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I'm Aware That I'm Rare: the phaware® podcast

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Sanjay Mehta, MD - phaware® interview 212

Jan 15, 2019

Sanjay Mehta, MD, FRCPC, FCCP, is PHA Canada’s Chair. He is additionally Professor of Medicine at the University of Western Ontario, and Director of the Southwest Ontario Pulmonary Hypertension Clinic at the London Health Sciences Center in London, Canada. He is also Chair of the Pulmonary Vascular Disease Committee of the Canadian Thoracic Society. Dr. Mehta has been caring for patients with PH and doing research in PH since 1989. In this episode, Dr. Mehta discusses the importance of early diagnosis.

I'm Dr. Sanjay Mehta. I'm a respirologist in London, Ontario, also a professor of medicine and most importantly I'm director of the Southwest Ontario Pulmonary Hypertension clinic and finally, but very importantly, I'm chair of Pulmonary Hypertension Association of Canada.

I've been looking after patients in the London clinic for 22 years, but I first got exposed and introduced to pulmonary hypertension 30 years ago as a medical resident at McGill University in Montreal. As a young physician, these were rare patients I didn't see very often, but I recognized they were very sick and needed our help. As well, I was quite interested in looking after them to learn more about them, their illness and especially the fascinating interaction of the heart and the lungs. Even then, I recognized that we could help these patients, and this is only still true today. They're very sick patients that need our help and they have much to teach all physicians.

Pulmonary hypertension is another form of hypertension or high blood pressure, but this time specifically in the blood vessels that feed the lungs or the pulmonary arteries.  The most important issue is that the right heart has to pump blood to the lungs and it gets under strain and pulmonary hypertension. The most important complication is failure of the right ventricle or the right side of the heart. Patients are affected by symptoms like shortness of breath and fatigue and eventually as the disease progresses, they get dizziness, syncope, and chest pain. The most important thing to recognize is it's a serious illness. This is an illness that still kills people in two to three years if it's not diagnosed or treated and yet we do have many treatment options that help patients.

The biggest issue is that pulmonary hypertension is not being recognized or diagnosed soon enough and this is the biggest issue we hear from patients and their families, because they have symptoms for often two years before they get a diagnosis and finally get treated. I don't think this is an issue of awareness. Most doctors have heard of pulmonary hypertension. The challenges is this is an issue of ignorance, not knowing what the disease is, how to recognize and especially diagnose the disease and maybe most importantly these days not recognizing this as a treatable illness, that we have excellent therapies that are helping many patients with pulmonary hypertension.

It's very hard for a good general doctor to be able to recognize all serious heart and lung disease. They're expected to deal with common illnesses like Asthma and COPD and heart failure that cause people to be short of breath, very much like pulmonary hypertension. The challenge is to always be aware that there are uncommon illnesses that are always going to be presenting themselves and to think if you're treating somebody that's not getting better and not responding as you expect, could it be something less common? Then to know that the most important test for pulmonary hypertension is an echocardiogram. This will pick up pulmonary hypertension in most patients, especially when it's clinically significant or important. Pulmonary hypertension cannot easily be diagnosed on physical examination and the history is challenging, because patients have the same symptoms as many heart and lung conditions and so I always emphasize, think about it, and then an echocardiogram is the first and best test.

Pulmonary hypertension is a difficult diagnosis. The problem is that the symptoms patients experience, shortness of breath, tiredness, eventually syncope, passing out or chest pain are very similar with many other common lung and heart diseases. For example, patients with Asthma and COPD are always short of breath, and so it's difficult for a family doctor or a community specialist to always think about pulmonary hypertension. The only issue is that when you treat somebody for common issues and they don't get better, they don't respond - always be thinking, could I be missing something and that's where pulmonary hypertension comes in. It's easily missed especially because on physical examination it's not an easy diagnosis to pick up and we don't recommend looking for it intensively. The most important thing is think about it and then an echocardiogram is the right first test, as it picks up pulmonary hypertension in most patients, especially if the disease is clinically important or significant.

The diagnosis of pulmonary hypertension is most challenging because it takes a little bit of a framework to understand what are the possible causes. I think doctors understand that you can get pulmonary hypertension if you have heart disease or lung disease or very importantly pulmonary thrombo embolism or pulmonary emboli, and so it takes a very clear workup where you look for those conditions, heart disease, lung disease, and blood clots, and recognize that many of those can contribute to pulmonary hypertension. The most important thing is to think about addressing each of those points. If you treat all those conditions, that's what it takes to improve a patient with pulmonary hypertension.

For the average family physician or community specialist who has a patient that's been diagnosed with pulmonary hypertension, I would strongly advise you to learn from your patient. Understand their illness, understand their symptoms and their limitations. As well, they're being cared for often in a PH expert center, and so learn from them. They'll help you understand what tests are important to diagnose pulmonary hypertension in your future patients, as well as what tests are useful to follow your patient with pulmonary hypertension, and then also what treatment options there are.

This is an illness that fortunately patients are better, they're living longer, and so a family doctor is so important because there are many other things that have to be looked after besides just the pulmonary hypertension. These patients need anticoagulation. They need diuretics to control their fluid and a family doctor can very well look after that. As well as patients live longer, they need the usual cancer screening, they need their diabetes and their heartburn looked after and their arthritis. There's many roles for a family physician to do a good job looking after pulmonary hypertension patients and to along the way, learn about pulmonary hypertension to diagnose the next patient.

It's been quite an honor to be involved in pulmonary hypertension and learn about it and look after patients for almost 30 years. It's also been an incredible time because the pace of development of research, knowledge and diagnostic approaches and treatments is almost unparalleled in any field of medicine. From an illness that used to kill everybody within two to three years without any treatment options, we're now at a stage that we can reliably offer patients a quality of life that's better and longer survival. Average seven to 10 years, but many patients now living 20 years and longer with pulmonary hypertension. So all of that's changed dramatically because of great science and research and it's a wonderful journey to be part of along with patients and their families.

Physicians in almost any branch of medicine will run into these patients. They present to family doctors. They present to emergency rooms, they present the specialist all the time, because they get many complications of their disease if they're not diagnosed and the most important thing to recognize is they need a diagnosis because you can help them. We can help them with the wonderful treatment options we have, medical and surgical. These have been shown to improve their quality of life, reduce their symptoms, improve their functional exercise capacity, and very importantly, live longer and better lives.

I'm Dr. Sanjay Mehta and I'm aware that I'm rare.

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