disorders, metabolic disorders and other various conditions
can lead to PH.
“There are a lot of other diseases that can also cause PH
like left heart failure or COPD that can be associated with a
mild pulmonary hypertension. But those are much different
than IPAH,” said McLaughlin. Forms of PH caused by other
diseases may sometimes be referred to as “secondary PH.”
Systemic high blood pressure is common enough in the
population that some people with PH also have high blood
pressure; but systemic hypertension itself is not considered
a cause of PH.
The symptoms for primary and secondary PH may
be similar, and symptoms are usually more severe as the
disease progresses. Symptoms may include:
• Shortness of breath
• Chest pain (Angina pectoris)
• Exercise intolerance. Loss of energy.
• Swelling of the arms, legs, ankles or abdomen
Even minimal activity may provoke symptoms in
• Dry cough
• Raynaud’s phenomenon (chalky white or dusky blue
fingers that may be painful and can sometimes be
provoked by the cold)
advanced stages. Patients in advanced stages may experience
irregular heartbeat, a racing pulse, syncope and difficulty
breathing even at rest.
Diagnosis of pulmonary hypertension is sometimes
delayed because its main symptom — shortness of
breath — is also a symptom of other, more common and
less threatening conditions like asthma. Doctors may
look for those more common conditions first. In fact,
because PH is often initially misdiagnosed, patients may
go months or even years believing they have something
other than PH.
“The most common test that makes us start suspecting
PH is the echocardiogram where you might see an
enlargement of the right heart chambers and the Doppler
estimates of the pulmonary pressure,” McLaughlin said.
There are a number of preliminary tests for PH,
including blood tests to check the oxygen level, chest X-rays
to check for an enlarged heart and lung abnormalities,
electrocardiograms and echocardiograms to check the
functioning of the heart, lung function tests and exercise
All of these tests may point to a PH diagnosis,
but in order to diagnose it definitively, a right-heart
catheterization must be performed. This is the only test
“The most common test that makes us
start suspecting PH is the echocardiogram
where you might see an enlargement of
the right heart chambers and the Doppler
estimates of the pulmonary pressure.”
Pulmonary Hypertension Association
The Pulmonary Hypertension Association (PHA) is a
patient-founded and patient-led association that has
been around for 25 years. They work with about 275
patient-support groups nationwide and also provide
online continuing education for healthcare professionals.
Since 2014, PHA has accredited more than 40
Pulmonary Hypertension Care Centers and is evaluating
more. For a list of centers, visit their website.
Dr. Vallerie McLaughlin