Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Primer

Transcription

1 Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Primer H. Page McAdams, MD Duke University Medical Center Durham, NC page.mcadams@duke.edu

2 Question Which of the following imaging tests is LEAST helpful for distinguishing CTEPH from other causes of pulmonary hypertension? a. CT angiography b. MR angiography c. Chest radiography d. Ventilation-Perfusion scintigraphy (V/Q) e. Thin-section CT

3 Pre-Capillary Pulm HTN Capillaries Pulmonary artery Pulmonary vein RV LA

4 Pre-Capillary Pulm HTN Capillaries Primary Pulmonary HTN Pulmonary artery Pulmonary vein Eisenmenger (ASD/VSD/PDA) Chronic Emboli (CTEPH) RV LA

5 CTEPH: Pathogenesis Normal fibrinolytic system lysis fragmentation more peripheral emboli organization recanalization

6 CTEPH: Pathogenesis Normal fibrinolytic system Occasionally fails chronic scarring secondary pulmonary hypertension (CTEPH)

7 CTEPH: Pathogenesis

8 CTEPH: Pathogenesis

9 CTEPH: Pathogenesis Normal fibrinolytic system Occasionally fails PAH develops enough small vessels occluded CTEPH develops 5% acute PE

10 CTEPH: Pathogenesis Normal fibrinolytic system Occasionally fails PAH develops Up to 50% have no history of previous embolic events

11 CTEPH Radiologic evaluation CXR V/Q scan all patients Chest CT/CTA

12 CTEPH Radiologic evaluation CXR V/Q scan Chest CT/CTA PAgram, RH cath MR imaging selected patients

13 CTEPH: CT (MR) Findings Enlarged PAs, right heart Peripheral, mural thrombus Webs, bands Small, occluded vessels Mosaic attenuation Collateral vessels

14 CTEPH: CT Findings Mural thrombus peripheral obtuse angles with vessel wall calcification uncommon

15 Webs CTEPH: CT Findings thin, central filling defects not acute emboli

16 Webs CTEPH: CT Findings thin, central filling defects not acute emboli MPRs to distinguish from bifurcation

17 Bands CTEPH: CT Findings circumferential constriction best seen on MPRs

18 CTEPH: CT Findings Small, occluded vessels

19 Mosaic attenuation CTEPH: CT Findings

20 CTEPH: CT Findings Collateral vessels

21 CTEPH: CT Findings Collateral vessels

22 CTEPH: Treatment Anticoagulation IVC filter Thromboendarterectomy substantially improve PAH, RH function CTA modality of choice for presurgical evaluation

23 CTEPH: Good Surgical Candidate Organized thrombi proximal to segmental arteries Clinical younger patients thrombophilic disorders

24 Severe PAH:? CTEPH

25 Severe PAH:? CTEPH

26 Severe PAH:? CTEPH

27 Severe PAH: CTEPH Good Surgical Candidate

28 Severe PAH:?CTEPH

29 Severe PAH:?CTEPH Severe IV Contrast Allergy

30 Severe PAH: CTEPH Good Surgical Candidate

31 CTEPH: Poor Surgical Candidate Doesn t have CTEPH Distal clot only Clinical older patients splenectomy, malignancy less likely to remember acute PE episode

32 Severe PAH:?CTEPH

33 Severe PAH:?CTEPH

34 Severe PAH:?CTEPH Poor Surgical Candidate Severe PAH: CTEPH (prob)

35 CTEPH: Summary Important, treatable cause of PAH Many have no prior history of PE Imaging evaluation key CTA modality of choice findings subtle, easily overlooked need to see most/all findings

36 Question Which of the following imaging tests is LEAST helpful for distinguishing CTEPH from other causes of pulmonary hypertension? a. CT angiography b. MR angiography c. Chest radiography d. Ventilation-Perfusion scintigraphy (V/Q) e. Thin-section CT

37 References Castañer E, et al. CT diagnosis of chronic pulmonary thromboembolism. Radiographics 2009; 29: Devaraj A, Hansell DM. Computed tomography signs of pulmonary hypertension: old and new observations. Clin Radiol 2009; 64: Frazier AA, et al. From the archives of the AFIP: pulmonary vasculature: hypertension and infarction. Radiographics 2000; 20: Grosse C, Grosse A. CT findings in diseases associated with pulmonary hypertension: a current review. Radiographics 2010; 30:

38 Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Primer H. Page McAdams, MD Duke University Medical Center Durham, NC page.mcadams@duke.edu