Draft

Thalassemia and Pulmonary Hypertension individuals, it is important that patients undergo annual screening If I have PAH, what should I do to improve my overall health?
There are several things you can do to improve your health if youhave PAH. These include: Keep your weight at a healthy level.
Adopt a healthy diet that is high in fruits and vegetables and What is pulmonary hypertension?
low in animal fats and hydrogenated oils.
Pulmonary hypertension (often referred Pulmonary arteries Keep active. You don’t want to overdo things, but a modest amount of exercise can help. Consult with your physician to leading from the heart to the lungs) getnarrower. As a result, the blood PAH is a serious condition; however, PAH can usually be detected early and effective treatment begun in thalassemia patients in a clinical care setting with a team of experienced thalassemia The Thalassemia Clinical Research Network (TCRN) currently has an 1) muscles within the artery wall tightening, open trial to treat PAH with sildenafil or arginine. Patients with an 3) micro blood clots forming in the vessels causing blockage.
For more information about PAH or the TCRN trial, please contact So high blood pressure and PAH are the same?
CAF at (800) 522-7222 or info@cooleysanemia.org.
No. When we talk about “regular” hypertension (high bloodpressure), we’re usually talking about the reading you get when youstrap an inflatable cuff to your arm and measure the blood flowthere. In those cases, a normal reading is somewhere around120/80.
But a normal blood pressure reading in the pulmonary arteries ismuch lower, around 25/15. It’s possible to have both “regular” Published by the Cooley’s Anemia Foundation, 330 Seventh Ave., #900, hypertension and PAH, but it’s also possible to have a normal blood New York, NY 10001 www.cooleysanemia.org (800) 522-7222. pressure reading and still have PAH.
This publication is made possible by an unrestricted educational grant fromNovartis Pharmaceuticals.
What happens when the blood pressure in these pulmonary arteries
gets too high?
The information in this publication is for educational purposes only and is not intended The left side of the heart and the right side of the heart have to substitute for medical advice. You should not use this information to diagnose ortreat a health problem or disease without consulting a qualified health care provider. different functions. Simply put, the left ventricle pumps blood all The Cooley’s Anemia Foundation strongly encourages you to consult your health care around the body; it has to work harder and is more “muscular” than provider with any questions you may have regarding your condition. the right. The right ventricle pumps the blood to the nearby lungs; Thalassemia and Pulmonary Hypertension Thalassemia and Pulmonary Hypertension it doesn’t need to exert as much effort, so it is less “muscular.” How do I know if I have PAH?
In PAH, the pulmonary arteries, which lead from the right side of the In non-thalassemia cases, symptoms of PAH include shortness of heart to the lungs are narrowed. Therefore, the right ventricle of the breath with minimal exertion, fatigue, chest pain, dizzy spells and heart must exert greater pressure to circulate fainting. If you experience these symptoms, you should bring them to the attention of your physician. S/he may then suggest one ormore tests, ranging from a physical exam or an x-ray to an Working harder causes the right ventricle to get electrocardiogram or a cardiopulmonary exercise test. larger; after a period of time, it also becomesoverstressed, which in turn causes it to become However, many thalassemia patients with PAH are asymptomatic – weaker. When this happens, you have a right they don’t show any symptoms at all. For that reason, you should ventricle that has become larger than it should make sure that you undergo a complete comprehensive care be but, over time, has actually become weaker examination in association with a qualified Thalassemia Center of Excellence every year; such regular thorough exams, which include blood to the lungs. In some cases, this can an echocardiogram (also called ECHO), will often enable a physician to detect asymptomatic PAH in its early stages.
What are some of the other complications?
How is PAH diagnosed?
In addition to an enlarged right ventricle and An ECHO can be used to assess right heart pressures. However, the danger of heart failure, PAH can lead to assessment of right heart pressure is not routinely performed on a blood clotting or to fluid in the lungs. Both of standard ECHO. Therefore, patients should receive their annual ECHO at a Center of Excellence to ensure right heart pressures aremeasured. If right-sided pressures are elevated, your physician may What causes PAH?
suggest a cardiac catheterization to determine the exact pressure.
The cause of PAH is not always known. In some instances, it may bethe result of an underlying disease or condition, such as emphysema How is PAH treated?
or bronchitis. PAH in individuals with thalassemia is thought to be In typical (non-thalassemic) cases, there are a number of options related to conditions associated with thalassemia, such as a chronic available for treatment, including blood thinners, calcium channel anemia, hemolysis, the breakdown of blood cells and the increased blockers, blood vessel dilators and sildenafil. (Sildenafil is a drug, tendency to form clots, particularly micro clots in the lungs.
popularly known as Viagra, that helps to open up and widen blood Splenectomy patients may have a greater risk for PAH. The reasons PAH occurs in some people with thalassemia are still being studied.
However, the awareness of the occurrence of PAH in thalassemia is How does thalassemia complicate the risks associated with PAH?
still a relatively new development. The prognosis of PAH in Thalassemic individuals whose hemoglobin levels are consistently too thalassemia at this point is unclear, although several small scale low and are not being adequately transfused may be more studies have indicated that treatment with sildenafil is effective if susceptible to developing PAH. The bodies of patients with lower the PAH is diagnosed promptly. Several prospective trials are hemoglobin levels often try to compensate for the lack of ongoing which will provide physicians with a better understanding of hemoglobin via an increased heart rate which places additional effort the special characteristics of PAH in thalassemia, as well as its Some studies have indicated that PAH may be somewhat more There is a great deal about PAH in thalassemia that still needs to be common in patients with thalassemia intermedia than in those with learned; however, because it is common in individuals with thalassemia major. However, all individuals with thalassemia should thalassemia, and because it is so often asymptomatic in these be aware that it can become an issue for them.

Source: http://cooleysanemia.org/updates/PHT.1.pdf