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| | __NOTOC__ |
| | {| class="infobox" style="float:right;" |
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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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| Image = |
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| Caption = |
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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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| OMIM = |
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| MeshID = D006976 |
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| {{Pulmonary hypertension}} | | {{Pulmonary hypertension}} |
| {{CMG}}, Richard Channick, M.D.; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]], [[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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| | ==[[Pulmonary hypertension historical perspective|Historical Perspective]]== |
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| ==[[Pulmonary hypertension pathophysiology|Pathogenesis]]== | | ==[[Pulmonary hypertension classifications|Classification]]== |
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| | ==[[Pulmonary hypertension pathophysiology|Pathophysiology]]== |
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| ==[[Pulmonary hypertension causes|Causes]]== | | ==[[Pulmonary hypertension causes|Causes]]== |
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| ==[[Pulmonary hypertension history and symptoms|History & Symptoms]]== | | ==[[Pulmonary hypertension differential diagnosis|Differentiating Pulmonary hypertension from other Diseases]]== |
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| ==[[Physical Examination|Signs on Physical Examination]]== | | ==[[Pulmonary hypertension epidemiology and demographics|Epidemiology and Demographics]]== |
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| | ==[[Pulmonary hypertension risk factors|Risk Factors]]== |
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| ==Diagnosis== | | ==[[Pulmonary hypertension screening|Screening]]== |
| 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.
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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]].
| | ==[[Pulmonary hypertension natural history|Natural History, Complications and Prognosis]]== |
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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.
| | ==Diagnosis== |
| | | [[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]] |
| 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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| ==Imaging==
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| [[Pulmonary hypertension chest x ray|Chest x-ray]] | [[Pulmonary hypertension CT]] | [[MRI]] | [[Echocardiography or Ultrasound]] | |
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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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| ==[[Pulmonary hypertension epidemiology and demographics|Epidemiology]]==
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| ==[[Pulmonary hypertension medical therapy|Treatment]]== | | == Treatment == |
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| [[Pulmonary hypertension medical therapy|Medical therapy]] | [[Pulmonary hypertension surgery|Surgery]] | | [[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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| ==[[Pulmonary hypertension natural history|Prognosis]]== | | ==Case Studies== |
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| ==References==
| | [[Pulmonary hypertension case study one|Case #1]] |
| {{Reflist|2}}
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| == External links == | | == External links == |
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| [[pt:Hipertensão pulmonar]] | | [[pt:Hipertensão pulmonar]] |
| [[tr:Pulmoner yüksek tansiyon]] | | [[tr:Pulmoner yüksek tansiyon]] |
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