Affecting only about one in 500 people, hypertrophic cardiomyopathy (HCM) is nonetheless the most common cardiovascular disease with a genetic cause. Because many cardiologists see only a handful of cases throughout their careers, however, patients with this life-threatening condition can optimize their outcomes by receiving specialized treatment at a high-volume center that offers interdisciplinary expertise. Meeting the unique needs of those patients, both regionally and nationally, is why Westchester Medical Center, a member of the Westchester Medical Center Health Network (WMCHealth), initiated its Hypertrophic Cardiomyopathy Program.
Tanya Dutta, MD, Director of Echocardiography at Westchester Medical Center, and Julio Panza, MD, Chief of Cardiology at WMCHealth Network, review a patient’s echocardiogram.
The program began in fall 2016 and expanded with an outpatient center on Long Island in January of this year, says Julio A. Panza, MD, Chief of Cardiology at WMCHealth Network.
“The WMCHealth Heart and Vascular Institute was looking for ways to improve care for patients in the Hudson Valley and beyond,” Dr. Panza says. “We have long treated patients who have HCM, and now we have increased our capabilities to provide the full range of expertise required to be a national center of excellence.”
Rare enough to need specialized care, yet common enough to affect people in communities across the country, HCM challenges providers in terms of both diagnosis and treatment, and also with regard to marshaling institutional support to develop dedicated programs to address it.
“HCM is relatively uncommon, yet about 600,000 people in the U.S. have it,” Dr. Panza says. “The majority of cases are still undiagnosed because most cases are asymptomatic for decades, and even when symptoms are present a firm diagnosis of HCM can be elusive. As a result, most general cardiologists are likely to see only a few HCM patients in their practice, with patients not infrequently presenting late in their disease course. Because one characteristic of the disease is that it manifests in multiple ways — with sudden death or profound heart failure being the first presentation in many cases — many cardiologists do not feel comfortable evaluating and treating patients with HCM.”
Westchester Medical Center is meeting these challenges by concentrating expertise in one location, where patients of all ages can receive treatment. The HCM program has already drawn scores of patients from around the country and is poised to become a major destination center for patients with the disease.
Enter Dr. Naidu
Key to these efforts is Srihari S. Naidu, MD, Director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center. Atypically for directors of HCM programs, who are often general cardiologists or heart failure specialists, Dr. Naidu is an interventional cardiologist. He joined the team at Westchester Medical Center both to pursue his passion for healing HCM and to work in an atmosphere of advanced care and vigorous institutional support.
Dr. Dutta, Srihari Naidu, MD, Director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center, and Dr. Panza discuss the care of a hypertrophic cardiomyopathy patient.
First, Westchester Medical Center already had strong programs in pediatrics, electrophysiology, advanced heart failure and cardiac surgery, he explains. Next, the Chief of Cardiology was extremely supportive of his endeavors, understanding exactly what an HCM program needed.
“Dr. Panza recognized the potential of the HCM program,” Dr. Naidu says. “When you congregate all the right elements, you can attract patients from Manhattan, and even nationally. We’ve seen patients from around the country, including from distant cities such as Seattle and Atlanta. That really happens only if you have a team that can deliver high-quality care.”
Westchester Medical Center’s program includes two dedicated program coordinators to oversee the patient experience from the first phone call or referral through treatment and lifelong follow-up. Additionally, the program has a dedicated echo sonographer who performs every study according to a standardized HCM protocol.
“In 10 years of caring for HCM patients, I learned that to achieve high quality, you need unified protocols used consistently, properly and with high accuracy,” Dr. Naidu says. “That way, we can make important decisions regarding patients based on reliable and reproducible information. We coordinate with a tremendous level of transparency.”
Meetings with surgeons and other providers facilitate clear communication, Dr. Naidu says, and patients learn from the first time they call the office that cohesive therapy is the standard.
“Our HCM coordinators and staff meticulously manage the care of our patients and give them concierge service,” he says. “We have a dedicated number that goes straight to the coordinator, who sees every patient and who knows how we care for them. Everything is bundled into a single visit, so we can move efficiently and determine the most appropriate course of treatment, whether medical therapy, device placement, alcohol septal ablation (ASA) therapy, open-heart surgery or transplantation.”
Patients rely on that smooth care process, Dr. Naidu says.
“We offer them seamless continuity of care,” he says. “In fact, we took the program to the next level, providing imaging, testing, genetics, electrophysiology, psychiatry, pediatric cardiology and all treatment options in the same location.”
Dr. Naidu’s training and background, combined with Westchester Medical Center’s institutional excellence, made the program an ideal fit for new and existing patients alike.
Meeting Diagnostic Challenges
HCM is unusually diagnostically complex, making it vital for patient care to be handled by experienced specialists.
Anthon Fuisz, MD, Director of Advanced Cardiac Imaging at Westchester Medical Center, and Dr. Naidu review the cardiac MRI of an HCM patient.
“A number of questions arise as a physician completes the initial evaluation of a patient,” Dr. Panza says. “There are issues related to potential disease in family members and the need to screen them. There are issues regarding the course of treatment, which typically depends on the presentation in its totality rather than a single factor in the presentation.”
Because the disease is rooted in multiple genetic mutations, Dr. Panza says, genetic screening is a challenge that must be addressed by specialized experts in this field, including both genetic counselors and physician geneticists.
“Even in patients with the same gene mutation — specifically patients in the same family and with the same disease pedigree — the manifestation can be extremely variable,” he says. “Therefore, treatment must be tailored according to the disease presentation.”
Some patients may experience chest pain, syncope or shortness of breath. Others may have no symptomatic disease but learn of their condition when they have a physical or electrocardiogram.
Beneath these presentations lie different structural manifestations of the disease. Enlargement of the heart muscle varies from mild to severe. Some patients may have an outflow obstruction. Patients with this manifestation are candidates for ASA or surgery, Dr. Panza says. Other presentations may warrant defibrillator placement.
“Because presentation is variable, the cardiologist must consider many forms of treatment,” he adds.
A comprehensive HCM program, therefore, requires all the elements Drs. Panza and Naidu have brought together at Westchester Medical Center: clinical experience, a rigorous diagnostic protocol, including exact and detailed imaging studies, and numerous subspecialties and technical abilities housed in the same location.
A Clearer Picture
Cardiologists tackle the diagnostic conundrum Dr. Panza describes with the aid of imaging studies specific to the needs of the HCM population. Imaging can also provide a wealth of material for longitudinal and comparative studies, and it plays a key role in several procedures.
Dr. Naidu performs an alcohol septal ablation procedure on a hypertrophic cardiomyopathy patient.
At Westchester Medical Center, Tanya Dutta, MD, Director of Echocardiography, has interests in research as well as imaging, equipping her to oversee this complex endeavor. She worked with Dr. Naidu to develop patient protocols, and she ensures consistent imaging, enabling the team to store ample data for research into HCM and its treatments.
“Our research and clinical work support each other,” Dr. Dutta says. “On the imaging side, we wanted the best protocol for the echocardiography lab so that all patients we follow would have the same echocardiogram with the same technical details for all studies. This way we can track them over time and compare their success with national outcomes for the disease.”
Clinically, accurate imaging helps physicians make the right decision for each patient. HCM studies differ from other cardiology studies in key ways. In particular, imaging for HCM focuses on very precise measurements in multiple locations that are standardized, including the apex of the heart, an area missed in a typical study. Patients receive an injection of contrast dye so that no problem areas or abnormalities are overlooked.
Due to the varieties of the disease, diagnostic imaging for HCM presents challenges: Many patients with the genotype for HCM are asymptomatic. Imaging helps physicians identify the “sweet spot” for treatment, when the disease appears in imaging studies but does not yet cause clinical symptoms.
“If symptoms have already developed when we begin treatment, we are working backwards,” Dr. Dutta says. “We want to identify patients early so we can prevent or alleviate any symptoms they might develop.”
Dr. Dutta and her colleagues also use imaging in the operating room or catheterization lab during procedures for HCM. Using portable echocardiography equipment, the sonographers take images before, during and after procedures to guide treatment and identify complications or areas that need further attention.
“This was a new approach for us, but we felt confident because we and our technologists were used to imaging for structural heart procedures,” she says. “Dr. Naidu offered training, and I went off-site to train as well. Together we perfected a technique and then taught it throughout the lab. There are few other imaging studies in which you immediately see a great effect in the outcomes of treatment and the patient’s well-being, so it is rewarding to perform imaging for HCM procedures. It is a team effort, and we are heavily involved in the outcome.”
Alcohol Septal Ablation
Key among the procedures used to treat HCM is alcohol septal ablation (ASA). ASA is only about a quarter of a century old, and Dr. Naidu has played an important part in its development.
Dr. Naidu examines a patient following an alcohol septal ablation procedure.
Performed in patients who have not benefited sufficiently from medical therapy, the procedure involves using alcohol injected via catheter to create an infarction in a narrowly targeted area of the heart, the upper interventricular septum. This reduces the pressure overload many patients experience due to the thickening of the left ventricle and accompanying narrowing of the left ventricular outflow tract.
As in angioplasty, the physician uses catheters to access the target. Echocardiography with contrast dye injected via balloon catheter guides the procedure. Alcohol is introduced into the target location until the gradient, or pressure differential, along the left ventricular outflow tract is sufficiently reduced, heralding short- and long-term improvements in heart function and symptoms.
Dr. Naidu is an exceptionally high-volume ASA interventionalist who is recognized widely as a leader in the technique, guiding innovations in the field for over a decade.
“One of the big evolutions we have introduced is a tag-team procedure including electrophysiology and interventional cardiology,” he says. “Our electrophysiologists implant a temporary pacemaker, placing a screw-in lead through the neck so patients can move around and keep the lead in longer, allowing us to watch for complications.”
In fact, the need for a pacemaker after the procedure is one of the very complications Dr. Naidu is on the lookout for.
“We used to place pacemakers intravenously through the leg, but patients couldn’t keep them in that long,” he says. “With the newer approach, they can keep the pacemaker longer with a virtual elimination of associated complications, while becoming mobile soon after their procedure.”
As a result, the team can tell, while the pacemaker is still in place, whether it will be needed as a long-term assist for the patient.
“Our high-volume program has the full spectrum of advanced capabilities and the broad experience that patients need. We will never have to send them elsewhere and lose control of the quality of their care.”
— Srihari S. Naidu, MD, Director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center
Srihari Naidu, MD, Director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center
Echocardiography imaging during the procedure is a second innovation Dr. Naidu has embraced.
“We have the echocardiography team in the lab the entire case,” he says. “We use more echo guidance than fluoroscopic imaging, and we use it to guide the whole procedure, not just during the ablation. Along with the neck-placed pacemaker for added patient safety and comfort, echo guidance throughout the procedure is unique to our program.”
Dr. Naidu is performing clinical research into minimizing ASA-associated complications, such as the risk of needing a permanent pacemaker. Westchester Medical Center will also be involved with a trial of next-generation pharmaceutical alternatives to standard medical therapy and HCM procedures.
A Comprehensive Program
An HCM program must encompass patients and family members alike, Dr. Panza says. Wraparound care includes disciplines from genetics to psychiatry. Westchester Medical Center offers the comprehensive setting to make that possible.
Dr. Naidu with team members in Westchester Medical Center’s Cardiac Catheterization Lab
“Pediatric cardiology is important because patients very rarely have HCM at birth but also very rarely develop it as older adults,” Dr. Panza says. “The disease often develops during childhood or adolescence, usually during a period of rapid growth. Careful monitoring during these years is essential.”
Of these patients, first-degree relatives of the index case need to be screened, Dr. Panza says. Therefore, genetics makes an important ancillary to HCM treatment.
“Screening should be performed in the context of a discussion with the parent because the disease has a genetic background that is detectable in only about 50 percent of cases,” he says. “There is no single genetic mutation that causes all cases of HCM, so screening is not a simple task.”
Screening should involve psychiatry as well, Dr. Panza says.
“The disease can be debilitating at a physical level for some patients who have symptoms, and yet it can be compatible with a completely normal life for those who do not,” he says. “One clear recommendation even for HCM patients who have no symptoms is to avoid competitive athletics. For a teenager who is an athlete, that can have a profound psychological impact. Knowing you have a condition that exposes you to the risk of sudden death can be a terrible psychological burden. Hence, our program has the ability to provide appropriate psychological support.”
This human component, combined with an advanced imaging protocol and Dr. Naidu’s experienced clinical and interventional treatments, makes Westchester Medical Center a key destination for multifaceted HCM care.
“We want primary care physicians and cardiologists to know we have a comprehensive program that offers all potential forms of treatment a patient may need,” Dr. Panza says. “We look forward to collaborating with them.”
Visit westchestermedicalcenter.com/heart to learn more.