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 Diagnosis of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

2020 ◽  
Vol 55 (6) ◽  
pp. 2000189 ◽  
Author(s):  
Fredrikus A. Klok ◽  
Francis Couturaud ◽  
Marion Delcroix ◽  
Marc Humbert
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Acute Pulmonary Embolism ◽  
Functional Limitations ◽  
Diagnostic Delay ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Clear Guideline

Chronic thromboembolic pulmonary hypertension (CTEPH) is the most severe long-term complication of acute pulmonary embolism (PE). Untreated CTEPH is fatal, but, if diagnosed in time, successful surgical (pulmonary endarterectomy), medical (pulmonary hypertension drugs) and/or interventional (balloon pulmonary angioplasty) therapies have been shown to improve clinical outcomes, especially in case of successful pulmonary endarterectomy. Early diagnosis has however been demonstrated to be challenging. Poor awareness of the disease by patients and physicians, high prevalence of the post-PE syndrome (i.e. persistent dyspnoea, functional limitations and/or decreased quality of life following an acute PE diagnosis), lack of clear guideline recommendations as well as inefficient application of diagnostic tests in clinical practice lead to a reported staggering diagnostic delay >1 year. Hence, there is a great need to improve current clinical practice and diagnose CTEPH earlier. In this review, we will focus on the clinical presentation of and risk factors for CTEPH, and provide best practices for PE follow-up programmes from expert centres, based on a clinical case.

scholarly journals Dynamic vascular changes in chronic thromboembolic pulmonary hypertension after pulmonary endarterectomy

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402090788 ◽  
Author(s):  
Dieuwertje Ruigrok ◽  
Natalia J. Braams ◽  
Esther J. Nossent ◽  
Peter I. Bonta ◽  
Anco Boonstra ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Vascular Lesions ◽  
Heart Catheterization ◽  
Pulmonary Endarterectomy ◽  
Ct Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Residual Pulmonary Hypertension

Residual pulmonary hypertension is an important sequela after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Recurrent thrombosis or embolism could be a contributor to this residual pulmonary hypertension but the potential extent of its role is unknown in part because data on incidence are lacking. We aimed to analyze the incidence of new intravascular abnormalities after pulmonary endarterectomy and determine hemodynamic and functional implications. A total of 33 chronic thromboembolic pulmonary hypertension patients underwent routine CT pulmonary angiography before and six months after pulmonary endarterectomy, together with right heart catheterization and exercise testing. New vascular lesions were defined as (1) a normal pulmonary artery before pulmonary endarterectomy and containing a thrombus, web, or early tapering six months after pulmonary endarterectomy or (2) a pulmonary artery already containing thrombus, web, or early tapering at baseline, but increasing six months after pulmonary endarterectomy. Nine of 33 (27%) chronic thromboembolic pulmonary hypertension patients showed new vascular lesions on CT pulmonary angiography six months after pulmonary endarterectomy. In a subgroup of patients undergoing CT pulmonary angiography 18 months after pulmonary endarterectomy, no further changes in lesions were noted. Hemodynamic and functional outcomes were not different between patients with and without new vascular lesions. New vascular lesions are common after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension; currently their origin, dynamics, and long-term consequences remain unknown.

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