CTEPH

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by chronic blood clots in the lungs that result in increased pressure in the pulmonary arteries. Over time, these clots can lead to scarring and narrowing of the blood vessels, making it harder for the heart to pump blood through the lungs and causing strain on the heart.

Symptoms

• Shortness of breath: Initially during exertion, but as the condition progresses, it can occur at rest.
• Fatigue: General feeling of being tired or weak.
• Chest pain: Discomfort or pain, often worse with physical activity.
• Heart palpitations: Irregular or rapid heartbeat.
• Swelling: Particularly in the ankles, legs, and abdomen (edema).
• Fainting or dizziness: Especially during physical activity.

Risk Factors

• Previous pulmonary embolism: The primary risk factor for CTEPH.
• Inherited blood clotting disorders: Such as factor V Leiden.
• Chronic inflammatory diseases: Including inflammatory bowel disease.
• Cancer: Certain cancers can increase the risk of blood clots.
• Splenectomy: Removal of the spleen can increase the risk.

Diagnosis

• Ventilation/Perfusion (V/Q) Scan: Detects areas of the lung that are not receiving enough blood flow.
• CT Pulmonary Angiography: Visualizes the pulmonary arteries and can detect chronic clots.
• Right Heart Catheterization: Measures the pressure in the pulmonary arteries and evaluates the severity of the hypertension.
• Pulmonary Angiography: Provides detailed images of the blood vessels in the lungs.
• Echocardiogram: Assesses the function and structure of the heart.

Treatment

• Pulmonary Endarterectomy (PEA): Surgical removal of the chronic clots and scar tissue from the pulmonary arteries, considered the treatment of choice and potentially curative.
• Balloon Pulmonary Angioplasty (BPA): A less invasive procedure that uses a balloon to widen the narrowed pulmonary arteries.
• Medications: Targeted pulmonary hypertension therapies, such as endothelin receptor antagonists, phosphodiesterase inhibitors, or prostacyclin analogs.
• Anticoagulants: Blood thinners to prevent new clots from forming.
• Oxygen therapy: To improve oxygen levels in the blood.

Prognosis

The prognosis for CTEPH can vary depending on the severity of the condition and the success of treatments. Pulmonary endarterectomy can be curative in many cases, significantly improving symptoms and survival rates. For those who cannot undergo surgery, the prognosis is more guarded, and lifelong medical management may be necessary.

Monitoring and Follow-up

Regular follow-up with a specialist in pulmonary hypertension is crucial to monitor the condition, adjust treatments, and manage any complications. This typically involves periodic imaging studies, right heart catheterization, and functional assessments.

Early detection and appropriate management are key to improving outcomes and quality of life for individuals with CTEPH.