Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Introduction

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a severe and potentially fatal form of pulmonary hypertension caused by unresolved blood clots in the lungs. Unlike acute pulmonary embolism (PE), where clots dissolve with time and treatment, CTEPH occurs when these clots become organized fibrotic tissue, obstructing the pulmonary arteries and leading to increased pressure in the pulmonary circulation.

Causes and Risk Factors

CTEPH typically arises from previous episodes of pulmonary embolism. However, not all individuals who suffer from PE will develop CTEPH. Risk factors include: - Recurrent or unprovoked pulmonary embolism - Hypercoagulable states (e.g., antiphospholipid syndrome, Factor V Leiden mutation) - Splenectomy - Inflammatory diseases such as vasculitis - Infected pacemaker leads or ventriculoatrial shunts

Symptoms

CTEPH presents similar to other forms of pulmonary hypertension and includes: - Progressive shortness of breath (dyspnea), especially with exertion - Fatigue and weakness - Chest discomfort or pain - Palpitations - Syncope (fainting) - Symptoms of right heart failure (swelling of the legs, ascites, jugular vein distension)

Diagnosis

Diagnosing CTEPH involves multiple tests: 1. Ventilation/Perfusion (V/Q) Scan – Gold standard for detecting chronic thromboembolic disease. 2. Computed Tomography Pulmonary Angiography (CTPA) – Helps visualize the pulmonary vasculature. 3. Right Heart Catheterization – Confirms increased pulmonary artery pressure. 4. Pulmonary Angiography – Defines the exact location and extent of obstruction.

Treatment

CTEPH treatment focuses on restoring blood flow and reducing pulmonary hypertension: 1. Pulmonary Endarterectomy (PEA): The preferred curative surgery to remove organized clots. 2. Balloon Pulmonary Angioplasty (BPA): Catheter-based procedure for inoperable cases. 3. Medical Therapy: - Riociguat (a pulmonary vasodilator) is approved for patients who are inoperable or have residual pulmonary hypertension post-surgery. - Lifelong anticoagulation (e.g., warfarin) is required to prevent new clots.

Prognosis

With early diagnosis and appropriate intervention, CTEPH can be managed effectively. Pulmonary endarterectomy offers the best chance for long-term improvement, significantly reducing pulmonary pressures and improving overall quality of life. However, untreated CTEPH can lead to right heart failure and death.

Conclusion

CTEPH is a rare but serious condition that requires timely diagnosis and specialized care. If you have had a previous pulmonary embolism and experience persistent breathlessness, consult a pulmonologist or cardiologist for a thorough evaluation.

Source recommendations

1. 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension

1. https://academic.oup.com/eurheartj/article/43/38/3618/6673929
2. https://pubmed.ncbi.nlm.nih.gov/36017548/
3. https://publications.ersnet.org/content/erj/early/2022/08/25/1399300300879-2022.abstract
4. https://www.escardio.org/Guidelines/Clinical-Practice-Guidelines/Pulmonary-Hypertension-Guidelines-on-Diagnosis-and-Treatment-of
5. https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2022/08/30/19/11/2022-ESC-Guidelines-for-Pulmonary-Hypertension-ESC-2022

2. CHEST Guidelines on Antithrombotic Therapy for VTE Disease

1. https://journal.chestnet.org/article/S0012-3692(21)01506-3/fulltext
2. https://www.chestnet.org/newsroom/press-releases/2021/08/chest-releases-new-guidelines-for-antithrombotic-therapy-for-vte-disease
3. https://journal.chestnet.org/article/S0012-3692(24)00292-7/fulltext
4. https://www.sciencedirect.com/science/article/pii/S0012369224002927
5. https://pubmed.ncbi.nlm.nih.gov/26867832/