Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
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Questions and Answers

What is the systolic pulmonary arterial pressure (sPAP) threshold that raises suspicion of underlying chronic thromboembolic pulmonary hypertension (CTEPH)?

  • 30 mm Hg
  • >60 mm Hg (correct)
  • 50 mm Hg
  • 40 mm Hg
  • The 3-year survival rate for operable patients with CTEPH is over 90%.

    True

    Name one condition that has been associated with CTEPH.

    Antiphospholipid syndrome

    Patients with right ventricular dysfunction and hypertrophy may have underlying ________.

    <p>CTEPH</p> Signup and view all the answers

    Which of the following could be a reason for patient resistance to referral to CTEPH centers?

    <p>Invasiveness of surgery</p> Signup and view all the answers

    Combination therapeutic approaches for CTEPH have shown less effectiveness than single approaches.

    <p>False</p> Signup and view all the answers

    What is the concern regarding the referral of patients to CTEPH centers?

    <p>Invasiveness of surgery</p> Signup and view all the answers

    Match the following conditions with their association to CTEPH:

    <p>Antiphospholipid syndrome = Associated Malignancies = Associated Ventriculo-atrial shunts = Associated Asthma = Not associated</p> Signup and view all the answers

    What does CTEPH stand for?

    <p>Chronic Thromboembolic Pulmonary Hypertension</p> Signup and view all the answers

    What is a sign of chronic thromboembolic pulmonary disease (CTEPD)?

    <p>Intravascular webs or bands</p> Signup and view all the answers

    High-flow correction can contribute to microvasculopathy in patients with CTEPH.

    <p>True</p> Signup and view all the answers

    What is one technique that has been described to assess microvasculopathy in CTEPH patients?

    <p>Measurement of mPAP</p> Signup and view all the answers

    Microvascular disease in chronic thromboembolic disease appears as a central area of perfusion defects.

    <p>False</p> Signup and view all the answers

    The anastomoses between systemic collateral circulation and pulmonary arteries are essential to prevent _____ in obstructed territories.

    <p>lung ischaemia</p> Signup and view all the answers

    What has been proposed for acute haemodynamic testing in patients?

    <p>Inhaled nitric oxide</p> Signup and view all the answers

    Which imaging technique is suggested for optimal treatment planning in CTEPD?

    <p>CTPA</p> Signup and view all the answers

    What is one possible cause of pulmonary vascular resistance (PVR) in patients with CTEPH?

    <p>Intravascular obstruction of pulmonary arteries</p> Signup and view all the answers

    CTEPD without pulmonary hypertension indicates an advanced stage of CTEPH.

    <p>False</p> Signup and view all the answers

    Match the conditions with their descriptions:

    <p>CTEPH = Condition resulting from chronic thromboembolic issues in the lungs Microvasculopathy = Pathological changes in small blood vessels Arteriopathy = Changes in the artery structure due to stress Haemoptysis = Coughing up blood from the respiratory tract</p> Signup and view all the answers

    Dual-energy CT provides excellent morphological images of the pulmonary arteries with _______ images of the lungs.

    <p>perfusion</p> Signup and view all the answers

    The pulmonary artery occlusion technique is universally established as part of invasive haemodynamic assessment.

    <p>False</p> Signup and view all the answers

    Match the following CT techniques with their characteristics:

    <p>CTPA = Visualizes arterial occlusions Dual-energy CT = Provides iodine maps for perfusion DSA = Assesses morphology of pulmonary arteries Perfusion imaging = Shows blood flow in the lungs</p> Signup and view all the answers

    What are the two types of obstruction mentioned in relation to pulmonary arteries in patients with CTEPH?

    <p>Intravascular obstruction and microvasculopathy</p> Signup and view all the answers

    What condition can lead to the progression of CTEPH despite optimal anticoagulation?

    <p>Microvasculopathy</p> Signup and view all the answers

    What causes the arteriopathy in the obstructed territories?

    <p>High flow from systemic collateral circulation</p> Signup and view all the answers

    Direct imaging of microvascular changes is currently possible.

    <p>False</p> Signup and view all the answers

    In CTEPH, the intravascular obstruction of pulmonary arteries is caused by __________.

    <p>organised fibrotic clots</p> Signup and view all the answers

    In CTEPH, PVR can be partitioned into larger arterial and _____ components.

    <p>small arterial plus venous</p> Signup and view all the answers

    Match the following aspects related to pulmonary hypertension with their descriptions:

    <p>Post-capillary pulmonary hypertension = Involves congestion due to left heart failure Pre-capillary arterial disease = Characterized by high pulmonary vascular resistance Microvasculopathy = Refers to vessel obstruction in both obstructed and non-obstructed lung areas CTEPH = Chronic Thromboembolic Pulmonary Hypertension</p> Signup and view all the answers

    The measurement of PVR refers to the measurement of _____ in pulmonary circulation.

    <p>Pulmonary Vascular Resistance</p> Signup and view all the answers

    Match the type of obstruction with its description.

    <p>Lobar obstruction = Obstruction in the main lobar arteries Subsegmental obstruction = Obstruction in smaller, distal segments of the artery Microvascular changes = Pathological changes in small pulmonary vessels</p> Signup and view all the answers

    Which option correctly describes the structural change in the arteries associated with CTEPH?

    <p>Obstruction by fibrotic clots</p> Signup and view all the answers

    Why might microvascular lesions be reversible?

    <p>If the cause is removed</p> Signup and view all the answers

    Histological examination can reveal changes in both lobar and subsegmental arteries in CTEPH patients.

    <p>True</p> Signup and view all the answers

    Imaging of vessel obstruction provides an objective assessment of microvasculopathy.

    <p>False</p> Signup and view all the answers

    What imaging technique can provide information regarding the status of subsegmental arteries?

    <p>Optical coherence tomography</p> Signup and view all the answers

    What contributes to the discordance between perfusion defects and PVR in CTEPH?

    <p>Remodelling of pre-capillary arteries</p> Signup and view all the answers

    What is the most commonly experienced complication of BPA?

    <p>Pulmonary haemorrhage</p> Signup and view all the answers

    A PVR of more than 320 dyn·s·cm–⁵ is typically targeted for balloon pulmonary angioplasty.

    <p>True</p> Signup and view all the answers

    What is one potential outcome of the complication known as haemoptysis during BPA?

    <p>Development of severe lung injury</p> Signup and view all the answers

    A staged concept is preferred to reduce the risk of __________ injury during the treatment.

    <p>reperfusion</p> Signup and view all the answers

    Match the following terms with their descriptions:

    <p>PVR = A measure related to pulmonary vascular resistance BPA = A procedure to treat obstructive lesions in pulmonary arteries guidewires = Used to cross obstructing lesions during angioplasty haemoptysis = Coughing up blood from the respiratory tract</p> Signup and view all the answers

    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.

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