Learning objectives
To illustrate imaging findings of chronic thromboembolic pulmonary hypertension (CTEPH) suitable for Balloon Pulmonary Angioplasty (BPA) in patients not eligible for Pulmonary Endo-Arterectomy (PEA) according to a single centre experience and literature analysis.
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...
Findings and procedure details
CTPA plays an essential role in evaluating patients with suspected CTEPH clearly depicting highly suggestive findings for CTEPH essentially divided into vascular and parenchymal signs10,11 (Figure 2).
Principal vascular signs are:
vessel complete obstruction;
vessel narrowing;
intimal irregularities;
bands and webs;
thickening of arterial wall;
collateral systemic supply.
Principal parenchymal signs are:
scars from prior pulmonary infarction;
mosaic pattern perfusion;
focal ground glass opacities;
cylindrical bronchial airway dilatation;
systemic perfusion of the peripheral pulmonary arterial bed.
Moreover CT may allow visualization of course,
connections and...
Conclusion
CTEPH is a life-threating condition especially for those patients with severe and inoperable disease.
BPA is a new alternative treatment option potentially able to provide marked improvements in symptoms and hemodynamics in selected CTEPH patients ineligible for PEA.
Therefore,
radiologists should be aware of this novel endovascular procedure and should be able to promptly recognize the main peri- and post-procedural complications.
References
1.
Simonneau G,
Gatzoulis MA,
Adatia I,
Celermajer D,
Denton C,
Ghofrani A,
Gomez Sanchez MA,
Krishna Kumar R,
Landzberg M,
Machado RF,
Olschewski H,
Robbins IM,
Souza R.
Updated clinical classification of pulmonary hypertension.
J Am Coll Cardiol.
2013;62(suppl 25):D34–D41.
2.
Galiè N,
Humbert M,
Vachiery JL,
et al.
2015.
ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society...