Pulmonary
hypertension (PH) is an
increase of blood pressure in the pulmonary artery, pulmonary vein, or
pulmonary capillaries, together known as the lung vasculature, leading to
shortness of breath, dizziness, fainting, leg swelling and other symptoms. Pulmonary
hypertension can be a severe disease with a markedly decreased exercise
tolerance and heart failure. It was first identified by Ernst von Romberg in
1891. According to the most recent
classification, it can be one of six different types.
Because pulmonary hypertension can be of five major types, a series of tests must be performed to distinguish pulmonary arterial hypertension from venous, hypoxic, thromboembolic, or miscellaneous varieties.
Further procedures are required to confirm the presence of pulmonary hypertension and exclude other possible diagnoses. These generally include pulmonary function tests; blood tests to exclue HIV, autoimmune diseases, and liver disease; electrocardiography (ECG); arterial blood gas measurements; X-rays of the chest (followed by high-resolution CT scanning if interstitial lung disease is suspected); and ventilation-perfusion or V/Q scanning 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; further, lung biopsies are fraught with risks of bleeding due to the high intrapulmonary blood pressure.
Diagnosis
Because pulmonary hypertension can be of five major types, a series of tests must be performed to distinguish pulmonary arterial hypertension from venous, hypoxic, thromboembolic, or miscellaneous varieties.
Further procedures are required to confirm the presence of pulmonary hypertension and exclude other possible diagnoses. These generally include pulmonary function tests; blood tests to exclue HIV, autoimmune diseases, and liver disease; electrocardiography (ECG); arterial blood gas measurements; X-rays of the chest (followed by high-resolution CT scanning if interstitial lung disease is suspected); and ventilation-perfusion or V/Q scanning 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; further, lung biopsies are fraught with risks of bleeding due to the high intrapulmonary blood pressure.
Diagnosis of PAH
requires the presence of pulmonary hypertension. Although pulmonary arterial
pressure can be estimated on the basis of echocardiography, pressure
measurements with a Swan-Ganz catheter through the right side of the heart
provides the most definite assessment. PAOP (pulmonary artery occlusion pressure)
and PVR (pulmonary vascular resistance) cannot be measured directly with echocardiography.
Therefore diagnosis of PAH requires right-sided 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.
Physical Examination
A physical
examination is performed to look for typical signs of pulmonary hypertension.
These include altered heart sounds, such as a widely split S2 or
second heart sound, a loud P2 or pulmonic valve closure sound (part
of the second heart sound), (para)sternal heave, possible S3 or third
heart sound, and pulmonary regurgitation. Other signs include an elevated jjugular
venous pressure, peripheral edema (swelling of the ankles and feet), ascites
(abdominal swelling due to the accumulation of fluid), hepatojugular reflex,
and clubbing.
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