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Pulmonary Arterial Hypertension (PAH)
· Sustained pulmonary arterial pressure
· Pathogenesis: o Primary PAH (rare) = unknown cause · Diagnosis of exclusion o Clinically unexplained progressive pulmonary arterial hypertension without evidence for thromboembolic disease and pulmonary venoocclusive disease · Clinical o Age § 3rd decade; females > males o Dyspnea on exertion o Syncope o Easy fatigability o Hyperventilation o Chest pain · Imaging findings o Main pulmonary artery usually prominent o Right and left pulmonary arteries large and taper rapidly o Peripheral pulmonary arteries are narrow and inconspicuous o Diffuse oligemia of the lungs
Frontal radiograph of the chest shows an enlarged main pulmonary
artery
o No overinflation o Nuclear medicine § Diffuse patchy defects on perfusion scans (low probability) o On CT, main pulmonary artery diameter should be less than 30mm · Secondary PAH (more common) o Primary pleuropulmonic disease o Parenchymal pulmonary disease o COPD o Emphysema o Chronic bronchitis o Asthma o Bronchiectasis o Granulomatous disease o Cystic fibrosis o End-stage fibrotic lung o S/P lung resection o Idiopathic hemosiderosis o Alveolar proteinosis · Alveolar hypoventilation = hypoxic pulmonary arterial hyperperfusion o Chronic high altitude o Sleep apnea o Hypoventilation due to neuromuscular disease or obesity · Pleural disease and chest deformity o Fibrothorax o Thoracoplasty o Kyphoscoliosis · Primary vascular disease o Congenital heart disease § Increased flow: large L-R shunt § Decreased flow: Tetralogy of Fallot o Capillary obliteration § Chronic pulmonary thromboembolism § Persistent fetal circulation § Arteritides (eg, Takayasu) o Venous obliteration § Pulmonary venoocclusive disease o Pulmonary venous hypertension · Prognosis o Majority of patients have a median survival of 2-3 years
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