Pulmonary hypertension

From Simple English Wikipedia, the free encyclopedia

Pulmonary hypertension or PH is a disease where there is high blood pressure in the lungs. The disease makes it hard to breathe. Some people with the disease need extra oxygen. This disease can also make a person dizzy and become easily tired. Some people with the disease faint easily. The symptoms get worse when exercising or working hard. Pulmonary hypertension is a serious disease, and it can be fatal. The disease makes it harder for the heart to pump blood. Because the heart has to work harder, it can also become sick. Some people who are very sick may need a lung transplant or a heart-lung transplant to live.

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[edit] Signs and symptoms

People with pulmonary hypertension have difficulty breathing. They also tire easily. Some of them faint easily too. They might have chest pain. Some patients have swelling of the feet and ankles. These symptoms get worse during exercise or hard work.

Because many diseases can make it hard to breathe, a doctor must learn about the patient's background. This helps the doctor to treat the patient, even if the patient has another disease. The doctor also does several tests. Pulmonary hypertension makes the heart sound different. One test is to measure the blood presssure inside the pulmonary artery, the blood vessel that goes from the heart to the lungs.

[edit] What goes wrong with the body

In pulmonary hypertension, the blood vessels in the lungs become too narrow. The blood pressure in the lungs becomes high. The heart works very hard to pump blood through the narrow blood vessels. Later on, the blood vessels in the lungs become hard and thick. The heart must work harder.

The heart can work so hard that it becomes sick. This is called heart failure. The sick heart cannot pump blood well. Less blood goes to the lungs, so the blood gets less oxygen. This makes it hard to breathe. This gets worse when exercising or working hard.

[edit] Causes

The most common cause of pulmonary hypertension is left heart failure. This causes pulmonary venous hypertension. This leads to pulmonary edema, or fluid build-up in the lungs.

Many illnesses can cause pulmonary arterial hypertension (PAH).

  • Lung diseases that make the blood have less oxygen, such as:
  • chronic obstructive pulmonary disease or COPD
  • interstitial lung disease
  • Pickwickian syndrome
  • sleep apnea
  • AIDS
  • scleroderma
  • other autoimmune disorders
  • cirrhosis
  • portal hypertension
  • other causes
  • taking pills to lose weight, such as Fen-Phen, Aminorex, fenfluramine (Pondimin), and phentermine[1]
  • sickle cell disease,[2]
  • congenital heart disease
  • thyroid diseases,[3]
  • taking drugs like cocaine
  • possibly Human herpesvirus 8[4]

When a person has pulmonary hypertension without any other cause, this is called idiopathic pulmonary arterial hypertension or IPAH. If that person has relatives with the same disease, this is called familial pulmonary arterial hypertension or FPAH.

When a family history exists, the disease is termed familial pulmonary arterial hypertension (FPAH). IPAH and FPAH are now considered to be genetic disorders linked to mutations in the BMPR2 gene, which encodes a receptor for bone morphogenetic proteins,[5] as well as the 5-HT(2B) gene, which codes for a serotonin receptor.[6]

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[edit] References

  1. Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;335:609-16. PMID 8692238.
  2. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95. PMID 14985486.
  3. Curnock AL, Dweik RA, Higgins BH, Saadi HF, Arroliga AC. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999;318:289-292. PMID 10555089.
  4. Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, Geraci MW, Brown KK, Routes JM, Tuder RM, Voelkel NF. Expression of Human Herpesvirus 8 in Primary Pulmonary Hypertension.N Engl J Med 2003;349:1113-22. PMID 13679525.
  5. Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44. PMID 10903931.
  6. Blanpain C, Le Poul E, Parma J, Knoop C, Detheux M, Parmentier M, Vassart G, Abramowicz MJ. Serotonin 5-HT(2B) receptor loss of function mutation in a patient with fenfluramine-associated primary pulmonary hypertension. Cardiovasc Res 2003;60(3):518-28. PMID 14659797.