Chronic Thromboembolic Pulmonary Hypertension (Suspected)
Pathway Home
Population Covered By The Guidance
This pathway provides guidance on the imaging of adult patients with suspected chronic thromboembolic pulmonary hypertension.
Date reviewed: November 2018
Date of next review: August 2023
Published: March 2019
Quick User Guide
Move the mouse cursor over the PINK text boxes inside the flow chart to bring up a pop up box with salient points.
Clicking on the PINK text box will bring up the full text.
The relative radiation level (RRL) of each imaging investigation is displayed in the pop up box.
SYMBOL | RRL | EFFECTIVE DOSE RANGE |
![]() |
None | 0 |
![]() |
Minimal | < 1 millisieverts |
![]() |
Low | 1-5 mSv |
![]() |
Medium | 5-10 mSv |
![]() |
High | >10 mSv |
Teaching Points
Teaching Points
- A diagnosis of CTEPH was previously associated with a poor prognosis, however it is now potentially curable with surgery or balloon pulmonary angioplasty. Improvements in medical and endovascular treatments have led to some improved survival in inoperable or refractory CTEPH
- Lung scintigraphy (V/Q or ventilation/perfusion scan) is the first-imaging modality to investigate suspected CTEPH. A normal V/Q scan rules out a diagnosis of CTEPH with high certainty. CTEPH may still be present with an intermediate scan
- CT pulmonary angiography (CTPA) alone frequently misses the diagnosis of CTEPH so it is not recommended to rule it out
- Possible cases of CTEPH or pulmonary hypertension due to other causes should be referred to a specialist centre for further evaluation
- Transthoracic echocardiogram (TTE) may suggest pulmonary hypertension, but invasive measurement of right heart pressures and pulmonary artery wedge pressures with right heart catheterisation is required to confirm the diagnosis
- The role of CTPA or magnetic resonance pulmonary angiography (MRPA) and cardiac MRI is to assess disease severity, distribution and location to allow planning for surgery or radiological interventions and to assess right ventricular volume and function as a marker for treatment response. The chance of success with surgery is greatest when vascular obstruction is present in the main pulmonary arteries or lobar arteries. Balloon pulmonary angioplasty is successful at treating segmental and subsegmental arteries
- Catheter pulmonary angiography remains the gold standard for diagnosis and treatment work up and all patients require this to plan intervention
References
References
References are graded from Level I to V according to the Oxford Centre for Evidence-Based Medicine, Levels of Evidence. Download the document
- Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A, Delcroix M, Pruszczyk P, Mairuhu AT, et al. Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature. Eur Respir J. 2017;49(2) (Level I evidence). View the reference
- Condliffe R, Kiely DG, Gibbs JS, Corris PA, Peacock AJ, Jenkins DP, et al. Prognostic and aetiological factors in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):332-8. (Level III evidence). View the reference
- Delcroix M, Lang I, Pepke-Zaba J, Jansa P, D'Armini AM, Snijder R, et al. Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation. 2016;133(9):859-71. (Level II evidence). View the reference
- Pepke-Zaba J, Delcroix M, Lang I, Mayer E, Jansa P, Ambroz D, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973-81. (Level II evidence). View the reference
- Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119. (Guideline). View the reference
- Konstantinides SV, Torbicki A, Agnelli G, Danchin N, Fitzmaurice D, Galie N, et al. 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J. 2014;35(43):3033-69, 69a-69k. (Guideline). View the reference
- Lang IM, Simonneau G, Pepke-Zaba JW, Mayer E, Ambroz D, Blanco I, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study. Thromb Haemost. 2013;110(1):83-91. (Level III evidence) View the reference
- Pengo V, Lensing AWA, Prins MH, Marchiori A, Davidson BL, Tiozzo F, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350(22):2257-64. (Level II evidence). View the reference
- Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48(5):680-4. (Level II-III evidence). View the reference
- Worsley DF, Palevsky HI, Alavi A. Ventilation-perfusion lung scanning in the evaluation of pulmonary hypertension. J Nucl Med. 1994;35(5):793-6. (Level II-III evidence). View the reference
- Rajaram S, Swift AJ, Telfer A, Hurdman J, Marshall H, Lorenz E, et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax. 2013;68(7):677-8. (Level II-III evidence). View the reference
- Donkers-van Rossum AB. Diagnostic strategies for suspected pulmonary embolism. The European respiratory journal. 2001;18(3):589-97. (Review article). View the reference
- Klok FA, Delcroix M, Bogaard HJ. Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism. J Thromb Haemost. 2018 (Review article). View the reference
- Litmanovich D, Tack D, Lin PJ, Boiselle PM, Raptopoulos V, Bankier AA. Female breast, lung, and pelvic organ radiation from dose-reduced 64-MDCT thoracic examination protocols: a phantom study. AJR American journal of roentgenology. 2011;197(4):929-34. (Level III evidence). View the reference
- Szucs-Farkas Z, Schibler F, Cullmann J, Torrente JC, Patak MA, Raible S, et al. Diagnostic accuracy of pulmonary CT angiography at low tube voltage: intraindividual comparison of a normal-dose protocol at 120 kVp and a low-dose protocol at 80 kVp using reduced amount of contrast medium in a simulation study. AJR American journal of roentgenology. 2011;197(5):W852-9. (Level III evidence). View the reference
- Szucs-Farkas Z, Kurmann L, Strautz T, Patak MA, Vock P, Schindera ST. Patient exposure and image quality of low-dose pulmonary computed tomography angiography: comparison of 100- and 80-kVp protocols. Investigative radiology. 2008;43(12):871-6. (Level III evidence). View the reference
- Zamboni GA, Guariglia S, Bonfante A, Martino C, Cavedon C, Mucelli RP. Low voltage CTPA for patients with suspected pulmonary embolism. European journal of radiology. 2012;81(4):e580-4. (Level III evidence). View the reference
- Ruggiero A, Screaton NJ. Imaging of acute and chronic thromboembolic disease: state of the art. Clin Radiol. 2017;72(5):375-88. (Review article). View the reference
- Hoeper MM, Lee SH, Voswinckel R, Palazzini M, Jais X, Marinelli A, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006;48(12):2546-52. (Level II evidence). View the reference
- Jenkins D. Pulmonary endarterectomy: the potentially curative treatment for patients with chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2015;24(136):263-71. (Review article). View the reference
- Tanabe N, Kawakami T, Satoh T, Matsubara H, Nakanishi N, Ogino H, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: A systematic review. Respiratory investigation. 2018;56(4):332-41. (Review article) View the reference
- Phan K, Jo HE, Xu J, Lau EM. Medical therapy versus balloon angioplasty for CTEPH: A systematic review and meta-analysis. Heart, lung & circulation. 2018;27(1):89-98. (Level I-II evidence) View the reference
- Tamada N, Nakayama K, Yanaka K, Onishi H, Shinkura Y, Tsuboi Y, et al. Early introduction of pulmonary endarterectomy or balloon pulmonary angioplasty contributes to better health-related quality of life in patients with chronic thromboembolic pulmonary hypertension. JACC Cardiovasc Interv. 2018;11(11):1114-6. (Level III evidence) View the reference
- Gopalan D, Delcroix M, Held M. Diagnosis of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143). (Review article) View the reference
- Rajaram S, Swift AJ, Capener D, Telfer A, Davies C, Hill C, et al. Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension. Eur Radiol. 2012;22(2):310-7. (Level II-III evidence). View the reference
- Berman M, Gopalan D, Sharples L, Screaton N, Maccan C, Sheares K, et al. Right ventricular reverse remodeling after pulmonary endarterectomy: magnetic resonance imaging and clinical and right heart catheterization assessment. Pulmonary Circulation. 2014;4(1):36-44. (Level III evidence) View the reference
Information for Consumers
Information for Consumers
Information from this website |
Information from the Royal Australian and New Zealand College of Radiologists’ website |
Consent to Procedure or Treatment Radiation Risks of X-rays and Scans Computed Tomography (CT) Angiography |
Iodine-Containing Contrast Medium Radiation Risk of Medical Imaging During Pregnancy Radiation Risk of Medical Imaging for Adults and Children Contrast Medium (Gadolinium versus Iodine) |
File Formats
Some documents for download on this website are in a Portable Document Format (PDF). To read these files you might need to download Adobe Acrobat Reader.