Presentation
Progressive shortness of breath
Patient Data
Severe enlargement of the pulmonary arteries with thin peripheral calcification. The contours are smooth and relatively spherical, rather than lobulated as seen in hilar lymphadenopathy.
Heart size is on the upper limit of normal.
No evidence of interstitial lung disease. No dilated esophagus to suggest an underlying connective tissue disorder.
Severe enlargement of the central pulmonary arteries with calcified atherosclerosis. Surprisingly, no right ventricular dilation or hypertrophy. No appreciable intraventricular or intra-atrial septal defect.
Geographic mosaic attenuation of the lungs as a result of a paucity of peripheral pulmonary arteries. No pulmonary fibrosis or emphysema.
Incidental hepatic cysts.
Case Discussion
This case demonstrates features of severe pulmonary hypertension. This was a case of idiopathic pulmonary arterial hypertension.
In an exam look for common causes of Pulmonary hypertension such as:
- pulmonary parenchymal disease (pulmonary fibrosis, emphysema, cystic fibrosis)
- pulmonary vessels (chronic pulmonary thromboembolism)
- cardiac (L>R shunts, mitral stenosis)
- connective tissue disorders (Scleroderma with dilated esophagus)
And consequences such as:
Unable to process the form. Check for errors and try again.