I just learned that an echocardiogram from 2.5 years ago indicated “mild pulmonary hypertension.” (sPAP was extimated at 38 mmHg.) The echo was done to try to diagnose sudden attacks of shortness of breath that have become less frequent since then. (No luck with a diagnosis, by the way.) My brand new pulmonologist wants to do another echo and a CT angiogram to try diagnosing again. Should I be concerned that over 2 years passed with no further exploration of such a potentially deadly disease, or are we even now acting in a timely manner? Are there other tests that make sense as well?
I think it is definitely worthwhile to repeat these tests especially the ECHO. I assume the CT is of your lung and its circulation.
Before I go further, my first question is about what you were told at the time, and why wasn’t it repeated in 3-6 months? An ECHO is only a snapshot of what is going on at the time and needs to be viewed in context of other clinical information!
Since your shortness of breath has improved, have you noticed any decreased exercise tolerance or swelling in your legs? Most patients with PH initially experience exertional shortness of breath, lethargy, and fatigue, that may progress if the right side of the heart begins to fail.
To reassure you, and before we go further, it is unlikely that your shortness of breath would improve with PH getting worse!
However to help you understand PH better, let me review it here. First you need to know more about your heart, including the fact it has two sides, a left and a right side, that work in series with each other. Read here how your heart works:
Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure and secondary failure of the right side of the heart. It is a life-threatening condition with a poor prognosis if untreated.
PH is associated with a variety of factors:
- The diet drug “fen-phen.” Although the appetite suppressant “fen-phen” (dexfenfluramine and phentermine) has been taken off the market, former fen-phen users have a 23-fold increase risk of developing pulmonary hypertension, possibly years later.
- Liver diseases, rheumatic disorders, lung conditions. Pulmonary hypertension also can occur as a result of other medical conditions, such as chronic liver disease and liver cirrhosis; rheumatic disorders such as scleroderma or systemic lupus erythematosus (lupus); and lung conditions including tumors, emphysema, chronic obstructive pulmonary disease (COPD), and pulmonary fibrosis.
- Certain heart diseases. Heart diseases including aortic valve disease, left heart failure, mitral valve disease, and congenital heart disease can also cause pulmonary hypertension.
- Thromboembolic disease. A blood clot in a large pulmonary artery can result in the development of pulmonary hypertension.
- Low-oxygen conditions. High altitude living, obesity, and sleep apnea can also lead to the development of pulmonary hypertension.
- Genetic predisposition. Pulmonary hypertension is inherited in a small number of cases. Knowing that someone in the family had or has pulmonary hypertension should prompt you to seek early evaluation should symptoms occur.
- A variety of systemic illnesses elsewhere that may involve the lungs
Most patients with PH initially experience exertional shortness of breath, lethargy, and fatigue, that may progress if the right side of the heart begins to fail:
A diagnostic evaluation should begin with an echocardiogram (ECHO) just as what happened with you. If you really have a problem, a repeat ECHO would show progression of disease during this interval. In addition there care a variety of other tests that might be ordered include:
- Complete metabolic panel (CMP): Examines liver and kidney function
- Autoantibody blood tests, such as ANA, ESR, and others: Screens for collagen vascular diseases
- Thyroid stimulating hormone (TSH): A screen for thyroid problems
- HIV: A screen for human immunodeficiency virus
- Arterial blood gases (ABG): Determines the level of oxygen in arterial blood.
- Complete blood count (CBC): Tests for infection, elevated hemoglobin, and anemia
- B-type natriuretic peptide (BNP): A marker for heart failure
Cardiac ECHO: Uses sound waves to show the function of the right ventricle, to measure blood flow through the heart valves, and then calculate the systolic pulmonary artery pressure.
Chest X-ray: Shows an enlarged right ventricle and enlarged pulmonary arteries.
6 minute walk test: Determines exercise tolerance level and blood oxygen saturation level during exercise.
Pulmonary function tests: Evaluates for other lung conditions such as chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis among others.
Polysomnogram or overnight oximetry: Screens for sleep apnea (results in low oxygen levels at night).
Right heart catheterization: Measures various heart pressures (ie, inside the pulmonary arteries, coming from the left side of the heart), the rate at which the heart is able to pump blood, and finds any leaks between the right and left sides of the heart.
Ventilation perfusion scan (V/Q scan): Looks for evidence of blood clots along the pathway to the lungs.
Pulmonary angiogram: Looks for blood clot blockages in the pulmonary arteries.
Chest CT scan: Looks for blood clots and other lung conditions that may be contributing to or worsening pulmonary hypertension.
If it shows a problem with the left side of your heart then treatment is aimed correction or control of your heart problem. Your previous ECHO probably did not show a problem on the left side, or you would have been told about it.
If this part is cleared, the next step involves your lungs and the circulation to your lungs. Because there so many prossibilites, it is useless to speculate what might be the case: the CAT scan will give a lot of information that will dictate the next steps.
Once a diagnosis is made (may take a number of more tests), treatment consists of correcting the underlying problem if possible::
Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depend on the severity of pulmonary hypertension, the likelihood of progression, and individual drug tolerance.
- Oxygen — replaces the low oxygen in your blood.
- Anticoagulants or “blood thinners” such as warfarin sodium (Coumadin) — decreases blood clot formation so blood flows more freely through blood vessels. Note: when taking anticoagulant medications, it is important for you to monitor bleeding complications and have regular lab work to monitor the level of medication in your bloodstream.
- Diuretics or “water pills” [such as furosemide (Lasix)] — removes extra fluid from the tissues and bloodstream, which reduces swelling and makes breathing easier.
- Potassium (such as K-dur) — replaces potassium (an essential nutrient) that may be lost with increased urination when taking diuretics.
- Inotropic agents (such as digoxin) — improves the heart’s pumping ability.
- Vasodilators [such as nifedipine (Procardia) or diltiazem (Cardizem)] — lowers pulmonary blood pressure and may improve the pumping ability of the right side of the heart.
- Bosentan (Tracleer) — helps block the action of endothelin, a substance that causes narrowing of lung blood vessels.
- Epoprostenol (Flolan), treprostinil sodium (Remodulin), iloprost (Ventavis) — dilates pulmonary arteries and helps prevent blood clots from forming.
- Sildenafil (Revatio) — relaxes pulmonary smooth muscle cells, which leads to dilation of the pulmonary arteries.
If present, a large clot in the pulmonary artery may be surgically removed to improve blood flow and lung function.
Currently, this is the only cure for primary pulmonary hypertension. Transplantation is reserved for advanced pulmonary hypertension that is not responsive to medical therapy. The right side of the heart will generally return to normal after the lung/lungs have been transplanted. About 1,000 lung transplants are performed annually in the United States. Many people are on the waiting list, yet a shortage of donors is the major limiting factor. Your health care provider will discuss transplantation if it is an appropriate treatment option for your condition.
Heart / lung transplantation
This type of double organ transplant is very rare but may be necessary for patients who have combined lung and left heart failure.
Hope this helps,