During the past few months, we have not had quite as much time as usual to address our readers questions. Apologies for that. When I am not caring for pulmonary hypertension patients, I wear a critical care doctor’s hat and have been taking care of a growing number of gravely ill COVID patients. At Inspire Pulmonary Media and PulmonaryhypertnesionRN.com we appreciate our readers and hope that you are safe during this stressful time.
What is borderline pulmonary hypertension?
Usually we encounter this term in an echocardiogram report. The echocardiogram uses ultrasound waves to image the heart and estimate the pressure in the pulmonary arteries. When the pressure estimate is just around the upper limit of normal, the cardiologist will often report borderline pulmonary hypertension or mild pulmonary hypertension. In my experience most of the time, this is not PAH. A more thorough evaluation of the echocardiogram report is required to suggest PAH. I have written many articles on this site about diastolic heart failure and PH versus PAH. Click here to learn more about diastolic heart failure. Often having a consultation with a PAH specialist can clarify whether there is reason to worry about PAH.
What does the BNP test mean?
BNP and Nt-proBNP are blood tests that are commonly used to monitor patients with heart failure and PAH. BNP and NT-proBNP are hormones that are released by the right and left ventricle in response to stress (working too hard). The higher the value, the greater the stress the heart is experiencing. By itself, BNP or NT-proBNP can’t be used to diagnose PAH. In my practice, If I see a patient with an elevated BNP or NT-proBNP, the next step is to get an echocardiogram to further evaluate the situation.
Can second hand smoke lead to PAH?
Heavy second hand smoke exposure can lead to COPD/emphysema. Patients with severe COPD/emphysema can in turn develop pulmonary hypertension but this is not PAH. So, the short answer is that tobacco smoke exposure does not cause PAH. Regardless, second hand smoke is bad for your lungs and everyone should avoid smoking and second hand smoke.
Is hand swelling a symptom of PAH?
Although leg swelling is a common symptom of PAH, dramatic hand swelling is less commonly caused by PAH. If you are retaining a great deal of salt and water and your weight has increased, then you can expect to have swelling all over your body. Many patients tell me that they can tell when they are retaining fluid when their rings are tight. When their rings are loose, they generally are not retaining salt and water.
Can abnormal blood vessels cause PAH?
There are a variety of congenital heart diseases that involve an abnormal blood vessel in the pulmonary circulation. The most common abnormality is partial anomalous pulmonary venous drainage. In this disease, one of the pulmonary veins (vessel that is supposed to carry oxygenated blood back to the left side of the heart) drains into the right side of the heart. This malformation is often associated with a large hole between the right and left upper chambers called and atrial septal defect. Partial anomalous pulmonary venous drainage often leads to pulmonary hypertension. If this problem is identified early enough, the malformation may be corrected surgically and prevent the development of PAH. When identified later there may also be benefit to repairing the defect.
What are the symptoms of PAH in children?
Children, like adults with PAH, often complain of chest pains, shortness of breath and decreased exercise capacity. These are very non-specific symptoms and can be seen in many other diseases that are much more common than PAH. If after looking for some of the more common childhood disorders, PAH remains a concern, an echocardiogram is very helpful in determining if PAH should be considered.
What are the least expensive PAH medications?
In the United States, sildenafil is the least expensive medication for PAH. Using GoodRx.com, a month of sildenafil (ninety 20mg tablets) currently costs less than $20. Tadalafil is the next least expensive medication costing less than $40/month using GoodRx.com pricing. Outside the United States, these two medications are also the cheapest.
When should I be started on a second medication for PAH?
This is a particularly important question. First, it is important to recognize that every patient with PAH is unique. Some patients may have a little bit of PAH in a sea of other disease processes. For example, a patient with advanced lung disease and very mild pulmonary hypertension is very different from a patient without any other lung disease who has very severe PAH. The former patient may be optimally treated on single agent low dose sildenafil where the latter patient should be treated much more aggressively with two or perhaps three drugs.
If you are on a single medication for your PAH, the best strategy is to ask your PH doctor how you are doing and have them explain to you in detail how they see your PH and what other diseases are present. It is ok to ask if you would benefit from more treatment. Remember that more is not always better. Although multiple studies now confirm that for patients with substantial PAH, a PDE5 inhibitor and an endothelin receptor antagonist started together lead to better outcomes than one therapy, the devil is in the details. Every medication carries side effects. Getting an opinion from a Comprehensive Care Center of Excellence is always a good idea.