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==Diagnosis==
==[[Pulmonary hypertension 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.
 
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]].
 
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.
 
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.
 
Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12&ndash;16 mm Hg (1600&ndash;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.
 
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>).


==Imaging==
==Imaging==

Revision as of 21:52, 12 September 2011

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Pulmonary hypertension
ICD-10 I27.0, I27.2
ICD-9 416
DiseasesDB 10998
MeSH D006976

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Richard Channick, M.D.; Assistant Editor(s)-in-Chief: Ralph Matar, Lisa Prior, Ann Slater, R.N.

Overview

Pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery or lung vasculature, leading to 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.[1] It can be one of five different types, arterial, venous, hypoxic, thromboembolic, or miscellaneous.

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 Classification section below.


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Chest x-ray | Pulmonary hypertension CT | MRI | Echocardiography or Ultrasound


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References

  1. Romberg E von. Über Sklerose der Lungenarterie. Dtsch Arch Klin Med 1891-1892;48:197-206.

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