<unk> onocardiogram and jugular venous pulse tracing from a middle-aged man with<unk>  caused by cardiomyopathy. The jugular venous pulse tracing demonstrates a prominent a wave without a c or v wave being observed. The<unk> onocardiograms (fourth left interspace and cardiac apex) show a murmur of tricuspid insufficiency and ventricular and atrial gallops. Pulmonary artery catheter Severe tricuspid regurgitation In terms of the diagnosis of<unk> , it has five major types, and a series of tests must be performed to distinguish pulmonary arterial hypertension from venous, hypoxic, thromboembolic, or unclear multifactorial varieties. PAH is diagnosed after exclusion of other possible causes of<unk> .<unk> ysical examination A<unk> ysical examination is performed to look for typical signs of<unk>  (described above), and a detailed family history is established to determine whether the disease might be heritable. A history of exposure to drugs such as benfluorex (a fenfluramine derivative), dasatinib, cocaine, methamphetamine, ethanol leading to cirrhosis, and tobacco leading to emphysema is considered significant. Use of selective serotonin reuptake inhibitors during pregnancy (particularly late pregnancy) is associated with an increased risk of the baby developing persistent<unk>  of the newborn. Echocardiography If<unk>  is suspected based on the above assessments, echocardiography is performed as the next step. A meta-analysis of Doppler echocardiography for predicting the results of right heart catheterization reported a sensitivity and specificity of 88% and 56%, respectively. Thus, Doppler echocardiography can suggest the presence of<unk> , but right heart catheterization (described below) remains the gold standard for diagnosis of PAH. Echocardiography can also help to detect congenital heart disease as a cause of<unk> . File:UOTW 29 - Ultrasound of the Week 1.4-month-old with<unk>  as seen on ultrasound File:UOTW 29 - Ultrasound of the Week 2.4-month-old with<unk>  as seen on ultrasound File:UOTW 29 - Ultrasound of the Week 3.4-month-old with<unk>  as seen on ultrasound File:UOTW 29 - Ultrasound of the Week 4.4-month-old with<unk>  as seen on ultrasound File:UOTW 65 - Ultrasound of the Week 1.Long standing<unk>  Exclude other diseases If the echocardiogram is compatible with a diagnosis of<unk> , common causes of<unk>  (left heart disease and lung disease) are considered and further tests are performed accordingly. These tests generally include electrocardiography (ECG), pulmonary function tests including lung diffusion capacity for carbon monoxide and arterial blood gas measurements, X-rays of the chest and high-resolution computed tomography (CT) scanning. Ventilation/perfusion scintigraphy If heart disease and lung disease have been excluded, a ventilation/perfusion scan is performed to rule out CTEPH. If unmatched perfusion defects are found, further evaluation by CT pulmonary angiography, right heart catheterization, and selective pulmonary angiography is performed. CT scan Pulmonary artery hypertension and emphysema as seen on a CT scan with contrast Signs of<unk>  on CT scan of the chest are: * Enlargement of the pulmonary trunk (measured at its bifurcation). It is, however, a poor predictor of<unk>  in patients with interstitial lung disease. :*A diameter of more than 27 mm for women and 29 mm for men is suggested as a cutoff. :*A cutoff of 31.6 mm may be a more statistically robust in individuals without interstitial lung disease. * Increased ratio of the diameter of the main pulmonary artery (pulmonary trunk) to the ascending aorta (measured at its bifurcation). :*A ratio of 1.0 is suggested as a cutoff in adults. :*Cutoff ~1.09 in children. * Increased diameter ratio of segmental arteries to bronchi. This finding in three or four lobes, in the presence of a dilated pulmonary trunk (≥29 mm), and absence of significant structural lung disease confers a specificity of 100% for<unk> . * Mural calcification in central pulmonary arteries is most frequently seen in patients with Eisenmenger's syndrome. Right heart catheterization Although pulmonary arterial pressure (PAP) can be estimated on the basis of echocardiography, pressure measurements with a Swan-Ganz catheter inserted through the right side of the heart provide the most definite assessment.42<unk>  is defined as a mean PAP of at least 20 mm Hg (3300 Pa) at rest, and PAH is defined as precapillary<unk>  (i.e. mean PAP ≥ 20 mm Hg with pulmonary arterial occlusion pressure PAOP ≤ 15 mm Hg and pulmonary vascular resistance PVR > 3 Wood Units). PAOP and PVR 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; this can be used to calculate the cardiac index, which is far more important in measuring disease severity than the pulmonary arterial pressure. Mean PAP (mPAP) should not be confused with systolic PAP (sPAP), which is often reported on echocardiogram reports. A systolic pressure of 40 mm Hg typically implies a mean pressure of more than 25 mm Hg. Roughly, mPAP = 0.61•sPAP + 2. Due to the invasive nature of this procedure, the use of computational fluid dynamics based hemodynamic indices have been postulated. Other For people considered likely to have PAH based on the above tests, the specific associated condition is then determined based on the<unk> ysical examination, medical/family history and further specific diagnostic tests (for example, serological tests to detect underlying connective tissue disease, HIV infection or hepatitis, ultrasonography to confirm the presence of portal hypertension, echocardiography/cardiac magnetic resonance imaging for congenital heart disease, laboratory tests for schistosomiasis, and high-resolution CT for PVOD and pulmonary capillary hemangiomatosis). Routine lung biopsy is discouraged in patients with PAH, because of the risk to the patient and because the findings are unlikely to alter the diagnosis and treatment.
Pulmonary hypertension
Portopulmonary hypertension
Chronic thromboembolic pulmonary hypertension
Asthma
Chronic obstructive pulmonary disease
Emphysema
Hypertensive heart disease
Reactive airway disease