Do You Suffer From Pulmonary Hypertension

Even though the heart and the lungs help the body in different ways, they are also linked together in many unique ways. When the lungs develop a rise in blood pressure, known as pulmonary hypertension (PH), it can affect the heart as well. Patients suffering from PH may not be diagnosed for years. In fact, most patients are not diagnosed until almost three years of symptoms. Shortness of breathing, chest discomfort and passing-out can be signs of PH but certainly can be signs of other diagnoses as well.

PH can be associated with other disorders such as scleroderma, liver disease and even chronic lung disease such as emphysema and pulmonary fibrosis. Some patients will develop PH if they have used diet pills. Determining the cause of PH is important in order to find the right treatment. The usual evaluation of someone suspected of having PH will include breathing studies, laboratory blood testing and sometimes a heart catheterization. All patients will have an echocardiogram which is an ultrasound of the heart to get a good look at how the chambers of the heart are functioning.

Some patients will develop PH from having too many or too large blood clots in their lungs. This is known as a pulmonary embolism. In some patients with pulmonary embolisms, the blood clots do not dissolve as they should and this can lead to elevated pressures. Sometimes surgery is appropriate but other times medications will help.

In a smaller portion of patients, no primary cause of PH is found and they are termed “idiopathic.” This is simply a medical term for when no clear cause is known. This group of patients will sometimes be found to have a genetic cause of PH.

Once PH is diagnosed, patients then discuss their treatment options with a PH expert to determine what is appropriate as a next step. When the elevated pressures are secondary to something else, such as sleep apnea or emphysema, treatment is aimed at the underlying cause. In the case of emphysema or chronic obstructive lung disease, this can include inhalers, pulmonary rehabilitation and smoking cessation.

Some patients will benefit from oral medications. This first oral medication was approved by the FDA in 2001 and since then a number of additional medications are available. This can include inhaled medications but also infusion medications that are delivered continuously into a vein or subcutaneously similar to how an insulin pump works for diabetic patients. It should be pointed out that no PH medications should ever be given unless a heart catheterization confirms the severity and type of PH.

Follow-up of response to treatment is important and can include office visits with pulmonary hypertension physicians and nurses. Echocardiograms and breathing studies are commonly obtained on a yearly basis.

Involvement in clinical trial is also important for patients to consider. Some patient can be enrolled in trials of new medications and continued on these medications provided they have been proven successful. In fact, this year was remarkable in the PH community because three new oral medications were made available. One of these medications was unique in that it was the first of a new class of medications rather than an expansion of existing medications.

There are many resources for patients with PH. Local PH patients have been a part of the fight against PH at the national level through involvement with the Pulmonary Hypertension Association. This wonderful group is a patient-driven organization that brings patients and PH physicians together to improve the lives of those with PH.
If you believe you may have pulmonary hypertension, you should discuss it with your physician. Additionally, the Pulmonary Hypertension Association website is an excellent resource and can be found at phassociation.org.

Christian T. Tencza, MD
Fairfield Health Care Pulmonary Critical Care

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