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Pulmonary Hypertension: No Longer the Kingdom of the Near-Dead

Until recently, patients with pulmonary hypertension (PH) were said to be in “the kingdom of the near-dead.” That describes the years of my mother’s life between her PH diagnosis at 34 and her death at 50. She was frequently in the hospital, and in the years before her death, she depended on oxygen and a wheelchair. Going up a flight of stairs would leave her gasping for breath. But her limitations didn’t stop her from cheering at basketball games and tennis matches, hosting family get-togethers, attending piano recitals and just doing as much as she could while her illness would let her.

Pulmonary hypertension diagnosis
My mother was 34 when she was diagnosed with pulmonary hypertension.

As a kid, I didn’t understand the details of her disease, but as I got older, I found out that rheumatic fever had damaged the mitral valve in her heart, and that she’d had a tumor on her thymus gland (thymoma) removed with radiation as a teenager. The scarring from radiation resulted in pulmonary fibrosis, a scarring of the lung tissue. The heart valve damage combined with scarred lung tissue set the stage for a decades-long illness characterized by a slow decline in quality of life.

Recently I wondered: What if my mother had been diagnosed in 2017 rather than the 1980s — would she have lived longer or had a better quality of life? Sula Mazimba, MD, MPH, assistant professor of cardiovascular medicine at UVA, helped me find out what kinds of treatments are available today for those who suffer from chronic pulmonary hypertension.

A Challenging Condition

PH differs from regular high blood pressure (systemic hypertension) in that PH refers to elevated blood pressure in the arteries of the lungs. This high pressure strains the right side of the heart and makes it difficult for blood to flow into the lungs. The disease can have a number of causes, worsens over time and has no effective cure. Until recently, doctors treated PH with only oxygen, blood thinners, and diuretics to help the body get rid of excess water and salt, or digoxin, which helps the heart beat more regularly.

“PH patients had very poor quality of life,” says Mazimba. There was no effective way of slowing down the progression of the disease, and for this and many other reasons, a lot of doctors avoided treating patients with PH. The challenge attracted Mazimba.

“It’s always satisfying to treat the people that nobody wants to treat,” he says.

Five Categories of Pulmonary Hypertension

The World Health Organization (WHO) has grouped five categories of pulmonary hypertension; the majority of people who have PH fall into multiple categories. Aside from the first category, a rare form of high blood pressure in the lung arteries, most people with PH fall into one or more of these categories:

  • Left heart disease — damage or disease in heart valves
  • Lung problems — caused by COPD, interstitial lung disease, sleep-disordered breathing, previous lung surgery, chemotherapy or radiation therapy, developmental abnormalities or chronic exposure to high altitudes
  • Blood clots — pulmonary embolisms
  • Multifactorial — a mix of other causes besides the above categories

“We rarely see patients whose PH is caused by just one factor.” Mazimba. A typical patient might have damaged heart valves and COPD. Another might have heart disease and sleep-disordered breathing.

Do you get dizzy and short of breath?

Make an appointment with your doctor.

Whatever the cause, PH can start slowly, and some people barely notice it. In my mother’s case, the rheumatic fever initially made her vulnerable; the pulmonary fibrosis compounded her risk. Then, as today, doctors advise PH patients not to get pregnant. Her diagnosis came after having her last child. All of these factors contributed to her disease.

But not everyone who gets PH has such an array of contributing factors and symptoms. “PH can start out with feeling winded or dizzy,” Mazimba says. Even if you think it might be nothing, he says, get those symptoms checked out.

Treating Pulmonary Hypertension

“Some of these symptoms can be managed with lifestyle changes, like treating sleep apnea, quitting smoking, and using blood thinners if you have blood clots.  Controlling blood pressure with weight loss and exercise can also help,” he says. But with severe cases, and depending on the PH category, the treatments become more aggressive. Patients who come to UVA for treatment of their PH will have their care tailored to how advanced their disease is and the category or categories it falls into.

Researchers initially developed Sildenafil (same as Viagra) to treat PH, as it effectively opens the arteries in the lungs. It remains an available treatment option that improves symptoms.

IV drugs, inhaled medications and oral medications are all part of the common medical regimen for PH patients, who require careful and constant monitoring. Because these are long-term treatments of a chronic disease, PH patients will have a lifetime relationship with the healthcare team that treats them.

“These drug therapies weren’t available 10 or 20 years ago,” Mazimba says. “And now with monitoring and treatment, patients not only have longer lives, their quality of life is better, too.”

Heart and Lung Transplant to Treat Chronic PH

I ask Mazimba what kind of treatment someone presenting my mother’s symptoms could expect today.

“Someone with very severe pulmonary hypertension affecting the heart as well could be a candidate for a combined heart and lung transplant,” he says. I ask him if the risks of the transplant — and having to take medication to suppress one’s immune system — would be greater than living with the disease.

“Of course, you trade one set of problems for another,” he says and adds, “But being able to walk up a flight of stairs again after not being able to do so for many years would be a huge increase in her quality of life.”

If the therapies now available to PH patients were available in the early 1990s, maybe my mother would have been able to do the little things, like take a deep breath or go for a walk, that were impossible for her to do. While it’s too late for her, I’m happy to see how far treatment of PH has come.

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