of rehabilitation and physical therapy requiring multiple visits.

Traditional Rehab Done Well

The supervised cardiac rehab program offered in eight locations comprises up to 36 one-hour sessions. Over the course of 18 weeks, patients follow an individualized, supervised exercise program; learn how a low-fat, low-calorie diet can improve their heart health; and practice coping with stress through meditation, lectures and group discussions with a psychologist.

“Our focus is setting the groundwork for the future by helping patients integrate a healthier lifestyle into their daily routine,” Dr. Blackburn explains.

Typically around the 20th session of the 18-week program, patients begin exercising and making lifestyle changes at home. They meet with rehab staff periodically to discuss their progress and receive encouragement.

Intensive Rehab for Motivated Patients

For patients dedicated to making a profound impact on their health, the Cleveland Clinic Wellness Institute in Lyndhurst offers a Cardiac Lifestyle Intervention Program (CLIP). The 12-week, 72-hour program helps patients make critical yet achievable changes in what they eat, how they respond to stress, how physically active they are and how much love and support they experience.

“We teach patients how to take good care of themselves and achieve a healthy balance in their physical and emotional lives,” says Center for Lifestyle Medicine Director Mladen Golubić, MD, PhD.

Each CLIP session lasts four hours and includes exercise; lessons from a dietitian or chef in preparing low-fat vegetarian dishes; stress management in the form of yoga postures, breathing exercises or meditation; and a supportive group discussion encouraging emotional expression.

Studies have verified that maintaining the program over time can halt or reverse heart disease.

Because CLIP is significantly more intense than traditional cardiac rehab and is offered in an outpatient setting, patients must be in fair functional and stable physical condition. Candidates include patients with stable angina or a history of revascularization or myocardial infarction.

With either CLIP or traditional cardiac rehab, the key to achieving potential mortality benefit is to complete all sessions. “Adherent patients gain the most,” says Dr. Blackburn.

To refer a patient to the Miller Family Heart & Vascular Institute for traditional rehab, call 855.REFER.123, or for intensive rehab (CLIP), call 877.331.9355.

Aid for Adult Autoinflammatory Diseases

New clinic offers expert management for these rare and complex syndromes

Symptoms of Autoinflammatory Diseases

Patients may experience recurring:

  • Fever
  • Rash
  • Joint pain or swelling
  • Chest or abdominal pain
  • Eye redness, pain or dryness

Autoinflammatory diseases, or periodic fever syndromes, are newly grouped rheumatic conditions that can be complex and challenging to diagnose and manage. Autoinflammatory diseases are not the same as autoimmune diseases. Typically these patients do not have autoantibodies for autoimmune diseases such as lupus. But now, your adult patients with autoinflammatory diseases can get the specialized help they need at our new Clinic for Adult Autoinflammatory Diseases.

“This special clinic is a good resource for primary care physicians who have an interest in these conditions and patients with suspected or confirmed cases of such conditions in their practice — most of which are hereditary, caused by genetic anomalies,” explains Cleveland Clinic rheumatologist Qingping Yao, MD, PhD, who heads the new clinic.

A Comprehensive Resource

Over the past decade, significant advances have been made in understanding adult autoinflammatory diseases, which are now believed to be caused by primary dysregulation of the innate immune system. While classification of these diseases is ongoing, they include:

  • Periodic fever syndromes
  • NOD2-associated autoinflammatory disease (NAID)
  • Blau syndrome
  • Familial Mediterranean fever (FMF)
  • Cryopyrin-associated periodic syndromes (CAPS)
  • Tumor necrosis factor receptor-associated periodic syndrome (TRAPS)
  • Hyperimmunoglobulin D syndrome (HIDS)
  • Adult-onset Still’s disease
  • Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome
  • Undiagnosed fever, rash, joint pain or swelling

The Clinic for Adult Autoinflammatory Diseases is one of few centers in the United States to offer expert knowledge and management of these disorders, supported by genetic testing and counseling as well as groundbreaking research.

A Multidisciplinary Team

Joining Dr. Yao, who in 2011 led the discovery of NOD2- associated autoinflammatory disease (NAID), are Felicitas Lacbawan, MD, molecular genetics pathologist, Pathology & Laboratory Medicine Institute; Rocio Moran, MD, medical geneticist, Genomic Medicine Institute; and Christine McDonald, PhD, research scientist, Lerner Research Institute.

Together, the team offers patients the most advanced care and research available, including:

  • Proper diagnosis and treatment
  • Genetic testing on-site, providing faster results at more reasonable cost
  • Genetic counseling
  • Scientific research of the diseases

“While these diseases are rare, with increased familiarity by the medical community they are being diagnosed more frequently,” Dr. Moran says. “For any adult who has suggestive signs and symptoms, a referral to a specialty center can guide decisions on any genetic testing required and help manage the diagnosis of any of these complex conditions.”

To refer a patient for an evaluation in our Clinic for Adult Autoinflammatory Diseases Clinic, call 855.REFER.123.