These symptoms, which may be mild initially, can sometimes lead to a misdiagnosis, such asasthmaorheart failure.
These arteries carry blood from the right side of the heart through the lungs.
When this pressure gets too high, the arteries in the lungs become more narrow.
Dobrila Vignjevic / Getty Images
This limits blood flow, thus resulting in less oxygen in the blood.
Right heart failure shows up when the heart is too weak to pump adequate blood supply to the lungs.
PAH most commonly affects females and older adults over the age of 75.
Other symptoms of PAH include:
Causes
Experts don’t know precisely what causes PAH.
It may be genetic or brought on by certain toxins or drugs.
Pharmaceutical Treatment Options
The number oftreatment optionsfor PAH has grown over the past few decades.
Prostacyclin Analogs
Prostacyclin analogs were the first class of drugs approved for treating PAH.
They work by mimicking the effects of a natural hormone called prostacyclin.
Their mechanism of action includes stoppingplateletsfrom forming clots in your blood vessels and causing vasodilation of the pulmonary arteries.
When these blood vessels relax and widen, blood flows more efficiently, reducing the pressure.
Prostacyclin analogs are a standard long-term treatment for severe PAH.
Flolan (epoprostenol) was the first drug in this class.
It is used in severe cases if other treatments arent working well enough.
Prostacyclin analogs come in several forms.
An oral tablet option is also available as Orenitram (treprostinil).
Potentially serious side effects includehypotension (low blood pressure)and an increased risk of bleeding.
Its important not to stop or reduce the dosage of prostacyclin analogs abruptly.
Doing so may cause sudden worsening of PAH symptoms.
The inhalant forms of treprostinil are Tyvaso and Tyvaso DPI.
Tyvaso is an inhalant solution, while Tyvaso DPI is an inhalant power.
Endothelin is a small protein that causes blood vessels to constrict or narrow, thus raising blood pressure.
ERAs block endothelin-related effects on the smooth muscle cells lining your blood vessels.
ERAs are prescribed to help slow the progression of PAH.
In other words, PDE-5 inhibitors cause blood vessels to open wider, improving blood flow and decreasing pressure.
These medications are also used forerectile dysfunction.
Your body produces nitric oxide to relax blood vessels.
Nitric oxide attaches to receptors on blood vessel walls, triggering the production of cyclic GMP (cGMP).
cGMP signals your blood vessel muscles to relax, widening the vessels.
These drugs work by blocking an enzyme that would ordinarily break down cGMP.
Taking the drug causes cGMP to build up, amplifying its relaxing effects on the blood vessels.
PDE-5 inhibitors should not be taken withnitratesor riociguat due to the risk of dangerously low blood pressure.
PDE-5 inhibitors may also causesevere side effectssuch as vision changes andhearing loss.
This process is similar to the PDE5 inhibitors, so these two medicine classes aren’t taken together.
Adempas (riociguat) is the only drug in its class of soluble guanylate cyclase (sGC) stimulators.
Additionally, riociguat also offers protection againstfibrosis (scar tissue)in the lungs.
Irritation in the lungs can lead to fibrosis and inflammation.
However, riociguat can combat this and aid in diminishingpulmonary fibrosis.
Other PAH CCBs include Procardia XL (nifedipine) and Cardizem CD (diltiazem).
Some individuals may also experience heart palpitations.
However, these drugs arent specifically FDA-approved for this purpose.
Unfortunately, CCBs only seem helpful for a small percentage of people with PAH.
By regulating PAH cell growth, Winrevair increases exercise capacity and decreases the risk of PAH worsening in adults.
The goal of combination therapy is to target multiple pathways involved in PAH at the same time.
Typically, combination therapy is prescribed for people with severe PAH.
Combination therapies for PAH often include:
The choice between these two combination regimens depends on the patients needs.
Summary
Various treatment options are available for pulmonary arterial hypertension (PAH).
Oxygen therapycan also be used to increase oxygen levels in the blood.
In some cases, surgeries might be an option.
Treatment choices depend on the severity of your PAH (based on the WHO classification system).
A healthcare provider can discuss the best treatment plan for you.
Research is ongoing to develop new treatments for PAH.
Likewise, the Pediatric Pulmonary Hypertension web link continues investigating PAH treatment options for children.
However, trouble breathing is a common symptom of PAH.
Also known as blood thinners, these drugs help preventblood clotsfrom forming in the blood vessels.
Besides medications, some people may have surgeries to manage PAH.
Receiving alung transplantis an option for severe PAH.
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