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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Chronic thromboembolic pulmonary hypertension: an updated review
Irene M Lang1, Walter Klepetko
1Department of Internal Medicine II, Division of Cardiology, Austria. irene.lang@meduniwien.ac.at
Insights
Chronic thromboembolic pulmonary hypertension (CTEPH) affects 3.8% of patients post-pulmonary embolism. Early identification and surgical intervention offer significant hemodynamic improvements for this dual pulmonary vascular disorder.
Area of Science:
- Cardiovascular Medicine
- Pulmonary Medicine
- Vascular Biology
Background:
- Venous thromboembolism (VTE) encompasses a range of conditions, including deep vein thrombosis and pulmonary embolism.
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious long-term complication of acute pulmonary embolism.
- Understanding the pathophysiology of CTEPH is crucial for effective management.
Purpose of the Study:
- To review the incidence, pathophysiology, diagnosis, and treatment of chronic thromboembolic pulmonary hypertension (CTEPH).
- To highlight CTEPH as a sequela of pulmonary embolism and a dual pulmonary vascular disorder.
Main Methods:
- Analysis of a prospective long-term study on CTEPH incidence.
- Review of diagnostic modalities including lung perfusion scans and pulmonary angiography.
- Evaluation of surgical pulmonary endarterectomy as a primary treatment.
Main Results:
- An estimated 3.8% of patients surviving symptomatic idiopathic pulmonary embolism develop CTEPH within 2 years.
- Younger age, large perfusion defects, and idiopathic presentation are risk factors for CTEPH.
- Surgical pulmonary endarterectomy restores normal pulmonary hemodynamics in nearly 80% of patients with low mortality (<10%) in expert centers.
Conclusions:
- CTEPH is characterized by major and small vessel pulmonary vascular changes.
- Early diagnosis and interdisciplinary management are essential for optimal outcomes.
- Surgical intervention offers significant benefits for patients with CTEPH.
Purpose Of Review:
Venous thromboembolism is a spectrum of disease comprising deep vein thrombosis, thrombus in transit, acute pulmonary embolism, and chronic thromboembolic pulmonary hypertension (CTEPH) as a rare and late possible sequela.
Recent Findings:
On the basis of a prospective long-term study, the incidence of CTEPH is estimated at 3.8% within 2 years of all patients surviving an episode of symptomatic idiopathic pulmonary embolism. Young age, a large perfusion defect, and idiopathic clinical presentation are associated with a higher probability of CTEPH. Current pathophysiological concepts suggest a misguided thrombus resolution process that is triggered by infection, inflammation, autoimmunity, and malignancy. Diagnosis and therapy of CTEPH are interdisciplinary achievements and nowadays still based on a positive lung perfusion scan and for assessment of operability on a classical pulmonary angiography. Treatment of choice is surgical pulmonary endarterectomy of the pulmonary obstructions, which leads to restoration of normal pulmonary hemodynamics at rest in nearly 80% of patients. In expert centers, surgical mortality is under 10%.
Summary:
CTEPH has emerged as a 'dual' pulmonary vascular disorder with major vessel vascular remodeling of thrombus organization, combined with a small vessel pulmonary arteriopathy that is a target for classic vasodilator treatments.
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