scholarly journals Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH)

2021 ◽  
Author(s):  
Carmine Capone ◽  
Adele Valentini ◽  
Silvia Lina Spinillo ◽  
Catherine Klersy ◽  
Anna Celentano ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Microvascular Disease ◽  
Pulmonary Angiography ◽  
Key Points ◽  
Thromboembolic Pulmonary Hypertension ◽  
Perfusion Defects ◽  
Iodine Map ◽  
Vascular Obstruction

Abstract Objectives The aim of this study was to describe the radiological features of chronic thromboembolic pulmonary disease (CTEPD), not yet systematically described in the literature. Furthermore, we compared vascular scores between CTEPD and chronic thromboembolic pulmonary hypertension (CTEPH) patients, trying to explain why pulmonary hypertension does not develop at rest in CTEPD patients. Methods Eighty-five patients (40 CTEPD, 45 CTEPH) referred to our centre for pulmonary endarterectomy underwent dual-energy computed tomography pulmonary angiography (DE-CTPA) with iodine perfusion maps; other 6 CTEPD patients underwent single-source CTPA. CT scans were reviewed independently by an experienced cardiothoracic radiologist and a radiology resident to evaluate scores of vascular obstruction, hypoperfusion and mosaic attenuation, signs of pulmonary hypertension and other CT features typical of CTEPH. Results Vascular obstruction burden was similar in the two groups (p = 0.073), but CTEPD patients have a smaller extension of perfusion defects in the iodine map (p = 0.009) and a smaller number of these patients had mosaic attenuation (p < 0.001) than CTEPH patients, suggesting the absence of microvascular disease. Furthermore, as expected, the two groups were significantly different considering the indirect signs of pulmonary hypertension (p < 0.001). Conclusions CTEPD and CTEPH patients have significantly different radiological characteristics, in terms of signs of pulmonary hypertension, mosaic attenuation and iodine map perfusion extension. Importantly, our results suggest that the absence of peripheral microvascular disease, even in presence of an important thrombotic burden, might be the reason for the absence of pulmonary hypertension in CTEPD. Key Points • CTEPD and CTEPH patients have significantly different radiological characteristics. • The absence of peripheral microvascular disease might be the reason for the absence of pulmonary hypertension in CTEPD.

scholarly journals Chronic Thromboembolic Pulmonary Hypertension Medical Management

2021 ◽  
Vol 17 (2) ◽  
pp. e28-e32
Author(s):  
Ryan Logue ◽  
Zeenat Safdar
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Of Care ◽  
Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase Stimulator

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.

scholarly journals Clinical Updates on the Diagnosis and Management of Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
Vol 49 (5) ◽  
pp. 320-330
Author(s):  
Wen Ruan ◽  
Jonathan JL Yap ◽  
Kevin KH Quah ◽  
Foong Koon Cheah ◽  
Ghee Chee Phuah ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Residual Disease ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Pulmonary Endarterectomy ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Cardinal Symptom

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options. Methods: A PubMed search on articles relevant to pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed. Results: CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catherisation is important in the final diagnosis of CTEPH. Conclusion: Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options. Keywords: Balloon pulmonary angioplasty, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism, Pulmonary endarterectomy, Pulmonary hypertension

scholarly journals Frequency of computed tomography abnormalities in patients with chronic thromboembolic pulmonary hypertension: a comparative study between lung perfusion scan and computed tomography pulmonary angiography

2021 ◽  
Vol 16 ◽  
Author(s):  
Ahmed Fathala ◽  
Alaa Aldurabi
Keyword(s):  
Computed Tomography ◽  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Lung Perfusion ◽  
Computed Tomography Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Study Population ◽  
The Mean ◽  
Parenchymal Lung

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of pulmonary hypertension. Diagnosis of CTEPH can be established using various imaging techniques, including ventilation-perfusion scintigraphy (VQ) and multidetector computed tomography pulmonary angiography (CTPA). The aim of this study was to determine the frequency of direct pulmonary vascular, parenchymal lung, and cardiac abnormalities on CTPA in patients with CTEPH and to compare the diagnostic accuracy of both VQ scan CTPA in detecting CTEPH.Methods: we retrospectively included 54 patients who had been referred for pulmonary hypertension service (20 males, 34 females). All patients had VQ scan and CTPA within 15 days and underwent pulmonary artery endarterectomy (PEA) thereafter. VQ scan were reported according to modified PIOPED (Prospective Investigation of Pulmonary Embolism Diagnosis) criteria. CTPA was considered as diagnostic for CTEPH if it showed presence of thrombus, webs, stenosis, or perfusion lung abnormalities.Results: The mean age of the study population was 41±10 years. The mean pulmonary artery pressure was 53±13 mmHg. Fifty-three of 54 patients in the study population had high probability VQ scan and one patient had intermediate probability. CTPA was suggestive of CTEPH in all patients. The most frequent CTPA findings in the central pulmonary arteries and peripheral arteries were presence of thrombotic materials, abnormal vessel tapering and abrupt vessels-cut off (76% vs 65%, 67% vs 48%, and 48% vs 22%), respectively. The mosaic lung perfusion was present in 78% in the patients, and various cardiac morphology abnormalities were presents and most common was abnormal right to left ventricle ratio, 69%.Conclusion: Our findings indicate that both VQ scan and CTPA are highly sensitive for the detection of CTEPH confirmed by PEA. Most CTEPH patients had several pulmonary vascular, parenchymal lung and cardiac abnormalities. There was no sign with 100% sensitivity on CTPA for CTEPH detection.

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