Pulmonary hypertension (PH) is a progressive, life-threatening disease, which is often misdiagnosed and poorly understood, according to the Pulmonary Hypertension Association. The American Thoracic Society estimates hundreds of thousands of patients in the United States and the world, but the exact number is unknown.
Pulmonary hypertension is a general term describing a condition where blood pressure increases in the blood vessels within the lung. This restricts fresh (oxygenated) blood from reaching the heart and used (oxygen-depleted) blood from getting back to the lungs. There are many diseases that can cause PH, including ischemic heart disease, lung diseases like COPD and autoimmune diseases like lupus.
According to the Pulmonary Hypertension Association, there are five distinct groups in the classification of PH. One of the most treatable groups of PH is pulmonary arterial hypertension (PAH).
Paresh C. Giri, MD, pulmonary disease specialist at Loma Linda University Health, helps to raise awareness on the disease and wants to clarify common misconceptions of PAH.
- Myth#1 - PAH is the same as PH. PAH is a specific type of PH, which falls into one of the five different classifications of the disease. It is caused by many underlying diseases and requires specialized treatment. “There is a specific subset of pulmonary hypertension patients that have progressive narrowing of pulmonary vessels and ultimately heart failure,” Giri says. “Both PH and PAH mean that pulmonary artery pressures are elevated above 25 mmHg. However, PAH has two additional criteria that need to be met.” These measurements are taken from a procedure called a right heart catheterization, where a catheter is passed via a vein into the vessels in the lung, says Giri. Symptoms of PAH to look for include: shortness of breath, chest pain, dizziness or fainting episodes, swelling of the lower body and decreased exercise tolerance. “Unfortunately, these symptoms are non-specific and can be seen in a wide array of diseases more common than PAH,” Giri says. “This is a significant reason why PAH is often misdiagnosed, under-diagnosed or diagnosed too late.”
- Myth#2 - PAH is very common, and I can go to any hospital for treatment. PAH is a rare disease, with approximately 15 people out of every million being affected. “PAH is so rare because it’s uncommon to have such a severe, progressive narrowing of pulmonary vessels,” Giri says. “However, we also know that PAH is underdiagnosed.” This disease affects females more than males in a ratio of 3:1. While those ages 30 to 50 years are generally most affected, newer studies are showing that older males are now developing the disease, he says. PAH treatment involves taking medications developed specifically for the disease to dilate and reduce the inappropriate growth of cells in the pulmonary arteries. These medications are complex, involving numerous pills, inhalers and continuous infusions. Because the treatment is so specific, it requires those with the disease to visit expert centers. “While there is a national drive for accreditation of PAH treatment centers, there are currently only 40 such centers in the country, with Loma Linda University Health actively working to become an accredited center.” Giri says. Even with treatment and medications to combat the disease, there is no known cure.
- Myth#3 - PAH medications are only temporary. While medications are part of the treatment process for PAH, because there is currently no cure for the disease, these medications are needed lifelong to improve both quality of life and lifespan, says Giri. He explains that there are three classes of drugs used to treat PAH: endothelin receptor blockers, nitric oxide pathway activators and prostacyclins. Collectively known as pulmonary vasodilators, they work to open up the narrowed pulmonary vessels that contribute to elevation in pulmonary artery pressure. “Before the advent of current treatments, a diagnosis of PAH meant that the patient had less than three years to live,” Giri says. “While there is still no cure, survival has more than doubled. On average after being diagnosed, a patient can expect seven to eight years of life with the therapy that is currently available.”
Giri says it’s important to be educated and aware of pulmonary hypertension and the five classifications of the disease, including PAH. If you or a loved one has recently been diagnosed with PH or PAH, make an appointment with your doctor to talk about distinguishing the two and seeking expert opinion if you have PAH. Making notes before your visit, as well as taking along a trusted family member or friend, can help you through the first appointment.
PAH support groups are an excellent resource for help and encouragement — join now. Visit the Loma Linda Pulmonary Hypertension program website to learn more about PH, PAH and the options our program can provide you.