CTEPH Key Points

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) – Key Points

Definition

Chronic obstruction of pulmonary arteries by organized thromboembolic material leading to persistent pulmonary hypertension and progressive right heart failure.

Diagnostic Criteria

After ≥3 months of effective anticoagulation:

Mean pulmonary artery pressure (mPAP) >20 mmHg

Pulmonary vascular resistance (PVR) >2 Wood units

Pulmonary artery wedge pressure ≤15 mmHg

Imaging evidence of chronic thromboembolic disease

Pathophysiology

Incomplete resolution of pulmonary emboli

Fibrosis and organization of thrombus

Small vessel arteriopathy develops over time

Increased RV afterload → RV dysfunction/failure

Risk Factors

Previous pulmonary embolism/DVT

Recurrent venous thromboembolism

Splenectomy

Ventriculoatrial shunts

Chronic inflammatory disorders

Antiphospholipid syndrome

Infected pacemaker leads

Malignancy

Symptoms

Progressive exertional dyspnea

Fatigue

Exercise intolerance

Syncope/presyncope

Chest discomfort

Hemoptysis

Signs of right heart failure

Physical Findings

Loud P2

RV heave

Tricuspid regurgitation murmur

Elevated JVP

Peripheral edema

Hepatomegaly

ECG Findings

Right axis deviation

RV hypertrophy

Right atrial enlargement

RBBB

Echo Findings

RV dilation and dysfunction

Elevated pulmonary pressures

Septal flattening (“D-shaped LV”)

Dilated pulmonary artery

Best Screening Test

Ventilation–Perfusion (V/Q) scan

Highly sensitive

Normal V/Q scan virtually excludes CTEPH

CT Pulmonary Angiography Findings

Webs/bands

Pouch lesions

Mosaic perfusion

Chronic organized thrombi

Pulmonary artery stenosis

Gold Standard Hemodynamics

Right heart catheterization

Definitive Treatment

Pulmonary endarterectomy (PEA)

Potentially curative

Treatment of choice for operable disease

Other Treatment Options

Balloon Pulmonary Angioplasty (BPA)

For inoperable disease or residual PH after surgery

Medical Therapy

Lifelong anticoagulation

Riociguat approved for inoperable/residual CTEPH

Diuretics for right heart failure

Oxygen if hypoxemic

Prognosis

Untreated disease has poor prognosis due to progressive RV failure, but outcomes improve markedly with early diagnosis and intervention.

Comments

𝘼𝙣𝙩𝙞𝙘𝙤𝙖𝙜𝙪𝙡𝙖𝙩𝙞𝙤𝙣 𝘼𝙛𝙩𝙚𝙧 𝙎𝙩𝙧𝙤𝙠𝙚

𝘼𝙣𝙩𝙞𝙘𝙤𝙖𝙜𝙪𝙡𝙖𝙩𝙞𝙤𝙣 𝘼𝙛𝙩𝙚𝙧 𝙎𝙩𝙧𝙤𝙠𝙚 in Patient with AF and acute IS/TIA European Heart Association Guideline recommends: • 1 days after TIA • 3 days after mild stroke • 6 days after moderate stroke • 12 days after severe stroke Early anticoagulation can decrease a risk of recurrent stroke and embolic events but may increase a risk of secondary hemorrhagic transformation of brain infarcts. The 1-3-6-12-day rule is a known consensus with graded increase in delay of anticoagulation between 1 and 12 days after onset of ischemic stroke or transient ischemic attack(TIA), according to neurological severity based on European expert opinions. However, this rule might be somewhat later than currently used in a real-world practical setting.

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