Akshay Muralidhar, MD - phaware® interview 535

Dr. Akshay Muralidhar, Co-Director of the Pulmonary Hypertension Center of Excellence at Arizona Pulmonary Specialist in Phoenix, discusses the importance of having a specialized center for pulmonary hypertension. Dr. Muralidhar also highlights the challenges of managing patients with substance abuse issues, particularly methamphetamine use. Dr. Muralidhar emphasizes the importance of access to care, particularly for underserved communities, and the need for outreach programs to ensure that all patients have access to treatment options. 

This Special Edition Episode Sponsored by: Johnson & Johnson

My name is Akshay Muralidhar. I am a Co-Director of the Pulmonary Hypertension Center of Excellence at the Arizona Pulmonary Specialist in Phoenix, Arizona. I joined this group in 2019. We have been a Center of Excellence for nearly a decade now. We have been managing and caring for pulmonary hypertension patients in the Phoenix area for almost two decades, managing complex care across the spectrum of pulmonary hypertension disease process. 

I did my training at the University of Colorado at their Pulmonary Hypertension Center of Excellence. I graduated in 2019. I am a native of Phoenix, Arizona. I was brought up here. I was looking for a reason to come home. It was a nice coincidence that my passion was explored more here at the Pulmonary Hypertension Center of Excellence at Arizona Pulmonary Specialist. 

The importance of having a pulmonary hypertension center of excellence is something that I really felt deeply about, and that's something I looked for when I wanted to start my career as a new attending and a new graduate of a fellowship specialty in pulmonary hypertension. So what makes a pulmonary hypertension center of excellence important? 

Pulmonary hypertension is a rare disease in the general population, but is an incredibly complex disease requiring multidisciplinary care across a spectrum of organs in the human body. As anyone who suffers from pulmonary hypertension knows, the one effect of worsening pulmonary hypertension can affect every single organ in the body. The specialty care offered by a Pulmonary Hypertension Center of Excellence, I feel, really allows for that multidisciplinary comprehensive care that a pulmonary hypertension patient needs with the expertise of someone who has seen hundreds of patients with pulmonary hypertension. 

That is important because being a rare disease, pulmonary hypertension sometimes gets overlooked or is difficult to diagnose in patients who might present with what we call non-specific symptoms. These are symptoms that don't really tell you what the disease is, but are damaging and tend to affect patients significantly, such as shortness of breath, leg swelling, lightheadedness, symptoms often experienced by pulmonary hypertension patients. It takes an experienced diagnostician and management specialist to be able to correctly diagnose disease and manage it in the future. 

Pulmonary hypertension is a disease of the right side of the heart and the lungs themselves, which results in damage to the right heart and the pulmonary vasculature that goes to it. When people present with shortness of breath, leg swelling, and chest pains, oftentimes routine testing in the hospital or at your general cardiologist or general pulmonologist office involves an echocardiogram. This often shows severe dilation and dysfunction of the right ventricle with potential involvement of the elevations in pulmonary pressures. In that situation, people often get alarmed about the presence of pulmonary arterial hypertension. While it is important to be concerned about this, it is also important to take the next step in beginning diagnostic testing and the referral process to a Pulmonary Hypertension Center of Excellence.

The reason for this is because uniformly pulmonary arterial hypertension was a fatal disease in the 1990s. Thanks to expert care and expert medication and treatments, it is now well managed and completely treatable if the diagnostic tests indicate pulmonary arterial hypertension. The most important thing is having a low threshold for suspicion. Whenever we see patients with evidence of right heart failure, which include leg swelling, shortness of breath, lightheadedness, things of that nature, we have a low threshold for getting an echocardiogram just like any general cardiologist, pulmonologist would. If there's evidence of right-sided heart failure, a referral would be indicated to a pulmonary arterial hypertension center. In our center, we will do additional diagnostic testing and screening questions to be able to identify potential causes for this and then do confirmatory testing with right heart catheterization, which is the diagnostic test of choice to determine if the patient has pulmonary arterial hypertension. That allows an experienced physician to be able to formulate a management plan based on the severity of disease and the experience the patient is having. 

At a Pulmonary Hypertension Center of Excellence, the goal is to provide a comprehensive care management strategy for the patient. This includes appropriate diagnostic testing, determination if the patient truly does have pulmonary arterial hypertension, and then classification into one of several groups of pulmonary hypertension that are currently described including: Group 1 pulmonary hypertension, which is pulmonary arterial hypertension, Group 2 pulmonary hypertension, which is due to left heart disease, Group 3 pulmonary hypertension, which is due to lung disease and hypoxia, Group 4 pulmonary hypertension due to chronic thromboembolic pulmonary disease and Group 5, which is pulmonary hypertension due to other etiologies. Now, any experienced pulmonary hypertension physician will tell you that many patients fall into multiple categories. In this case, it takes an experienced physician to determine which is the predominant factor that is affecting the right heart in the pulmonary vasculature, and to be able to identify appropriate treatment strategies. Treatment strategies vary depending on the type of pulmonary hypertension you have. That is why it is very important to make sure that patients have appropriate diagnosis and classification of their disease process. 

After this, a comprehensive strategy to determine treatment is indicated. This includes meeting with an experienced nurse coordinator to be able to assist with management strategies as well as close follow-up routine lab testing to determine how treatment is progressing. If treatment involves therapies such as continuous infusions or injections, appropriate training is necessary to make sure that our patients are able to manage their disease properly as well as the side effects. 

As many patients who suffer from pulmonary hypertension know, side effects from medicines often are just as bad if not worse than the symptoms of the disease themselves. It takes very important coaching by experienced clinicians and nurse coordinators to be able to manage the side effects of the medications so that patients are able to take them without too much discomfort. Over time, many of these side effects are manageable and the true benefit of these medications are observed in the appropriate patient.

Furthermore, being able to determine which classification of pulmonary hypertension a patient has is important, because medications that work for some groups of pulmonary hypertension such as Group 1 and Group 4 disease, may not work for other groups of pulmonary hypertension such as Group 2 disease. It is extremely important to have an appropriate interview with a pulmonary hypertension trained physician to be able to determine which classification predominates. 

When Centers of Excellence are identified, patients have been referred to us from all over the state and sometimes even across the country. I've had patients from rural Arizona, which is a large swath of area as well as many Native American reservations. I've also seen patients from Southern Nevada and Western New Mexico who make the sometimes eight-hour trek to get to our center. So, we have a very large referral basis to be able to seek a Center of Excellence so that they can get comprehensive care for their pulmonary hypertension patients.

Outreach is incredibly important for any Center of Excellence dealing with rare diseases such as pulmonary hypertension, because it is very important to identify those that don't have access to care, to be able to get them to a Center of Excellence in an appropriate and timely manner. I personally do a month of pulmonary clinic at one of the Native American reservations. While it is a general pulmonary clinic, I've already identified several pulmonary hypertension patients in that area that I have then ensured that they are seen and managed by our Comprehensive Center of Excellence.

It's incredibly important that not only common diseases, but also rare diseases that are life-threatening such as pulmonary hypertension be identified with a low threshold for referral and ensuring that these patients have the appropriate resources to be able to do that. Pulmonary hypertension centers, as I mentioned, also provide a comprehensive management strategy to these patients. This not only includes access to nurse coordinators, physicians who are trained in pulmonary hypertension such as myself, but also access to things like support groups as well as symposia and learning materials and access to online materials that are important for patients to be able to take away and manage their disease at home.

Methamphetamine use is one of the most common drugs used in Phoenix, Arizona. In fact, Phoenix has unfortunately topped the charts in the country for methamphetamine use. Many of our patients who struggle with methamphetamine use get admitted either to the hospital with decompensated previously undiagnosed pulmonary arterial hypertension, or are seen in our clinic for progressive shortness of breath and are then found to have pulmonary hypertension. 

This is a particularly difficult population of patients to treat only because not only do we have to manage their medical disease as well as ensure that medications are administered appropriately, but we also want to address their ongoing substance abuse problem in a timely manner. Unfortunately, resources in general are lacking from management of substance abuse such as methamphetamines. So, a comprehensive care is only benefited many of our patients because of close follow up management strategies, support groups, things of that nature that really allow our patients to understand the lethal disease that they have because of their substance abuse problem and seek treatment before it's too late.

Pulmonary hypertension back in the 1990s was a uniformly fatal disease. Thanks to the development of IV therapy, specifically IV Epoprostenol, this started saving lives initially. Since then, thanks to the advent of clinical trial research programs, pulmonary hypertension is now an extraordinarily manageable disease. It's wonderful to see the advent of therapies available for pulmonary hypertension patients thanks to clinical trials. Part of being a Pulmonary Hypertension Center of Excellence is that we have a robust clinical trial program. We understand the importance of clinical trials in improving the nature of treatment for patients suffering from pulmonary hypertension. Our patients are wonderful in actively participating in clinical trial programs. Thanks to these, we have now been able to administer adequate treatment for pulmonary hypertension to many patients that just consist of oral therapies. Thanks to clinical trials, a recent drug, Sotatercept, was approved, which is a new first-in-class drug that has been shown to be beneficial for pulmonary hypertension patients, and the field is only expanding.

We have many partners in our pharmaceutical industry as well as in the clinical practice arena that developed a partnership with Centers of Excellence across the country thanks to being able to bring together many patients from a rare disease. In order to have a successful clinical trial, you need to enroll many patients and need to be able to demonstrate that your particular therapy has benefit to these patients. In a rare disease, the only way to be able to do that is to focus on centers that see these patients on a regular basis such as Pulmonary Arterial Hypertension Centers of Excellence. So, we personally have a robust clinical research program. We've been involved in most clinical trials that have come in the last decade, and ours the highest enroller in many of these as well thanks to an excellent clinical research team and most importantly, our amazing patients who are willing to participate in these trials so that they can benefit the field as a whole.

One of the biggest reasons why I joined a Pulmonary Hypertension Center of Excellence is because I am able to open the door to patients who are interested in expanding treatment options for the field so that everyone can benefit by participating in these clinical trials. Being part of a Pulmonary Hypertension Center of Excellence, I think it is very important to make sure that patients have appropriate strategies in place for the therapy. This not only involves diagnosis of the disease, but also appropriate risk stratification. Many of my patients fall squarely into the category of pulmonary arterial hypertension, but many others have comorbidities that put them across several groups of pulmonary hypertension. All these patients require a specifically tailored regiment of therapy that allows for simplicity of management so that patients know what they're taking, how they're taking it, and to make sure that they're able to take it appropriately without overdoing it in terms of therapy.

The advent of oral and inhaled therapy and now with Sotatercept has really made a difference in terms of being able to simplify the regimen. However, some of our sicker patients, while they require multiple therapies, 1, 2, 3, or even four therapies now with Sotatercept, many of our patients do just fine with dual oral combination therapy, which is basically standard of care at this point with close monitoring and management. However, pulmonary hypertension is still, by definition, an incurable disease. It's manageable, and management means making sure that the patients are not only on the appropriate therapy, but not overdoing it or not underdoing it. This requires close risk stratification, echocardiogram, right heart catheterization data if necessary, as well as management by an experienced clinician found mostly at a PH Center of Excellence. 

It really is important, I think, for patients to realize that their general cardiologists, their general pulmonologists, do an amazing job in being able to identify the disease. Most of the time, we're not the ones identifying the disease. We're the ones managing it. We get referrals because pulmonologists, cardiologists, and general practitioners understand how serious this disease is and being able to improve the awareness of the severity of this disease, especially since pulmonary hypertension is a relatively uncommon disease compared to things like diabetes and coronary disease, but just as deadly. I think is extremely important to be able to identify and send over to a fellowship trained pulmonary hypertension physician for management.

This is particularly important in our underserved communities, which is why I think outreach is so important, especially in Native American reservations, across the landscape for patients in lower socioeconomic status who may not have access to not only diagnostic equipment, but treatment options too once they're diagnosed. We routinely care for patients who are not able to afford sometimes the expensive medications available that will treat their illness, requiring constant searches for sources of funding to provide for these patients to be able to manage their disease appropriately with the drugs that we know work for them. 

It is gratifying that pulmonary hypertension is now much more manageable than it was 20 years ago, but now it is very important to recognize the problem of access, the problem of specialty care, risk management, and treatment by Centers of Excellence because we need a comprehensive approach to management of disease. We cannot just treat the disease by itself because that will not help the patient. 

My name is Dr. Akshay Muralidhar, and I am aware that my patients are rare.

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