I Have Been Working Alone: A Story of PAH, Told From the Inside
Breathlessness on the stairs. Fatigue that never quite lifts. Near-fainting at the top of a staircase. The early signs of pulmonary arterial hypertension are easy to explain away — here is why, told from inside the right ventricle.
- In pulmonary arterial hypertension, the small blood vessels inside the lungs narrow gradually over months and years, and the right side of the heart compensates by pushing harder.
- Because the right ventricle compensates, early PAH often produces normal results on a chest X-ray, a breathing test and basic blood work.
- Half of Canadian patients with PAH waited more than two years from their first symptoms to a confirmed diagnosis. Only about one in four was diagnosed within the first year.
- Breathlessness during activity that does not improve with an inhaler, fatigue out of proportion to age and fitness, and light-headedness during exertion are the signs worth investigating further.
- Normal standard tests are not the end of the investigation. They are a reason to look carefully at the right side of the heart.
Let Me Introduce Myself: The Right Ventricle
I am the right ventricle, the lower right chamber of your heart. Most people have never thought about me, and that is fine. I am not the side of the heart that gets talked about. I am the quiet one. My job is simple: I take blood coming back from the body and pump it through the vessels in the lungs, where it picks up fresh oxygen before the left side of the heart sends it back out to all the other organs. Low pressure, short trip, no drama. I do this about 100,000 times a day without anyone noticing.
When I become noticeable, it usually means something has been building for a long time. In pulmonary arterial hypertension, what builds is a gradual narrowing of the small blood vessels inside the lungs. The vessel walls thicken over months and years, quietly reducing the space available to push blood through. The pressure I work against goes up. I adapt. I push harder. I grow a thicker wall to keep up. And because I keep compensating, nothing obvious shows up for a long time. The person I live inside keeps going to work, keeps explaining away the tiredness, keeps waiting for the breathlessness to pass on its own.
That person, my person, was Sofia. She was 41, a school principal in Halifax.
What It Feels Like When the Pressure Rises
Imagine being asked to push water through a hose that is slowly being pinched closed. At first, you just push a little harder and the water keeps moving. You barely notice the extra effort. But the hose keeps narrowing, week after week, and at some point pushing harder is not enough on its own. You need to get stronger to keep up. So you do. You build up. You manage. From the outside, the water is still flowing, so no one thinks anything is wrong.
That is what the early years of pulmonary arterial hypertension felt like from where I sit. The pressure climbed. I thickened my walls to generate more force. The blood kept moving. At the beginning, Sofia noticed she got winded on stairs. She mentioned it to her doctor. Her lungs sounded clear, her breathing test came back normal, her chest X-ray looked fine. She was given an inhaler in case it was asthma and sent home reassured. The inhaler did nothing for me, because I am not an airway.
I am a pump working against a narrowing pipe, and no one looked at the pipe yet.
This is the part of pulmonary arterial hypertension that makes it so hard to catch early. The standard tests that doctors reach for when someone is short of breath, the chest X-ray, the breathing test, the basic blood work, are not designed to find me. They look at the lungs, the airways, the blood. In early PAH, all of those things look more or less fine. What is not fine is the pressure building inside the vessels between the heart and the lungs, and that pressure does not show up on a chest X-ray until it has been high for a long time.
How Pulmonary Arterial Hypertension Hides in Plain Sight
About eighteen months into Sofia’s symptoms, a cardiologist ordered an echocardiogram, an ultrasound of the heart. I was right there in the image. My walls were already thicker than they should have been. The pressure in the vessels ahead of me was already elevated. But the report that came back focused mainly on the left side of the heart, which looked normal. The findings on the right ventricle were mentioned briefly, described as mildly unusual, and put down to body type. Sofia was told her heart looked essentially fine.
Why the Right Side of the Heart Gets Less Attention
This was not a criticism of anyone who read that scan. It reflected something true about how hearts are examined. The left ventricle, the powerful side, gets most of the attention in a standard heart ultrasound. It is larger, easier to measure, and where most common heart problems show up. The right side is harder to image clearly and has historically been considered less important. In many standard reports, a single sentence covers everything about my size and function. The key measurement that reflects pressure in the lung vessels, the speed of a small amount of blood leaking back through the valve on my right side, can be missing entirely from the report if the signal was hard to capture. I was in the picture. I just was not what anyone was looking for.
How Long Canadians Wait for a PAH Diagnosis
This gap in how echocardiograms are read is one of the main reasons pulmonary arterial hypertension takes so long to diagnose. A 2021 survey of Canadian patients with PAH found that half of them waited more than two years from their first symptoms to a confirmed diagnosis. Only about one in four was diagnosed within the first year. Many were told along the way that their symptoms were caused by anxiety, asthma, or simply being out of shape. Most of them were women between 30 and 60, active and otherwise healthy, whose gradual decline was easy to explain away.
Winded on stairs. Chest X-ray, breathing test and exam all normal. Sent home with an inhaler for possible asthma.
Echocardiogram ordered. Right ventricular findings noted briefly and attributed to body type. Reported as essentially normal.
Elevator instead of stairs. Sitting through meetings she used to run standing. Symptoms no longer mentioned.
Fainting at the top of a staircase. An ECG shows right heart strain. A repeat echocardiogram is read with the right side in focus. Right heart catheterization confirms PAH. Treatment starts that week.
What Happens When the Right Ventricle Reaches Its Limit
There is a point where adapting is no longer enough. My walls can only grow so thick. As a muscle, I can only work so hard. When the pressure in the lung vessels keeps rising past what I can manage through effort alone, I start to stretch. My chamber gets bigger. This changes the shape of the whole heart. The wall I share with the left ventricle starts to bulge toward the left side under the pressure on my side, which makes the left ventricle work less efficiently too. Things that were compensated start to become visible. Sofia started taking the elevator instead of the stairs. She sat during meetings she used to run standing up. She stopped mentioning the breathlessness because she had run out of explanations and had started quietly reorganizing her life around something she could not name.
Two and a half years after her first doctor’s visit about breathlessness, Sofia passed out at the top of a staircase at school. She went to the emergency department that evening. An ECG showed a pattern that suggested the right side of the heart was under strain. A heart ultrasound done the same night was read with the right side in focus this time. The pressure in the lung vessels was significantly elevated. My chamber was enlarged. The word that appeared in the report for the first time was pulmonary hypertension. Sofia was referred to a specialist centre two hours away, where a procedure called a right heart catheterization, a thin tube passed into the heart to measure pressure directly, confirmed the diagnosis.
Treatment started the same week. The pressure began to come down. I had been working against that pressure for three years, and it was the first time I felt the resistance ease. I do not have a way to express that. I just have the numbers, and the numbers changed.
Early Signs of PAH and Why Early Treatment Matters
How Common Is Pulmonary Arterial Hypertension?
Pulmonary arterial hypertension is not common, but it is not as rare as most people think. Registry data estimate a prevalence of roughly 50 per million adults, and that number is likely an undercount because so many cases take years to reach a confirmed diagnosis. It most often affects women between 30 and 60, though it can develop in anyone. The disease cannot be cured, but it can be treated, and treatment works better when it starts earlier, before years of rising pressure have changed the structure of the heart.
The Early Signs Worth Paying Attention To
- Breathlessness during activity that does not improve with an inhaler or standard heart medication.
- Fatigue out of proportion to the person’s age and fitness level.
- Light-headedness or near-fainting during exertion.
If a chest X-ray and breathing test both come back normal in someone with those symptoms, that is not the end of the investigation. It is a reason to look more carefully at the heart, specifically at the right side. A heart ultrasound that includes careful measurement of right-sided pressures, and a referral to a pulmonary hypertension centre if those pressures are elevated, can change the entire course of what happens next.
I have been here the whole time. I send signals when something is wrong. I just need someone paying attention to the right side of the picture.
What to Ask About
Pulmonary arterial hypertension is diagnosed by a procedure called right heart catheterization, which directly measures the pressure inside the heart and lung vessels.
If you or someone you know has unexplained shortness of breath during activity, and standard tests have come back normal, ask about getting a heart ultrasound that includes a careful look at the right side of the heart.
Early referral to a pulmonary hypertension specialist centre makes a real difference in outcomes.
Frequently Asked Questions
What are the early signs of pulmonary arterial hypertension?
The signs worth paying attention to are breathlessness during activity that does not improve with an inhaler or standard heart medication, fatigue that seems out of proportion to someone’s age and fitness level, and episodes of light-headedness or near-fainting during exertion.
Why do standard tests often miss pulmonary arterial hypertension?
The standard tests used when someone is short of breath, such as a chest X-ray, a breathing test and basic blood work, look at the lungs, the airways and the blood. In early PAH those results may all be normal or only subtly abnormal. The problem is that these tests are not designed to measure pulmonary artery pressure. Echocardiography is the appropriate screening test when PAH is suspected.
Who is most likely to develop pulmonary arterial hypertension?
PAH most often affects women between 30 and 60, though it can develop in anyone. Registry data estimate a prevalence of roughly 50 per million adults, although the true number is likely higher because diagnosis is often delayed.
How is pulmonary arterial hypertension diagnosed?
An echocardiogram is usually the first test used to look for signs of pulmonary hypertension. If the findings suggest PAH, the diagnosis is confirmed with a right heart catheterization, which directly measures pressure inside the heart and pulmonary arteries.
How long does it take to be diagnosed with PAH in Canada?
The Pulmonary Hypertension Association of Canada’s 2021 Canadian PH Community Survey found that half of patients with PAH waited more than two years from their first symptoms to a confirmed diagnosis, and only about one in four was diagnosed within the first year.
References
- Pulmonary Hypertension Association of Canada. Canadian PH Community Survey 2021: National Report. Toronto: PHA Canada; 2021.
- Pulmonary Hypertension Association of Canada. The Impact of Pulmonary Hypertension: Canadian PH Community Survey Summary Report. Toronto: PHA Canada; 2023.
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731.
- RESPIPLUS. Pulmonary Arterial Hypertension in Canada: Closing the Gaps. White Paper. Montreal: RESPIPLUS; November 2025.
- RESPIPLUS. Report on Adult Pulmonary Arterial Hypertension. Montreal: RESPIPLUS; 2025.
- Zelt JG, McArthur E, Al-Omari M, et al. Mortality trends in pulmonary arterial hypertension in Canada: a population-based cohort study. Can J Cardiol. 2022;38(7):1029-1037.
- Wijeratne DT, Lajkosz K, Brogly SB, et al. Increasing incidence and prevalence of World Health Organization Groups 1 to 4 pulmonary hypertension: a population-based cohort study in Ontario, Canada. Circ Cardiovasc Qual Outcomes. 2018;11(2):e003973.
- PHA Canada & Canadian VIGOUR Centre. Socioeconomic Burden of PAH Survey: Final Report. Toronto: PHA Canada; 2023.
Keep listening
The eXpand Podcast · Season 6
A full season on pulmonary arterial hypertension, with Canadian specialists.
Sofia is a composite patient created for educational purposes and does not represent a specific individual. This content is intended for education and does not replace clinical judgement or individualized medical advice.
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