Podcast
Questions and Answers
What is the systolic pulmonary arterial pressure (sPAP) threshold that raises suspicion of underlying chronic thromboembolic pulmonary hypertension (CTEPH)?
What is the systolic pulmonary arterial pressure (sPAP) threshold that raises suspicion of underlying chronic thromboembolic pulmonary hypertension (CTEPH)?
The 3-year survival rate for operable patients with CTEPH is over 90%.
The 3-year survival rate for operable patients with CTEPH is over 90%.
True
Name one condition that has been associated with CTEPH.
Name one condition that has been associated with CTEPH.
Antiphospholipid syndrome
Patients with right ventricular dysfunction and hypertrophy may have underlying ________.
Patients with right ventricular dysfunction and hypertrophy may have underlying ________.
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Which of the following could be a reason for patient resistance to referral to CTEPH centers?
Which of the following could be a reason for patient resistance to referral to CTEPH centers?
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Combination therapeutic approaches for CTEPH have shown less effectiveness than single approaches.
Combination therapeutic approaches for CTEPH have shown less effectiveness than single approaches.
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What is the concern regarding the referral of patients to CTEPH centers?
What is the concern regarding the referral of patients to CTEPH centers?
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Match the following conditions with their association to CTEPH:
Match the following conditions with their association to CTEPH:
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What does CTEPH stand for?
What does CTEPH stand for?
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What is a sign of chronic thromboembolic pulmonary disease (CTEPD)?
What is a sign of chronic thromboembolic pulmonary disease (CTEPD)?
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High-flow correction can contribute to microvasculopathy in patients with CTEPH.
High-flow correction can contribute to microvasculopathy in patients with CTEPH.
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What is one technique that has been described to assess microvasculopathy in CTEPH patients?
What is one technique that has been described to assess microvasculopathy in CTEPH patients?
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Microvascular disease in chronic thromboembolic disease appears as a central area of perfusion defects.
Microvascular disease in chronic thromboembolic disease appears as a central area of perfusion defects.
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The anastomoses between systemic collateral circulation and pulmonary arteries are essential to prevent _____ in obstructed territories.
The anastomoses between systemic collateral circulation and pulmonary arteries are essential to prevent _____ in obstructed territories.
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What has been proposed for acute haemodynamic testing in patients?
What has been proposed for acute haemodynamic testing in patients?
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Which imaging technique is suggested for optimal treatment planning in CTEPD?
Which imaging technique is suggested for optimal treatment planning in CTEPD?
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What is one possible cause of pulmonary vascular resistance (PVR) in patients with CTEPH?
What is one possible cause of pulmonary vascular resistance (PVR) in patients with CTEPH?
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CTEPD without pulmonary hypertension indicates an advanced stage of CTEPH.
CTEPD without pulmonary hypertension indicates an advanced stage of CTEPH.
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Match the conditions with their descriptions:
Match the conditions with their descriptions:
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Dual-energy CT provides excellent morphological images of the pulmonary arteries with _______ images of the lungs.
Dual-energy CT provides excellent morphological images of the pulmonary arteries with _______ images of the lungs.
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The pulmonary artery occlusion technique is universally established as part of invasive haemodynamic assessment.
The pulmonary artery occlusion technique is universally established as part of invasive haemodynamic assessment.
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Match the following CT techniques with their characteristics:
Match the following CT techniques with their characteristics:
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What are the two types of obstruction mentioned in relation to pulmonary arteries in patients with CTEPH?
What are the two types of obstruction mentioned in relation to pulmonary arteries in patients with CTEPH?
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What condition can lead to the progression of CTEPH despite optimal anticoagulation?
What condition can lead to the progression of CTEPH despite optimal anticoagulation?
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What causes the arteriopathy in the obstructed territories?
What causes the arteriopathy in the obstructed territories?
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Direct imaging of microvascular changes is currently possible.
Direct imaging of microvascular changes is currently possible.
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In CTEPH, the intravascular obstruction of pulmonary arteries is caused by __________.
In CTEPH, the intravascular obstruction of pulmonary arteries is caused by __________.
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In CTEPH, PVR can be partitioned into larger arterial and _____ components.
In CTEPH, PVR can be partitioned into larger arterial and _____ components.
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Match the following aspects related to pulmonary hypertension with their descriptions:
Match the following aspects related to pulmonary hypertension with their descriptions:
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The measurement of PVR refers to the measurement of _____ in pulmonary circulation.
The measurement of PVR refers to the measurement of _____ in pulmonary circulation.
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Match the type of obstruction with its description.
Match the type of obstruction with its description.
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Which option correctly describes the structural change in the arteries associated with CTEPH?
Which option correctly describes the structural change in the arteries associated with CTEPH?
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Why might microvascular lesions be reversible?
Why might microvascular lesions be reversible?
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Histological examination can reveal changes in both lobar and subsegmental arteries in CTEPH patients.
Histological examination can reveal changes in both lobar and subsegmental arteries in CTEPH patients.
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Imaging of vessel obstruction provides an objective assessment of microvasculopathy.
Imaging of vessel obstruction provides an objective assessment of microvasculopathy.
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What imaging technique can provide information regarding the status of subsegmental arteries?
What imaging technique can provide information regarding the status of subsegmental arteries?
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What contributes to the discordance between perfusion defects and PVR in CTEPH?
What contributes to the discordance between perfusion defects and PVR in CTEPH?
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What is the most commonly experienced complication of BPA?
What is the most commonly experienced complication of BPA?
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A PVR of more than 320 dyn·s·cm–⁵ is typically targeted for balloon pulmonary angioplasty.
A PVR of more than 320 dyn·s·cm–⁵ is typically targeted for balloon pulmonary angioplasty.
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What is one potential outcome of the complication known as haemoptysis during BPA?
What is one potential outcome of the complication known as haemoptysis during BPA?
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A staged concept is preferred to reduce the risk of __________ injury during the treatment.
A staged concept is preferred to reduce the risk of __________ injury during the treatment.
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Match the following terms with their descriptions:
Match the following terms with their descriptions:
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Study Notes
Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
- CTEPH is a rare complication of acute pulmonary embolism
- Characterised by persistent fibrothrombotic obstruction of pulmonary arteries
- Incidence is ~3% in pulmonary embolism survivors
- Symptoms are often non-specific, including exertional dyspnea and fatigue
- Can present with right heart failure and, rarely, hemoptysis
- Affects more than a third of patients referred to pulmonary hypertension centers
- Imaging characteristics have improved diagnosis
- Advances in multimodal management have improved 3-year survival to >90% for both operable and inoperable patients
Disease Mechanisms
- Resolution of pulmonary embolism thrombi resolves within 3 months in most patients
- Thrombi transition from erythrocyte-rich to fibrin- and collagen-rich composition
- Neutrophils and monocyte-derived macrophages are recruited sequentially in this process, enhancing collagen within thrombi
- Neovascularisation is observed, leading to restoration of blood flow
- Inflammatory components and inflammatory mediators contribute to thrombus resolution and development of CTEPH
Imaging Characteristics
- CT pulmonary angiography (CTPA), digital subtraction angiography (DSA), and selective angiography help distinguish proximal and distal obstructive disease.
- Peripheral changes in the microvasculature are difficult to image directly.
- Comparing imaging findings with haemodynamic severity is key for diagnosis
Treatment Strategies
- Multimodal approach, combining surgery, intervention, and medication, is important
- Pulmonary endarterectomy (PEA)—surgical removal of obstructive material—is recommended for operable patients
- Balloon Pulmonary Angioplasty (BPA)—dilating vascular lesions—is an option for inoperable patients or those with persistent pulmonary hypertension after PEA.
- Pulmonary hypertension drugs are used to treat microvasculopathy, and long-term follow-up is required after interventions.
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Description
This quiz covers the details of Chronic Thromboembolic Pulmonary Hypertension (CTEPH), a rare complication following pulmonary embolism. It explores its incidence, symptoms, disease mechanisms, and advancements in diagnosis and management. Test your understanding of this complex condition and its impact on patient care.