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Updated: May 26, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference
Heinrike Wilkens1, Irene Lang, Jürgen Behr
1Clinic for Internal Medicine V, Saarland University Hospital, Homburg. heinrike.wilkens@uks.eu
Insights
Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable form of pulmonary hypertension (PH). Early diagnosis via ventilation/perfusion scans and referral to specialized centers improve patient outcomes.
Area of Science:
- Cardiology
- Pulmonology
- Vascular Surgery
Background:
- Pulmonary hypertension (PH) encompasses various conditions, including chronic thromboembolic pulmonary hypertension (CTEPH).
- CTEPH is a potentially curable subset of PH, treatable with pulmonary endarterectomy (PEA).
- Current management often involves underdiagnosis and suboptimal treatment of CTEPH.
Framework:
- The 2009 European Guidelines provide a framework for CTEPH diagnosis and treatment.
- Implementation of these guidelines is critical for effective patient management.
- This recommendation updates and comments on the European guidelines, incorporating recent advancements.
Implementation:
- Ventilation/perfusion (V/Q) lung scans are the recommended screening tool for unexplained PH.
- CT angiography can also identify signs suggestive of CTEPH.
- Patients with suspected CTEPH require referral to specialized centers for expert evaluation and management.
Implications:
- Timely diagnosis and appropriate management of CTEPH can lead to a cure.
- Surgical assessment by an experienced PEA surgeon is essential for determining operability.
- Improved adherence to guidelines and updated information can enhance clinical practice and patient outcomes.
Abstract:
In the 2009 European Guidelines on the diagnosis and treatment of pulmonary hypertension (PH), one section covers aspects of pathophysiology, diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The practical implementation of the guidelines for this disease is of crucial importance, because CTEPH is a subset of PH which can potentially be cured by pulmonary endarterectomy (PEA). Nowadays, CTEPH is commonly underdiagnosed and not properly managed. Any patient with unexplained PH should be evaluated for the presence of CTEPH, and a ventilation/perfusion (V/Q) lung scan is recommended as screening method of choice. If the V/Q scan or CT angiography reveals signs of CTEPH, the patient should be referred to a specialized center with expertise in the medical and surgical management of this disease. Every case has to be reviewed by an experienced PEA surgeon for the assessment of operability. In this updated recommendation, important contents of the European guidelines were commented, and more recent information regarding diagnosis and treatment was added.
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