Konstantinides et al reviewed clinical findings seen in patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
Clinical features:
(1) The patient may or may not have a history of acute pulmonary emboli.
(2) Initial symptoms are nonspecific with a significant delay in diagnosis.
Criteria for diagnosis:
(1) > 3 months of therapeutic anticoagulation
(2) tricuspid regurgitation velocity > 2.8 m/s on echocardiography
(3) positive findings (at least 1 defect in segmental or larger bronchial arteries) on imaging study (V/Q scan, MDCT angiography, pulmonary cineangiography)
(4) mean pulmonary arterial pressure >= 25 mm Hg with pulmonary arterial wedge pressure <= 15 mm Hg
Findings on pulmonary angiography:
(1) pulmonary artery webs and bands
(2) vascular wall irregularities
(3) stenoses
(4) aneurysms
(5) complete vascular obstructions
(6) collaterals
Differential diagnosis:
(1) acute or subacute pulmonary embolism
(2) other causes of pulmonary hypertension
