Pulmonary Arterial Hypertension: A Silent Killer

Pulmonary Arterial Hypertension (PAH) is a disease that has befuddled doctors for decades.

Not many Americans know what it is, but PAH is a breath-stealing, life-threatening condition that is very often misdiagnosed.

What the statistics show is that it takes between two and three years before the disease is recognized and the patients are correctly diagnosed. The scary part of this is that the more the diagnosis is delayed, the worse the prognosis is.

Expert management is required to carefully diagnose and properly treat this dangerous disease. Unfortunately, there are only few pulmonologists with that special passion and training, as well as only few PAH centers or clinics in the state of Florida and the country. These patients require very special care and dedication. The specialized physicians practically become their primary care physician due the complexity of the management of the drugs that are utilized to treat the disease. They really establish a relationship with those patients. 

There are also several foundations, organizations, and PAH patient groups that provide support to the PAH community and research. 

It’s probably fair to ask why – in this age of CT scans, echocardiograms, ventilation perfusion scans and pulmonary function tests – PAH is so difficult to diagnose.

The short answer is because PAH can easily be mistaken for other diseases such as congestive heart failure, chronic hypoxia due to emphysema, pulmonary fibrosis or severe obstructive sleep apnea, blood clots in the lungs, and many others unclassified. 

Also, in many cases, there is another condition that leads to the problem. Any of the following illnesses can lead to high blood pressure in your lungs: HIV, cirrhosis of the liver, Lupus, scleroderma, rheumatoid arthritis and other autoimmune diseases; a heart defect you’re born with, and others. 

That explains why the diagnosis can be so incredibly difficult. There’s a great deal to look at – and hopefully eliminate – but all that takes time, expert criteria and dedication.

The final diagnosis is established with a cardiac catherization of the right side of the heart that confirmes the elevated pressures of the pulmonary artery. 

In a nutshell, having PAH means that you have high blood pressure in the arteries that go from your right side of your heart to your lungs. But this particular form of high blood pressure will not show up in the routine blood pressure readings your doctor performs using the traditional cuff or sphygmomanometer.

The tiny arteries in your lungs become narrow or blocked. It’s harder for blood to flow through them and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries and after a while the heart muscle gets weak, and that can lead to catastrophic heart failure.

The American Lung Foundation ominously adds, “Since patients with PAH are often not diagnosed until their symptoms are severe, they may only have a few years to live unless they get proper treatment. Fortunately, advances in therapies have led to impressive improvements for patients with pulmonary arterial hypertension.”

Those advanced therapies include up to 15 relatively new drugs called pulmonary vasodilators. Unfortunately, the disease has no cure, but luckily these drugs have delivered impressive results by increasing the  survival rate and have been able to change the patients quality of life.

Symptoms of PAH may include shortness of breath when active that gets worse over time, chest pains, general fatigue, dizziness or fainting spells, a racing pulse or heart palpitations, and even a seemingly innocuous swelling in the ankles and legs.

It is advised that people with the symptoms above to speak with their primary care physicians. If PAH treatment is needed, help is now available in specialized centers.