CTEPH Diagnosis
CTEPH diagnosis is challenging
Difficult to distinguish from other forms of PH1
Common symptoms of CTEPH include progressive dyspnea on exertion, rapid exhaustion, and fatigue. These symptoms and the clinical course of CTEPH can be indistinguishable from other forms of severe pulmonary hypertension (PH).1 If symptoms, signs, and history suggest PH, patients should be urgently referred for further assessments.
Can present without history of PE1,2
Patients with CTEPH can present without a history of pulmonary embolism (PE), but estimates vary.1,2 An international prospective registry including 679 patients showed that 75% of patients with CTEPH had a history of acute PE.2
Conversely, Lang reported in 2004 that of 142 consecutive CTEPH patients, 90 (63%) had no history of acute PE.3
Symptoms can be delayed1
CTEPH can progress quickly, though some patients may experience a “honeymoon period” of months to years between their acute PE and clinical signs of CTEPH1
-
Signs of right-heart failure occur late in the course of the disease1
Diagnosis
Diagnosis of CTEPH requires comprehensive evaluation4
- Echocardiography is used in the initial assessment of suspected PH5
- A ventilation/perfusion (V/Q) lung scan should be performed as the first examination for CTEPH screening and remains the most effective tool in excluding CTEPD6,7
- A mismatched perfusion defect is a characteristic diagnostic feature of CTEPH7
- Diagnosis of CTEPH can be supported by characteristic findings during multislice CT angiography, including a mosaic perfusion pattern, dilatation of proximal pulmonary arteries and right heart chambers, and the presence of vascular stenosis or obstruction5
- Computed tomography pulmonary angiography (CTPA) is an important next step in CTEPH diagnosis and treatment decision-making6
- CTPA can be used to detect direct or indirect signs of CTEPH, including filling defects, webs or bands in the pulmonary angiographies (PAs), PA retraction/dilatation, mosaic perfusion, or enlarged bronchial arteries7
- CTPA is also the imaging choice for acute PE, and initial signs suggestive of preexisting CTEPH can be detected during the assessment for PE; when obtaining CTPA for the workup of CTEPH, proper protocol and acquisition are critical to ensure visualization of the more subtle findings in CTEPH compared with PE6
- A negative CTPA does not exclude CTEPD and digital subtraction angiography (DSA) can still be used to assess treatment options when CTPA is inconclusive7
- Right heart catheterization (RHC) and catheter-based pulmonary angiography are mandatory to confirm a CTEPH diagnosis6
- A complete hemodynamic evaluation by RHC including cardiac output is recommended to assess the prognosis and risks associated with pulmonary thromboendarterectomy (PTE)6
- Referral to specialized centers for testing to define anatomical locations and extent of obstructions and to quantify the degree of PH is essential8
- The sequence of these tests may vary according to the factors suggestive of PH9
Adapted from ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension, 2022.
aCTEPH suspected from history of PE, including elevated sPAP on echocardiography and signs suggesting CTEPH on CTPA performed at the time of the acute PE.
bAlternative perfusion imaging techniques—such as iodine subtraction mapping, DECT, and MRI perfusion—are currently under evaluation.
cTypical pattern, including low PETCO2, high VE/VCO2, low VO2/HR, and low peak VO2.
dComprehensive work-up after 3 months of therapeutic anticoagulation or sooner in unstable or rapidly deteriorating patients. Ideally, CTPA, DSA, and RHC are performed in CTEPH centers, but they are sometimes performed in PH centers, depending on the country and organization.
CPET = cardiopulmonary exercise test; CTEPD = chronic thromboembolic pulmonary disease; CTEPH = chronic thromboembolic pulmonary hypertension; CTPA = computed tomography pulmonary angiography; DECT = dual-energy computed tomography; DSA = digital subtraction angiography; MDT = multidisciplinary team; MRI = magnetic resonance imaging; N = no; PE = pulmonary embolism; PETCO2 = end-tidal partial pressure of carbon dioxide; PH = pulmonary hypertension; RHC = right heart catheterization; sPAP = systolic pulmonary arterial pressure; V/Q = ventilation/perfusion; VE/VCO2 = ventilatory equivalents for carbon dioxide; VO2/HR = oxygen pulse; VO2 = oxygen uptake; Y = yes.
References:
1. Hoeper M, Mayer E, Simmoneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113(16):2011-2020. 2. Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973-1981. 3. Lang IM. Chronic thromboembolic pulmonary hypertension—not so rare at all. N Engl J Med. 2004;350:2236-2238. 4. Galiè N, Humbert M, Vachiery JL, et al; ESC Scientific Document Group. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119. 5. Jenkins D, Mayer E, Screaton N, Madani M. State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev. 2012;21(123):32-39. 6. Kim NH, D'Armini AM, Delcroix M, et al. Chronic thromboembolic pulmonary disease. Eur Respir J. 2024;64(4):2401294. 7. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. 8. Piazza G and Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351-360. 9. McLaughlin W, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(116):2250-2294.
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