Type:
Educational Exhibit
Keywords:
Pulmonary vessels, Thorax, Interventional vascular, CT-Angiography, CT-High Resolution, Angioplasty, Education, Education and training
Authors:
V. Cosi1, G. Zanirato Rambaldi1, D. Attinà1, R. Ioppolo1, A. M. Chiesa1, F. Buia1, F. Niro2, V. Russo1, L. Lovato1; 1Bologna/IT, 2Bologna, Italy/IT
DOI:
10.26044/ecr2019/C-1610
Background
CTEPH is a potentially curable form of pulmonary hypertension classified into group 4 according to the latest guideline for pulmonary hypertension1,2.
It is due to the presence of organized thrombi causing pulmonary artery stenosis and/or obstructions with consequent abnormal elevation in the pulmonary artery resistance.
Furthermore,
it results in pulmonary hypertension,
hypoxia,
and right ventricular failure3.
Previously the prognosis of untreated patients with CTEPH and mean pulmonary arterial pressure (PAP) >30 mmHg was very poor (3-year survival rate of only 10%4,5).
CTEPH diagnosis is a complex process based on a multidisciplinary evaluation including detailed medical history,
physical examination,
chest radiography,
chest computed tomography,
transthoracic echocardiography,
lung ventilation–perfusion scintigraphy (evidence of ventilation-perfusion mismatch),
right heart catheterization,
and angiographic demonstration of multiple stenoses and obstructions of the bilateral pulmonary arteries6 (Figure 1).
In particular,
computed tomography pulmonary angiography (CTPA) is an imaging technique with a crucial role for definitive diagnosis of CTEPH and moreover,
once the diagnosis of CTEPH is confirmed,
it may be also used for assessment of operability providing anatomical details for surgical or interventional strategy planning.
Although the current established gold standard curative therapy for CTEPH is surgical,
based on pulmonary endarterectomy (PEA)3,
one-third of patients with CTEPH result inoperable according to the CTEPH international registry due to comorbidities and high-risk status predominantly because of distal lesions7.
For those patients with CTEPH judged inoperable,
are available medical (ie,
riociguat) and/or endovascular (balloon pulmonary angioplasty [BPA]) therapy8.
In particular,
BPA recently is showning to have an emerging role as an alternative treatment options for patients not eligible for surgery or with recurrent or persistent pulmonary hypertension following PEA9.