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| | | [[File:Siren.gif|link=Pulmonary hypertension resident survival guide|41x41px]] |
| | | [[Pulmonary hypertension resident survival guide|'''Resident'''<br>'''Survival'''<br>'''Guide''']] |
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| '''For patient information click [[Pulmonary hypertension (patient information)|here]]''' | | '''For patient information click [[Pulmonary hypertension (patient information)|here]]''' |
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| {{Infobox_Disease |
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| Name = {{PAGENAME}} |
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| DiseasesDB = 10998 |
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| ICD10 = {{ICD10|I|27|0|i|26}}, {{ICD10|I|27|2|i|26}} |
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| ICD9 = {{ICD9|416}} |
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| MeshID = D006976 |
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| {{Pulmonary hypertension}} | | {{Pulmonary hypertension}} |
| '''Editor(s)-in-Chief:''' Richard Channick, M.D.; [[User:C Michael Gibson |C. Michael Gibson, M.S., M.D.]] [mailto:mgibson@perfuse.org] Phone:617-632-7753; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]], [[Ann Slater|Ann Slater, R.N.]] | | {{CMG}}; '''Assistant Editor(s)-in-Chief:''' {{Jose}}, [[User:Ralph Matar|Ralph Matar]], [[User:Lisa Prior|Lisa Prior]], [[Ann Slater|Ann Slater, R.N.]], [[User:Rim Halaby|Rim Halaby]], {{MM}} |
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| ==Overview==
| | {{SK}} Hypertensive pulmonary vascular disease; pulmonary arterial hypertension; PAH; hypertensive pulmonary vascular disease; Ayerza syndrome; Ayerza's syndrome; Ayerza-Arrilaga syndrome |
| '''Pulmonary hypertension''' (PH) is an increase in blood pressure in the [[pulmonary artery]] or [[lung]] [[Pulmonary circulation|vasculature]], leading to [[dypsnea|shortness of breath]], [[dizziness]], [[fainting]], and other symptoms, all of which are exacerbated by exertion. Depending on the cause, pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and right-sided [[heart failure]]. It was first identified by Dr Ernst von Romberg in 1891.<ref>Romberg E von. Über Sklerose der Lungenarterie. ''Dtsch Arch Klin Med'' 1891-1892;48:197-206.</ref> It can be one of five different types, ''arterial, venous, hypoxic, thromboembolic,'' or ''miscellaneous''.
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| Although the terms primary pulmonary hypertension (meaning of unknown cause) and secondary pulmonary hypertension (meaning due to another medical condition) still persist in materials disseminated to patients and the general public, these terms have largely been abandoned in the medical literature. This change has occurred because the older dichotomous classification did not reflect pathophysiology or outcome. It led to erroneous therapeutic decisions, i.e. treat "primary" pulmonary hypertension only. This in turn led to therapeutic nihilism for many patients labeled "secondary" pulmonary hypertension, and could have contributed to their deaths. The term "primary pulmonary hypertension" has now been replaced with "idiopathic pulmonary arterial hypertension". The terms "primary" and "secondary" pulmonary hypertension should not be used any longer. Further details are in the [[Pulmonary hypertension#Classification|Classification section]] below.
| | ==[[Pulmonary hypertension overview|Overview]]== |
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| ==Signs and symptoms== | | ==[[Pulmonary hypertension historical perspective|Historical Perspective]]== |
| A history usually reveals gradual onset of [[shortness of breath]], [[fatigue (physical)|fatigue]], non-productive [[cough]], [[angina pectoris]], [[fainting]] or syncope, peripheral [[oedema|edema]] (swelling of the limbs, especially around the ankles and feet), and rarely [[hemoptysis]] (coughing up blood). Pulmonary ''arterial'' hypertension ('''PAH''') typically does not present with [[orthopnea]] or [[paroxysmal nocturnal dyspnea]], while pulmonary ''venous'' hypertension typically does.
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| In order to establish the cause, the physician will generally conduct a thorough medical history. A detailed family history is taken to determine whether the disease might be familial. A history of exposure to [[cocaine]], [[methamphetamine]], [[alcohol]] leading to [[cirrhosis]], and smoking leading to [[emphysema]] are considered significant. [[Physical examination]] is performed to look for typical signs of pulmonary hypertension including a loud P2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal [[edema]], [[ascites]], [[Abdominojugular test|hepatojugular reflux]], [[clubbing]] etc. Physical examination evidence of [[tricuspid insufficiency]] is also sought and, if present, is consistent with the presence of long-standing pulmonary hypertension.
| | ==[[Pulmonary hypertension classifications|Classification]]== |
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| ==Differential Diagnosis of the Causes of Pulmonary Hypertension== | | ==[[Pulmonary hypertension pathophysiology|Pathophysiology]]== |
| *Alveolar hypoventilation
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| *[[Atrial septal defect]]
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| *[[Bronchiectasis]]
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| *[[Chronic obstructive pulmonary disease|COPD]]
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| *Collagen vascular diseases
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| *[[Cystic fibrosis]]
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| *[[Infection]]
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| *[[Interstitial lung disease]]
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| *[[Portal hypertension]]
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| *[[Pulmonary embolism]]
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| *Pulmonary venous obstruction
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| *[[Sarcoidosis]]
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| *[[Scleroderma]]
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| *[[Sickle cell disease]]
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| *[[Sleep apnea]]
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| ==Causes== | | ==[[Pulmonary hypertension causes|Causes]]== |
| The most common cause of pulmonary hypertension is left [[heart failure]] leading to pulmonary ''venous'' hypertension. This may be due to [[systolic]] or [[diastolic]] malfunction of the left [[Ventricle (heart)|ventricle]] or due to valvular dysfunction such as [[mitral regurgitation]] or [[mitral stenosis]]. It usually manifests as [[pulmonary edema]]. Because the malfunctioning heart does not pump efficiently, blood fails to leave the [[pulmonary circulation]] in a timely manner, leading to abnormally high pressure in the [[pulmonary vein]].<ref name="PH guide 51">Hayes, Gail Boyer. ''Pulmonary Hypertension Patient's Survival Guide'' 3rd edition, p. 51</ref> The increased pressure in the pulmonary vein can be carried through to the [[pulmonary artery]].<ref name="PH guide 51"/>
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| Common causes of pulmonary ''arterial'' hypertension (PAH) include [[HIV]], [[scleroderma]] and other [[autoimmune]] disorders, [[cirrhosis]] and [[portal hypertension]], [[sickle cell disease]],<ref>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.</ref> [[congenital heart disease]], and others. Use of weight loss pills such as [[Fen-Phen]], [[Aminorex]], [[fenfluramine]] (Pondimin), and [[phentermine]] led to the development of PAH in the past.<ref>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.</ref> Other causes include [[sarcoidosis]], [[histiocytosis X]], and fibrosing mediastinitis.
| | ==[[Pulmonary hypertension differential diagnosis|Differentiating Pulmonary hypertension from other Diseases]]== |
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| [[Pulmonary embolism]] also leads to pulmonary hypertension, acutely as well as chronically. When none of these causes can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH). There appears to be a link between IPAH and [[thyroid]] diseases,<ref>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.</ref>, but this is not regarded as causative. | | ==[[Pulmonary hypertension epidemiology and demographics|Epidemiology and Demographics]]== |
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| Lung diseases that lower oxygen in the blood ([[Hypoxia (medical)|hypoxia]]) are well known causes of pulmonary hypertension, including [[COPD]], [[interstitial lung disease]], [[Pickwickian syndrome]] or obesity-hypoventilation syndrome, and possibly [[sleep apnea]]. [[Human herpesvirus 8]], also associated with [[Kaposi's sarcoma]], has been demonstrated in patients with PAH, suggesting that this virus may play a role in its development.<ref>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.</ref> Recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.
| | ==[[Pulmonary hypertension risk factors|Risk Factors]]== |
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| When a family history exists, the disease is termed familial pulmonary arterial hypertension (FPAH). IPAH and FPAH are now considered to be [[genetic disorder]]s linked to mutations in the ''BMPR2'' gene, which encodes a [[receptor (biochemistry)|receptor]] for bone morphogenetic proteins,<ref>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.</ref> as well as the ''5-HT(2B)'' gene, which codes for a [[serotonin]] receptor.<ref>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.</ref>
| | ==[[Pulmonary hypertension screening|Screening]]== |
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| There seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and [[Trisomy 21]].
| | ==[[Pulmonary hypertension natural history|Natural History, Complications and Prognosis]]== |
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| ==Pathogenesis==
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| Whatever the initial cause, [[pulmonary hypertension]] involves the [[Vasoconstrictor|tightening of blood vessels]] connected to and within the lungs. This makes it harder for the heart to pump blood through the [[lungs]], much as it is harder to make water flow through a narrow pipe as opposed to a wide one. Over time, the affected blood vessels become both [[Fibrosis|stiffer and thicker]], further increasing the blood pressure within the lungs and impairing blood flow. In addition, the increased workload of the heart causes thickening and enlargement of the [[right ventricle]], making the heart less able to pump blood through the lungs, causing right [[heart failure]]. As the blood flowing through the lungs decreases, the left side of the heart receives less blood. This blood may also carry less oxygen than normal. Therefore it becomes harder and harder for the left side of the heart to pump to supply sufficient [[oxygen]] to the rest of the body, especially during physical activity.
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| ==Diagnosis== | | ==Diagnosis== |
| Because pulmonary hypertension can be of 5 major types, a series of tests must be performed to distinguish pulmonary ''arterial'' hypertension from ''venous, hypoxic, thomboembolic,'' or ''miscellaneous'' varieties.
| | [[Pulmonary hypertension diagnostic study of choice|Diagnostic Study of Choice]] | [[Pulmonary hypertension history and symptoms|History & Symptoms]] | [[Pulmonary hypertension physical examination|Physical Examination]] | [[Pulmonary hypertension laboratory tests|Laboratory Findings]] | [[Pulmonary hypertension electrocardiogram|Electrocardiography]] | [[Pulmonary hypertension chest x ray|Chest x-ray]] | [[Pulmonary hypertension CT|CT]] | [[Pulmonary hypertension MRI|MRI]] | [[Pulmonary hypertension echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Pulmonary hypertension right heart catheterization|Right heart catheterization]] | [[Pulmonary hypertension other diagnostic studies|Other Diagnostic Studies]] |
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| A [[physical examination]] is performed to look for typical signs of pulmonary hypertension. These include altered [[heart sounds]], such as a widely split S<sub>2</sub> or second heart sound, a loud P<sub>2</sub> or [[Pulmonary valve|pulmonic valve]] closure sound (part of the second heart sound), (para)sternal heave, possible S<sub>3</sub> or third heart sound, and pulmonary regurgitation. Other signs include jugular venous distension (enlargement of the [[jugular vein]]s), [[peripheral edema]] (swelling of the ankles and feet), [[ascites]] (abdominal swelling due to the accumulation of fluid), [[Abdominojugular test|hepatojugular reflux]], and [[clubbing]].
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| Further procedures are required to confirm the presence of pulmonary hypertension and exclude other possible diagnoses. These generally include [[pulmonary function test]]s, [[blood test]]s, [[electrocardiography]] (ECG), [[arterial blood gas]] measurements, [[X-ray]]s of the chest (followed by high-resolution [[CT scan]]ning if [[interstitial lung disease]] is suspected), and ventilation-perfusion or [[V/Q scan]]ning to exclude chronic thromboembolic pulmonary hypertension. Biopsy of the lung is usually not indicated unless the pulmonary hypertension is thought to be due to an underlying interstitial lung disease. But lung biopsies are fraught with risks of bleeding due to the high intrapulmonary blood pressure. Clinical improvement is often measured by a "six-minute walk test", i.e. the distance a patient can walk in six minutes. Stability and improvement in this measurement correlate with better survival.
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| Although pulmonary arterial pressure can be estimated on the basis of [[echocardiography]], pressure sampling with a [[Swan-Ganz catheter]] provides the most definite measurement. PAOP and PVR can not be measured directly with [[echocardiography]]. Therefore diagnosis of PAH requires a [[cardiac catheterization]]. A [[Swan-Ganz catheter]] can also measure the [[cardiac output]], which is far more important in measuring disease severity than the pulmonary arterial pressure.
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| Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12–16 mm Hg (1600–2100 Pa). Definite pulmonary hypertension is present when mean pressures at rest exceed 25 mm Hg (3300 Pa). If mean pulmonary artery pressure rises above 30 mm Hg (4000 Pa) with exercise, that is also considered pulmonary hypertension.
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| Diagnosis of PAH requires the presence of pulmonary hypertension with two other conditions. Pulmonary artery occlusion pressure (PAOP or PCWP) must be less than 15 mm Hg (2000 Pa) and pulmonary vascular resistance (PVR) must be greater than 3 Wood units (240 dyn•s•cm<sup>-5</sup> or 2.4 mN•s•cm<sup>-5</sup>).
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| ===Multi Sliced CT===
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| Images shown below are courtesy of RadsWiki and copylefted
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| <div align="left">
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| <gallery heights="175" widths="175">
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| Image:Pulmonary-artery-hypertension-101.jpg|MSCT: Pulmonary hypertension. Note increase in diameter of pulmonary artery.
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| Image:Pulmonary-artery-hypertension-102.jpg|MSCT: Pulmonary hypertension. Note increase in diameter of pulmonary artery.
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| </gallery>
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| </div>
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| ==Classification==
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| ===Current classification===
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| In 2003, the 3rd World Symposium on Pulmonary Arterial Hypertension was convened in Venice to modify the classification based on the new understanding of disease mechanisms. The revised system developed by this group provides the current framework for understanding pulmonary hypertension.
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| The system includes several improvements over the former 1998 Evian Classification system. Risk factor descriptions were updated, and the classification of congenital systemic-to pulmonary shunts was revised. A new classification of genetic factors in PH was recommended, but not implemented because available data were judged to be inadequate.
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| The Venice 2003 Revised Classification system can be summarized as follows:<ref>Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension. Venice, Italy, June 23-25, 2003. ''J Am Coll Cardiol'' 2004 Jun 16;43(12 Suppl S):1S-90S. PMID 15194171.</ref>
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| *WHO Group I - Pulmonary arterial hypertension (PAH)
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| *WHO Group II - Pulmonary hypertension associated with left heart disease
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| *WHO Group III - Pulmonary hypertension associated with lung diseases and/or hypoxemia
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| *WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/or embolic disease
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| *WHO Group V - Miscellaneous
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| ===Previous terminology===
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| The terms primary and secondary pulmonary hypertension (PPH and SPH) were formerly used to classify the disease. This led to the assumption that only the primary disease should be treated, and the secondary variety should be ignored in favor of treating only the underlying illness. In fact all forms of pulmonary arterial hypertension are treatable. Unfortunately, this classification system still persists in the minds of many physicians, and probably leads to many patients with being denied treatment. This approach to pulmonary arterial hypertension may also contribute to underdiagnosis. It is estimated that there are about 100,000 patients with PAH in the US, but only 15-20,000 have been diagnosed. Many others have been misdiagnosed as [[COPD]], [[asthma]], or [[congestive heart failure]].
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| The term primary pulmonary hypertension (PPH) has now been replaced with idiopathic pulmonary arterial hypertension (IPAH) in much of the medical literature. However, some physicians continue to use the older classification inappropriately.
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| ==Epidemiology==
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| IPAH is a rare disease with an incidence of about 2-3 per million per year and a prevalence of about 15 per million. Women are almost three times as likely to present with IPAH than men.
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| Other forms of PAH are far more common. In [[scleroderma]] the incidence has been estimated to be 6 to 60% of all patients, in [[rheumatoid arthritis]] up to 21%, in [[systemic lupus erythematosus]] 4 to 14%, in [[portal hypertension]] between 2 to 5%, in HIV about 0.5%, and in sickle cell disease ranging from 20 to 40%.
| | == Treatment == |
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| Diet pills such as [[Fen-Phen]] produced an annual incidence of 25-50 per million per year.
| | [[Pulmonary hypertension medical therapy|Medical Therapy]] | [[Pulmonary hypertension surgery|Surgery]] | [[Pulmonary hypertension primary prevention|Primary Prevention]] | [[Pulmonary hypertension secondary prevention|Secondary Prevention]] | [[Pulmonary hypertension cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Pulmonary hypertension future or investigational therapies|Future or Investigational Therapies]] |
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| ==Treatment== | | ==Case Studies== |
| Treatment is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. Since pulmonary ''venous'' hypertension is synonymous with [[congestive heart failure]], the treatment is to optimize left ventricular function by the use of [[diuretic]]s, [[beta blocker]]s, [[ACE inhibitor]]s, etc., or to repair/replace the [[mitral valve]] or [[aortic valve]].
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| In [[PAH]], lifestyle changes, [[digoxin]], [[diuretic]]s, oral [[anticoagulant]]s, and oxygen therapy are considered ''conventional'' therapy, but have never been proven to be beneficial in a randomized, prospective manner.
| | [[Pulmonary hypertension case study one|Case #1]] |
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| High dose [[calcium channel blocker]]s are useful in only 5% of IPAH patients who are ''vasoreactive'' by [[Swan-Ganz catheter]]. Unfortunately, calcium channel blockers have been largely misused, being prescribed to many patients with non-vasoreactive PAH, leading to excess morbidity and mortality.
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| ===Vasoactive substances===
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| Three major pathways are involved in the abnormal proliferation and contraction of the smooth-muscle cells of the pulmonary artery in patients with pulmonary arterial hypertension. These pathways correspond to important therapeutic targets in this condition and play a role in determining which of three classes of drugs — [[endothelin receptor antagonist]]s, [[phosphodiesterase]] type 5 inhibitors, and prostacyclin derivatives — will be used.
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| ====Prostaglandins====
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| [[Prostacyclin]] ([[prostaglandin]] I<sub>2</sub>) is commonly considered the most effective treatment for PAH. [[Epoprostenol]] (synthetic [[prostacyclin]], marketed as Flolan®) is given via continuous infusion that requires a semi-permanent [[central venous catheter]]. This delivery system can cause [[sepsis]] and [[thrombosis]]. Flolan® is unstable, and therefore has to be kept on ice during administration. Since it has a half-life of 3 to 5 minutes, the infusion has to be continuous (24/7), and interruption can be fatal. Other [[prostanoid]]s have therefore been developed. [[Treprostinil]] (Remodulin®) can be given intravenously or subcutaneously, but the subcutaneous form can be very painful. [[Iloprost]] (Ilomedin®) is also used in Europe intravenously and has a longer half life. [[Iloprost]] (marketed as Ventavis®) is the only inhaled form of prostacyclin approved for use in the US and Europe. This form of administration has the advantage of selective deposition in the lungs with less systemic side effects.
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| ====Endothelin receptor antagonists====
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| The dual (ET<sub>A</sub> and ET<sub>B</sub>) [[endothelin]] receptor antagonist [[bosentan]] (marketed as Tracleer®) was approved in 2001. Approved in June 2007, [[ambrisentan]] is marketed as Letairis® in U.S. by [[Gilead Sciences]].<ref>{{cite press release [[Sitaxsentan]], a selective endothelin receptor antagonist that blocks only the action of ET<sub>A</sub>, has been approved for use in Canada and the European Union, to be marketed under the name Thelin®.<ref name="Thelin">{{cite web |date=[[May 30]], [[2007]] |url=http://www.reuters.com/article/governmentFilingsNews/idUSBNG28335020070530 |title=UPDATE 1-Encysive gets Canadian approval for hypertension drug |publisher=Reuters |accessdate=2007-07-08}}</ref> Sitaxsentan has not been approved for marketing by the US FDA. A new trial is being planned to address FDA's concerns.
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| ====Phosphodiesterase type 5 inhibitors====
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| [[Sildenafil]], a selective inhibitor of [[cGMP specific phosphodiesterase type 5]] (PDE5), was approved for the treatment of PAH in 2005. It is marketed for PAH as Revatio®. [[Tadalafil]] (currently marketed as Cialis® for [[erectile dysfunction]]) is currently is Phase III [[clinical trial]]s.
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| ====Other agents====
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| [[Vasoactive intestinal peptide]] by inhalation should enter clinical trials for PAH in 2007. PRX-08066 is a serotonin antagonist currently being developed for hypoxic pulmonary hypertension.
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| ===Surgical===
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| Atrial septostomy is a surgical procedure that creates a communication between the right and left [[atria]]. It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood (hypoxia). It is best performed in experienced centers. [[Lung transplantation]] cures pulmonary arterial hypertension, but leaves the patient with the complications of transplantation, and a survival of about 5 years.
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| [[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension. It is the surgical removal of an organized [[thrombus]] (clot) along with the lining of the pulmonary artery; it is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
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| Treatment for hypoxic and miscellaneous varieties of pulmonary hypertension have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.
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| ==Prognosis==
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| The NIH IPAH registry from the 1980's showed an ''untreated'' median survival of 2-3 years from time of diagnosis, with the cause of death usually being right ventricular failure ([[cor pulmonale]]). Although this figure is widely quoted, it is probably irrelevant today. Outcomes have changed dramatically over the last two decades. This may be because of newer drug therapy, better overall care, and earlier diagnosis (lead time bias). A recent outcome study of those patients who had started treatment with bosentan (Tracleer®) showed that 86% patients were alive at 3 years. With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently. It would not be unreasonable to expect median survival to extend past 10 years in the near future.
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| ==References==
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| {{Reflist|2}}
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| == External links == | | == External links == |
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