Initial evaluation:
(1) diagnosis of pulmonary hypertension, usually involve echocardiography
(2) detection of one or more segmental mismatched defects in a pulmonary perfusion scan
Angiographic patterns seen in patients with CTPH:
(1) "pouching" (concavity seen in an obstructing or partially obstructing thrombus). A complete obstruction of the main left or right pulmonary artery can mimic the appearance of unilateral pulmonary artery agenesis
(2) pulmonary arterial webs or bands (lines of decreased opacity that cross the width of the pulmonary vessel)
(3) intimal irregularities resulting in a scalloped appearance to the pulmonary arterial wall
(4) abrupt narrowing of major pulmonary vessels, particularly the main descending pulmonary arteries
(5) obstruction of lobar vessels at the point of origin from the main pulmonary artery
The typical patient will have several of these findings, usually bilaterally.
