Can echos be wrong?
Yes, if the echo is wrong it tends to underestimate the pulmonary artery pressure. The gold standard is the right heart catheterization. However when echos are done carefully by experience echo cardiographers the pressures estimated by echo correlate very closely to those obtained during cardiac catheterization.
Does PH as opposed to PPH have a better prognosis/life expectancy?
PPH by definition is PH whose cause is unknown. Therefore by definition all other PH has a known cause and is often referred to as secondary pulmonary hypertension (SPH). The prognosis is SPH depends on the specific cause and the severity. SPH due to collagen vascular diseases like scleroderma has a similar prognosis to PPH. SPH due to chronic pulmonary embolism when properly treated has an excellent prognosis with little or no shortening of life expectancy. Most of the other forms of SPH fall somewhere in between.
Explain cardiac output measurements.
Cardiac output is the amount of blood the heart pumps in one minute. In a healthy individual this amounts to about 5 quarts per minute. In patients with advanced forms of pulmonary hypertension, this amount is reduced to 2 to 3 quarts per minute. The most common way this measurement is made is by placing a catheter with a temperature-sensitive tip in the pulmonary artery and measuring the change in temperature of the blood during the injection of a small (1/3 oz.) amount of cold sterile water into a central vein.
My echo shows pressures of 50, but the right heart cath showed normal. Could I still have PH?
The right heart catheterization is the most accurate way to measure pulmonary artery pressure. However, an isolated measurement by either method may not give a true representation of the pressure over a 24 hour period during a variety of activities since pressure may fluctuate. In general whenever 2 test don’t confirm each other, or the results are mildly abnormal or ambiguous, it is important to recheck the tests periodically in the future before firm conclusions are made.
I was just diagnosed and am scared. How long do I have to live?
No physician has a crystal ball and therefore can not accurately predict how long you will live. Pulmonary hypertension is a serious disease that can shorten life expectancy and as with all medical conditions, prognosis also depends on the severity of the problem when it is diagnosed. The severity of pulmonary hypertension found at the time of initial diagnosis has a wide range. There are effective treatments that prolong life and improve symptoms. New treatments are currently under evaluation and may further improve the outlook for this disease.
If I have SPH, should I still see a PH specialist?
Treatments for primary pulmonary hypertension (PPH) are often successful in treating secondary forms of pulmonary hypertension (SPH). Therefore patients often receive optimal benefit when treated by physicians with experience with these treatments.
Could I have PPH which is mistakenly diagnosed as asthma?
The symptoms of PPH are largely non-specific, i.e. they can be associated with a number of lung and heart diseases. Pulmonary hypertension can usually be diagnosed by echocardiography, so if there is any question about your diagnosis discuss the appropriateness of this test for you with your doctor.
Yes pulmonary hypertension runs is families. Approximately 6-10% of cases of primary pulmonary hypertension are familial. Therefore whenever a patient is diagnosed with PPH all first order relatives (siblings, children and parents) should be screened for the disease. The best screening test is an echocardiogram.
Is my doctor right when he tells me I would get better if I lost weight?
If you are overweight, losing weight is likely to make you feel better what ever the cause of your symptoms. So the recommendation to lose weight is almost always a good one. One can not comment on the cause of your symptoms without knowing all the details of your case. In the earlier stages of pulmonary hypertension, the symptoms can be non-specific and the findings on physical examination subtle. Therefore in some cases they may be attributed to anxiety or depression. Obesity itself may contribute to elevated pulmonary pressures, so it is to one's benefit to achieve optimum weight.
My echo shows pressures of 50-60, but the doctor says I do not have PH, can they be wrong?
The echo measurement is only an approximation of pulmonary artery pressure, however if correct an echo measurement estimating a pulmonary artery pressure of 50-60 mm Hg is far above normal. Therefore further evaluation is probably appropriate.
Why is it important for me to see a PH specialist?
Pulmonary hypertension is a very uncommon but serious disease and most generalists have little training or experience in treating the disease.
Pulmonary hypertension is caused by certain forms of congenital heart disease, lung disease and blood clots in the lung arteries. It is also associated with collagen vascular disease, portal hypertension (usually caused by liver disease), diet drugs, HIV infection and some other rare diseases. In some cases no cause can be identified and these cases are called primary pulmonary hypertension (PPH).
What is the difference between mean PA pressure and PA pressures measured by an echo?
The mean PA pressure is an average of the pulmonary pressure during one heart cycle. See response 14. The echo estimates the systolic PA pressure which is the highest pressure measured during the heart cycle. Therefore systolic PA pressure is always higher than the mean PA pressure. The systolic pressure is often 30 - 50% higher than the mean PA pressure.
What is the mean pulmonary artery pressure and how do you get the number?
Pressure in the pulmonary (lung) arteries, like blood pressure in the body arteries rises when the heart "beats" and ejects blood into the pulmonary artery and then falls during the period of time before the next beat. Therefore the pressure is always changing. The mean pulmonary artery pressure is the arithmetic average of the pressure during one cardiac cycle which is the time from the beginning of one heart beat until the beginning of the next. However, it is not as simple as taking the top number and bottom number and taking the average of the two. It is a continuous average of the pressure throughout one cardiac cycle. The mean pressure is always closer to the bottom number than the top.
What is the life expectancy with PH?
There is no simple answer to this question. Life expectancy depends on many variables. Without knowing the specifics of each case it is impossible to even hazard a guess. Patients with severe forms of pulmonary hypertension have a shortened life-expectancy, but recently developed treatments can often help even these patients.15.
What tests are done to determine PH?
The gold standard test for diagnosing PH is a right heart catheterization which directly measures the pressures in the pulmonary arteries. Prior to performing this test a number of non-invasive tests may suggest the diagnosis, including the electrocardiogram, chest xray and echocardiogram.
What tests are done to determine PPH over SPH?
The diagnosis of PPH is made by excluding all known causes of SPH. Therefore testing is done to determine if there is an identifiable cause of the pulmonary hypertension. If one or more of these tests are positive then the diagnosis is SPH. If the tests are all negative then the diagnosis is PPH. In most cases of SPH or PPH the number of tests done are about the same.
When should I start treatment if I have been diagnosed with mild PH?
This is a difficult question to answer without knowing all of the facts in your case, but in general most cases of pulmonary hypertension receive some form of treatment. In mild cases it is often a calcium channel blocker drug and a blood thinner, warfarin. Physicians still do not have a clear picture of what happens to someone with "mild&" pulmonary hypertension, unlike the experience with mild systemic blood pressure elevation. So conservative treatment is based mostly on "common sense" and careful follow-up is required.
Where can I find a PH specialist in my city or state?
The PHA provides a list with addresses of physicians who are board certified in cardiology, pulmonary medicine or pediatric cardiology who have indicated they have a special interest in treating pulmonary hypertension.
Why do I have chest pains with PPH?
Chest pain is a fairly common symptom in patients with PPH, occurring in at least 1/3 of patients. The cause is unknown, although many experts in the field suspect it is because the muscle wall of the right- sided pumping chamber of the heart, the right ventricle, is not getting enough blood supply to meet the needs of the increased work it has to do because of the high pulmonary artery pressure.
My doctor is either a cardiologist or a pulmonologist. Why do I need to see a specialist?
They are the proper specialists. However some of these specialist have very little training or experience in treating patients with pulmonary hypertension because it is an uncommon disease. If a heart or lung specialist has little experience in treating this disease, it is often helpful to have the patient have a consultation with an experienced specialist. The pulmonary hypertension specialist can communicate with the local specialist and the patient ends up with the best possible care.
What meds are the most commonly used for treatment?
Currently, the mainstays of treatment are the calcium channel blocking drugs and prostacyclin. Diuretics, digoxin and oxygen are also helpful in advanced cases. Most patients are also treated with the anticoagulant, warfarin.