Chronic Thromboembolic Pulmonary Hypertension

Mahmud E, Madani MM, Kim NH, et al.
Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease. J Am Coll Cardiol 2018;71:2468-2486.

The following are 10 points to remember from this review on evolving therapeutic approaches for operable and inoperable chronic thromboembolic pulmonary hypertension (CTEPH):

  1. CTEPH is a rare consequence of an acute pulmonary embolism (PE), but is underdiagnosed.
  2. Patients with CTEPH often present with progressive dyspnea, exercise intolerance, and nonspecific abnormalities on physical examination. As the disease progresses, there is a high risk of developing PH and right heart failure.
  3. The diagnosis of CTEPH should begin with an echocardiogram and ventilation/perfusion (V/Q) scan prior to computed tomography, magnetic resonance imaging, or invasive pulmonary angiogram. A normal V/Q scan usually excludes a diagnosis of CTEPH.
  4. Surgical pulmonary thromboendarterectomy (PTE) is the preferred treatment for patients with CTEPH. Since 1970, the perioperative mortality rate has declined from ~20% to <2% at expert institutions.
  5. A recent intraoperative classification of the disease (levels I-IV) categorizes patients based on the location of the fibrotic thromboembolic material, starting with the main pulmonary arteries (level I) and progressing to subsegmental branches (level IV).
  6. For inoperable patients with CTEPH, there is growing experience and excitement for balloon pulmonary angioplasty.
  7. Most patients undergoing balloon pulmonary angioplasty will require 4-6 separate sessions.
  8. More work is needed to develop standardized techniques and dedicated interventional equipment for balloon pulmonary angioplasty.
  9. Riociguat is the only approved medical therapy for patients with inoperable CTEPH or persistent PH after PTE.
  10. Expert multidisciplinary CTEPH teams should be developed at institutions caring for CTEPH patients.

Clinical Topics: Cardiac Surgery, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Heart Failure, Acute Heart Failure, Pulmonary Hypertension, Interventions and Imaging, Interventions and Vascular Medicine, Angiography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging

Keywords: Angiography, Angioplasty, Cardiac Surgical Procedures, Diagnostic Imaging, Dyspnea, Echocardiography, Endarterectomy, Heart Failure, Hypertension, Pulmonary, Magnetic Resonance Imaging, Pulmonary Embolism, Thromboembolism, Tomography, Vascular Diseases

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