Dr. John Feenstra, Lead Physician of the Pulmonary Hypertension service at the Prince Charles Hospital, Brisbane, provides an overview of Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially fatal condition where blood clots in the lungs become scar tissue, leading to high blood pressure in the pulmonary arteries. This condition can develop even in patients on blood thinners. Over time, the clots become organised and form scar tissue, obstructing blood flow.
Chronic thromboembolic pulmonary hypertension symptoms are non-specific and include shortness of breath, fatigue, chest pain, and exercise intolerance. Diagnosis often involves a combination of echocardiograms, ventilation-perfusion (VQ) scans, right heart catheterisation, and pulmonary angiography. These tests help determine the presence and extent of blockages in the pulmonary arteries.
Treatment options for Chronic thromboembolic pulmonary hypertension include lifelong anticoagulation, surgery, and medical therapy. Pulmonary endarterectomy (PEA) surgery is the gold standard and can be curative by removing the scar tissue from the pulmonary arteries. However, not all patients are suitable for surgery.
For non-operable patients or those with residual pulmonary hypertension after surgery, medical therapy with riociguat, a drug that opens up blood vessels in the lungs, is available. Additionally, balloon pulmonary angioplasty (BPA) is a newer treatment option that involves using a balloon to open up blocked blood vessels. BPA has shown promising results in improving blood flow and reducing symptoms.
Chronic thromboembolic pulmonary hypertension requires a multidisciplinary approach involving pulmonologists, cardiologists, radiologists, surgeons, and specialised nurses. Regular follow-ups and imaging are essential to monitor disease progression and treatment effectiveness.
Patients with CTEPH should be referred to expert centres for comprehensive evaluation and management. Lifelong anticoagulation is necessary, and warfarin is often preferred over newer agents. Exercise, tailored to individual capabilities, is also an important part of managing CTEPH.
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