Pulmonary hypertension needs close care

DEAR DR. GOTT: In a recent column, you addressed the condition of pulmonary hypertension and said to see a pulmonologist and get a chest X-ray to detect this. I did just that for my 9-year-old daughter, but the pulmonologist did not detect pulmonary hypertension from the results of the X-ray. He felt her breathing issue was due to allergies and treated her with allergy medication.

Thank goodness for her pediatrician. She felt something was still wrong and sent her for an echo. The technician immediately caught that she had something wrong with her and only told me that the results from the echo were sent to a pediatric cardiologist to be read. So the diagnosis came from a cardiologist, who is the one who treated the pulmonary hypertension.

I felt compelled to write to you because this disease is so rare. If left untreated, the person can die in just years. Since my daughter was diagnosed in September 2009, I have been doing a lot of reading about pulmonary hypertension. If you want to pass along a great website to anyone who asks about this condition, I recommend www.PHAssociation.org. My daughter’s doctor is affiliated with the Children’s Hospital of Philadelphia, and this is the only website he totally endorses for information regarding PH.

DEAR READER: Pulmonary hypertension is a complex condition. It is defined as continuous elevation of blood pressure in the pulmonary arteries. It can lead to an enlarged heart, which may eventually lose its ability to pump blood throughout the body.

There are two major categories of PH. The first is idiopathic. This type occurs sporadically in the population or is genetic. The second type is associated with other disorders, such as scleroderma, lupus, HIV, sickle-cell anemia and others, but it may also be associated with the use of illicit drugs. It can affect both children and adults but is most common in women. It is often misdiagnosed several times because early symptoms are not specific and can be attributed to other, more common conditions such as asthma or allergies.

According to the Pulmonary Hypertension Association, as many as 100,000 Americans may have PH, but thousands remain undiagnosed, and even more are misdiagnosed, delaying treatment. Diagnosis can be confirmed by several tests. A chest X-ray can be used to determine if the pulmonary arteries and right ventricle are enlarged. An echocardiogram (heart ultrasound) can show the size and thickness of the right ventricle, determine how well it is working, and estimate the pressure in the pulmonary arteries. An electrocardiogram (EKG) shows the heart rate, whether it is abnormal and may show whether the right ventricle is enlarged or strained. Right-heart catheterization can find leaks between the left and right side of the heart and show how well the heart is pumping. Unlike the other tests, this procedure is invasive, requiring insertion of a long, flexible tube into a major artery, which is then threaded into the right ventricle. It also carries rare but serious risks, such as heart attack or stroke.

Once diagnosis is made, testing to determine whether there is an underlying cause and what it is may be done. This can include lung-function tests, blood tests and more. Exercise testing is used to determine the severity of PH. There are four classes. The first has no limits, meaning activity does not cause PH symptoms. The second has slight or mild limits, meaning regular physical activity causes symptoms, and while at rest there are none. Class three continues to present no symptoms at rest, yet activity (even limited amounts) can cause symptoms. Class four is the most severe. Any physical activity can cause symptoms that may be present, even at rest.

There are several treatment options available today. The prognosis for treated adults is upward of 15 to 20 years. Children undergoing treatment have an even better outcome than adults. Women with PH should not become pregnant and give birth because there are life-threatening risks to both mother and child, even with treatment. There is also a risk of the child developing pulmonary hypertension. A review of reports published between 1978 and 1996 found that between 30 percent and 56 percent of women with PH died within 35 days after childbirth.

Unfortunately, you and your daughter have experienced a fairly typical road to diagnosis. Unless a physician knows to look for it, PH is often misdiagnosed as something else. I congratulate your daughter’s pediatrician for sticking to her guns with regard to diagnosis.

Everyone diagnosed with PH should be under the care of a pulmonary-hypertension specialist. Most of these specialists are cardiologists, rheumatologists and pulmonologists, but they can be any physician or nurse who has undergone special training in the diagnosis and treatment of PH.