TABLE 9.
Diagnosis and management of CTEPH: Summary of recommendations
| DIAGNOSIS OF CTEPH | ||
|---|---|---|
| Early detection of CTEPH in patients with acute pulmonary thromboembolism | ||
| Clinical question | Recommendation(s) | GRADE |
| Screening for CTEPH in asymptomatic patients after acute venous thromboembolic event | 1. We do not suggest routine echocardiographic screening for CTEPH in asymptomatic patients following an acute venous thromboembolic event. | 2C |
| 2. We do not suggest routine follow-up imaging (V/Q, CT or MR) as a screening tool for CTEPH in asymptomatic patients following an acute venous thromboembolic event. | 2C | |
| Assessment of the possible contribution of CTEPH in patients with PH | ||
| Clinical question | Recommendation(s) | GRADE |
| Nuclear V/Q lung scanning versus CT pulmonary angiography to rule out CTEPH | 3. We recommend nuclear V/Q lung scanning instead of CT pulmonary angiography as a screening test to rule out the possibility of CTEPH in patients diagnosed with PH. A normal V/Q scan effectively rules out the possibility of CTEPH. | 1C |
| Nuclear V/Q lung scanning to assess the surgical accessibility of CTEPH | 4. We recommend that the results of a V/Q lung scan should not be used to assess the anatomical extent of potentially surgically accessible CTEPH. | 1C |
| CT or MR pulmonary angiography versus conventional pulmonary angiography to assess surgical accessibility of CTEPH | 5. We suggest the routine clinical use of a positive CT pulmonary angiogram to assess the anatomical extent of surgically accessible CTEPH. A negative CT pulmonary angiogram does not effectively rule out the presence of surgically accessible CTEPH, which is better assessed by contrast pulmonary angiography. | 2C |
| 6. We do not suggest the routine use of MR pulmonary angiography in the preoperative assessment of patients with CTEPH. | 2C | |
| MEDICAL AND SURGICAL MANAGEMENT OF CTEPH | ||
| General medical management of CTEPH patients | ||
| Clinical question | Recommendation(s) | GRADE |
| Chronic anticoagulation | 7. We suggest that all CTEPH patients, including patients who do not undergo PEA and patients post-PEA surgery, be chronically anticoagulated. | 2C |
| Calcium-channel blocker therapy | 8. We strongly recommend against the use of high-dose calcium channel blockers in patients with CTEPH. | 1C |
| Digoxin therapy | 9. We do not suggest routine digoxin therapy in patients with CTEPH. The expert panel recognized that in selected CTEPH patients with RV failure or atrial arrhythmias, digoxin may be of some limited benefit. | 2C |
| Supplemental nocturnal/exertional oxygen therapy | 10. In patients with CTEPH who manifest isolated nocturnal and/or exertional oxygen desaturation, there was no expert consensus on a recommendation for or against the use of supplemental oxygen. Treatment with supplemental oxygen is clearly indicated for CTEPH patients who manifest hypoxaemia at rest. | – |
| Cardiopulmonary exercise rehabilitation | 11. We suggest cardiopulmonary exercise rehabilitation in CTEPH patients. | 2C |
| Use of IVC filters | 12. There was no expert consensus on a recommendation for or against the routine use of IVC filters in patients with CTEPH. | – |
| Management of CTEPH patients before PEA surgery | ||
| Clinical question | Recommendation(s) | GRADE |
| Parenteral prostanoid therapy in CTEPH patients pre-PEA surgery | 13. We suggest pre-PEA treatment with intravenous epoprostenol in selected patients with severe surgically accessible CTEPH (WHO class III or IV, with PVR >1200 dyne·s/cm5, and/or RV failure) because this may improve hemodynamics and operability. The decision to institute pre-PEA parenteral epoprostenol therapy should be at the discretion of a centre with experience treating CTEPH patients, in consultation with a PEA surgeon. | 2C |
| Oral PH-specific medical therapy in CTEPH patients pre-PEA surgery | 14. We do not currently recommend for or against the use of oral PH-specific medical therapy in CTEPH patients who are being considered for PEA. The panel emphasized that a decision to treat CTEPH patients with oral PH medications should not delay referral to an expert centre for consideration of PEA in patients with surgically accessible CTEPH because such a delay may adversely impact the clinical outcome. | – |
| PEA in CTEPH patients | ||
| Clinical question | Recommendation(s) | GRADE |
| PEA in patients with surgically accessible CTEPH | 15. We recommend PEA as the treatment of choice in patients with surgically accessible CTEPH. | 1C |
| PEA in patients with distal CTEPH | 16. We suggest that patients with distal CTEPH be considered for PEA on an individual basis at a center experienced with PEA. | 2C |
| Post-PEA long-term follow-up in a PH centre | 17. We suggest that post-PEA CTEPH patients may benefit from long-term follow-up in a PH expert centre. | 2C |
| PH-specific medical therapy in CTEPH patients with residual PH post-PEA surgery | 18. We suggest ERA monotherapy in patients with symptomatic, residual PH post-PEA. This recommendation currently applies only to the ERA bosentan because there are no data available to assess the potential benefits of other ERAs (eg, sitaxsentan and ambrisentan) in CTEPH patients. | 2C |
| 19. We suggest PDE-5i monotherapy in patients with symptomatic, residual PH post-PEA. This recommendation currently applies only to the PDE-5i sildenafil because there are no data available to assess the potential benefits of other PDE-5i’s (eg, tadalafil and vardenafil) in CTEPH patients. | 2C | |
| 20. We suggest that parenteral prostanoid monotherapy could be considered in specific patients with symptomatic, residual PH post-PEA in whom oral PH-specific therapy has not been effective or was not tolerated. This recommendation currently applies only to the parenteral prostanoids (eg, intravenous epoprostenol and subcutaneous/intravenous treprostinil) because there are no data available to assess the potential benefits of oral (eg, beraprost) or inhaled (eg, iloprost, treprostinil) prostanoids. | 2C | |
| Management of patients not eligible for PEA | ||
| Clinical question | Recommendation(s) | GRADE |
| PH-specific medical therapy in patients with inoperable CTEPH | 21. We suggest ERA monotherapy in patients with symptomatic, inoperable CTEPH to improve symptoms and exercise capacity, short-term hemodynamics and, possibly, survival. This recommendation currently applies only to the ERA bosentan because there are no data available to assess the potential benefits of other ERAs (eg, sitaxsentan and ambrisentan). | 2C |
| 22. We suggest PDE-5i monotherapy in patients with symptomatic, inoperable CTEPH to improve short-term hemodynamics and WHO functional class, and possibly long-term exercise capacity and HRQoL. This recommendation currently applies only to the PDE-5i sildenafil because there are no data available to assess the potential benefits of other PDE-5is (eg, tadalafil and vardenafil). | 2C | |
| 23. We suggest monotherapy with parenteral prostanoids (eg, intravenous epoprostenol, subcutaneous/intravenous treprostinil) could be considered in patients with symptomatic, inoperable CTEPH. | 2C | |
| Combination PH-specific medical therapy in CTEPH patients | 24. We do not currently recommend routine clinical use of combination PH-specific therapy in patients with inoperable CTEPH or residual PH post-PEA. | 1C |
| Balloon angioplasty in patients with CTEPH | 25. We suggest that balloon pulmonary angioplasty be considered in patients with surgically accessible CTEPH who are ineligible for PEA to improve pulmonary hemodynamics, WHO functional class and exercise capacity. | 2C |
| Consideration of transplantation in CTEPH patients | ||
| Clinical question | Recommendation(s) | GRADE |
| Referral of CTEPH patients for lung transplantation | 26. We recommend that CTEPH patients who are inoperable or have residual PH post-PEA and who remain in WHO functional class III or IV, despite optimal medical therapy, be referred for evaluation for lung transplantation. Because there can be significant delays until transplantation, early referral is important. | 1C |