Natalie Cole's Death Highlights the Danger of Pulmonary Arterial Hypertension

Millions of fans were shocked by the recent death of “Unforgettable” singer Natalie Cole.

Cole, who has battled several health issues, received a kidney transplant in 2009. Her family released a statement to the Associated Press saying the singer was diagnosed after her transplant with pulmonary arterial hypertension (PAH), a rare lung disease that ultimately led to heart failure and Cole’s untimely death at age 65.

Not many people have heard of or understand PAH. The disease is rare, with an estimated 15-20 cases per one million. PAH occurs when the pulmonary arteries, which connect the right side of the heart to the lungs, narrow or tighten. This leads to more pressure in the arteries, which restricts blood flow to the right side of the heart and causes it to become enlarged because it has to work harder to pump blood through the lungs.

Signs and Symptoms of PAH

People in the early stages of PAH often experience breathlessness, fatigue, dizziness, palpitations, chest pain and fluid retention that causes swelling in the limbs.

As the disease progresses, it can be difficult for patients to exercise or do routine physical activities. Blood circulation throughout the entire body also gets worse because of cardiac issues associated with the disease. PAH leads to weakness on the right side of the heart, which ultimately causes heart failure.

Diagnosis and Treatment Options

Unfortunately, most people with PAH aren’t diagnosed with the disease until it has advanced, which makes it more difficult to treat. This may be because many of the symptoms easily can be mistaken for other common illnesses, such as asthma or general fatigue. Patients are usually diagnosed with PAH about two years after they first begin to experience symptoms.

Early detection can improve patient outcomes and provide more treatment options. We typically perform a physical exam to look for things like abdominal swelling or peripheral edema (swelling in the hands, arms, legs, etc.), two common symptoms of PAH. However, PAH is a diagnosis of exclusion, meaning we need several tests to rule out other causes or illnesses.

An echocardiogram, which allows us to view an ultrasound of the heart and assess pressure in the pulmonary arteries, also can help us diagnose PAH. However, right heart catheterization is the best way to diagnose this disease. Right heart catheterization helps us determine pressure in the right side of the heart and pulmonary arteries. This procedure is more reliable than an echocardiogram, which must be used in conjunction with another diagnostic test to get accurate results.

There is no cure for PAH, but several treatment options can improve symptoms and slow the progression of the disease, depending on each patient’s diagnosis. Oxygen therapy can improve symptoms and patients’ ability to cope with the disease. Inhalers, pills and drugs administered subcutaneously or through an IV also may help open blood vessels that have narrowed or tightened. Calcium channel blockers also maybe an effective treatment option for people with PAH. These medications decrease blood pressure in the lungs and improve overall blood flow. 

If targeted therapies do not work, surgery may be the best treatment for people with severe PAH. A balloon atrial septostomy, for example, can create a small hole between the right and left sides of the heart to relieve the pressure. For patients with pulmonary hypertension resulting from of blood clots in the lungs, surgery to remove the clots may provide a cure. These patients may be placed on a pill to further reduce the pressure in their pulmonary arteries.

A lung transplant may be recommended in the most severe cases for patients who haven’t experienced improvement with other treatments.

PAH is a progressive and life-threatening condition, but we can improve symptoms and save more lives with early detection. If you experience any of the previous symptoms I mentioned, including shortness of breath, swelling in your limbs or abdomen, fatigue or chest pain, visit a doctor as soon as possible. Though these are all common symptoms of other health conditions, you won’t know until you see a qualified medical professional who can confirm or rule out a diagnosis of PAH.