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Evolving spectrum of treatment for CTEPH
Vikramjit S Khangoora1, Oksana A Shlobin
1Inova Fairfax Hospital, Advanced Lung Disease and Transplant Program, Falls Church, Virginia, USA.
Chronic thromboembolic pulmonary hypertension (CTEPH) treatment options include surgery (PTE), medication (riociguat), and balloon pulmonary angioplasty (BPA). PTE is preferred for accessible disease, while riociguat and BPA are options for inoperable cases.
Area of Science:
- Cardiology
- Pulmonary Medicine
- Vascular Surgery
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare condition causing pulmonary hypertension due to chronic blood clots in lung arteries.
- Untreated CTEPH leads to severe illness and death.
Purpose of the Study:
- To review current treatment strategies for chronic thromboembolic pulmonary hypertension (CTEPH).
- To highlight recent advancements in medical and procedural interventions for CTEPH.
Main Methods:
- Review of recent publications on CTEPH treatment.
- Analysis of data on surgical (PTE), medical (riociguat, ERAs, treprostinil), and interventional (BPA) therapies.
Main Results:
- Pulmonary thromboendarterectomy (PTE) is the primary treatment for surgically accessible CTEPH.
- Riociguat is the preferred medication for inoperable or persistent CTEPH.
- Balloon pulmonary angioplasty (BPA) shows promise as an adjunct therapy for inoperable disease affecting smaller vessels.
Conclusions:
- PTE remains the gold standard for operable CTEPH.
- Medical therapy with riociguat is effective for inoperable/persistent CTEPH.
- BPA is an emerging option for specific patient subsets, requiring further research on its role in the overall treatment algorithm.
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