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Chronic thromboembolic pulmonary hypertension

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of chronic thromboembolic pulmonary hypertension are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC/ERS 2022,2016), the European Respiratory Society (ERS 2021), the Canadian Cardiovascular Society (CCS/CTS 2020), the American Heart Association (AHA 2011), and the Canadian Thoracic Society (CTS 2010). ...
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Screening and diagnosis

Definition: as per ERS 2021 guidelines, consider viewing patients with symptoms attributable to post-thrombotic deposits within pulmonary arteries as having CTEPD, with or without pulmonary hypertension. Use CTEPH term in patients with pulmonary hypertension.
E
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  • Epidemiology

  • Pathophysiology

  • Indications for screening

  • Indications for testing

Diagnostic investigations

Echocardiography: as per ERS 2021 guidelines, obtain echocardiography as the test of choice in patients with suspected CTEPH. Consider using the Leiden CTEPH rule-out criteria to exclude the presence of CTEPH and/or to establish an alternative diagnosis.
E

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  • Perfusion imaging

  • Pulmonary angiography

  • Cardiopulmonary exercise testing

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Diagnostic procedures

Right heart catheterization: as per CCS/CTS 2020 guidelines, perform right heart catheterization in all patients with suspected CTEPH to confirm the hemodynamic diagnosis of precapillary pulmonary hypertension and to assess the severity of pulmonary hypertension.
B

Respiratory support

Supplemental oxygen
As per CTS 2010 guidelines:
Administer supplemental oxygen in patients with CTEPH exhibiting hypoxemia at rest.
Insufficient evidence to recommend for or against the use of supplemental oxygen in patients with CTEPH manifesting isolated nocturnal and/or exertional oxygen desaturation.
I

Medical management

General principles
As per ERS/ESC 2022 guidelines:
Review all patients with CTEPH by a CTEPH team for the assessment of multimodal management.
B
Consider offering a multimodality approach in patients with persistent pulmonary hypertension after pulmonary endarterectomy and in patients with inoperable CTEPH.
C

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  • Pulmonary arterial hypertension-specific pharmacotherapy (indications)

  • Pulmonary arterial hypertension-specific pharmacotherapy (combination therapy)

  • sGC stimulators

  • Endothelin receptor antagonists

  • PDE5 inhibitors

  • Prostanoids

  • Anticoagulation

  • Other medications

Nonpharmacologic interventions

Rehabilitation
As per ERS 2021 guidelines:
Offer rehabilitation, in hospital or home-based settings, in patients with inoperable CTEPH.
E
Consider offering a carefully monitored, low-dose rehabilitation program as standard of care in patients with CTEPH after pulmonary endarterectomy or balloon pulmonary angioplasty.
E

Therapeutic procedures

Balloon pulmonary angioplasty: as per ERS/ESC 2022 guidelines, consider performing balloon pulmonary angioplasty in technically operable patients with a high proportion of distal disease and an unfavorable risk-benefit ratio for pulmonary endarterectomy.
C
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Perioperative care

Preoperative pharmacotherapy: as per ERS 2021 guidelines, insufficient evidence that preoperative pharmacotherapy is beneficial in patients with operable CTEPH. Consider initiating pulmonary hypertension-targeted drugs as bridging therapy to pulmonary endarterectomy in selected high-risk patients (with high preoperative pulmonary vascular resistance) after multidisciplinary team assessment in expert centers.
I

Surgical interventions

Pulmonary endarterectomy: as per ERS/ESC 2022 guidelines, perform pulmonary endarterectomy as the treatment of choice in patients with CTEPH and fibrotic obstructions within pulmonary arteries accessible by surgery.
B

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Follow-up and surveillance

Post-PEA management, pharmacotherapy
As per ERS/ESC 2022 guidelines:
Initiate riociguat in symptomatic patients with persistent or recurrent pulmonary hypertension after pulmonary endarterectomy.
B
Consider initiating treprostinil SC in patients with persistent or recurrent pulmonary hypertension after pulmonary endarterectomy.
C

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  • Post-PEA management (follow-up)