Chronic Thromboembolic Pulmonary Hypertension
- Authors:
- Mahmud E, Madani MM, Kim NH, et al.
- Citation:
- 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):
- CTEPH is a rare consequence of an acute pulmonary embolism (PE), but is underdiagnosed.
- 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.
- 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.
- 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.
- 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).
- For inoperable patients with CTEPH, there is growing experience and excitement for balloon pulmonary angioplasty.
- Most patients undergoing balloon pulmonary angioplasty will require 4-6 separate sessions.
- More work is needed to develop standardized techniques and dedicated interventional equipment for balloon pulmonary angioplasty.
- Riociguat is the only approved medical therapy for patients with inoperable CTEPH or persistent PH after PTE.
- Expert multidisciplinary CTEPH teams should be developed at institutions caring for CTEPH patients.
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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