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Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Naoyuki Otani1, Ryo Watanabe2, Takashi Tomoe1

  • 1Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, Japan.

International Journal of Molecular Sciences
|February 25, 2023
PubMed

Insights

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious condition impacting the pulmonary artery. Treatments like pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) offer improved outcomes for patients.

Area of Science:

  • Cardiology
  • Pulmonology
  • Vascular Medicine

Background:

  • Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved blood clots in the pulmonary arteries, leading to pulmonary hypertension and right-sided heart failure.
  • Untreated CTEPH has a poor prognosis, characterized by progressive pulmonary vascular disease.
  • Anticoagulation therapy alone is insufficient for resolving established thrombi in CTEPH.

Purpose of the Study:

  • To review the complex pathogenesis of Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
  • To present the current standard of care, Pulmonary Endarterectomy (PEA), for CTEPH.
  • To discuss emerging treatments including Balloon Pulmonary Angioplasty (BPA) and pharmacotherapy for CTEPH.

Main Methods:

  • This review synthesizes current literature on CTEPH pathogenesis and treatment modalities.
  • It evaluates the efficacy and safety of Pulmonary Endarterectomy (PEA), the established surgical intervention.
  • It examines the advancements and clinical outcomes associated with Balloon Pulmonary Angioplasty (BPA) and pharmacological therapies.

Main Results:

  • Pulmonary Endarterectomy (PEA) remains the gold standard treatment, typically performed at specialized centers.
  • Balloon Pulmonary Angioplasty (BPA) has demonstrated significant progress in efficacy and safety for CTEPH management.
  • Several drug therapies have shown established evidence of effectiveness in treating CTEPH.

Conclusions:

  • CTEPH is a progressive disease requiring timely and specialized intervention.
  • PEA, BPA, and pharmacotherapy represent key treatment options for CTEPH, offering improved patient outcomes.
  • Ongoing research and technological advancements continue to enhance the management of CTEPH.

Keywords:
balloon pulmonary angioplastychronic thromboembolic pulmonary hypertensionpulmonary endarterectomy

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