Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable form of pulmonary hypertension and right heart failure. CTEPH (group 4 pulmonary hypertension) is caused by persistent organized thromboembolic obstruction of the pulmonary arteries from incompletely resolved acute pulmonary embolism. CTEPH also may present without prior VTE history, which can contribute to its underrecognition. The true incidence of CTEPH is unclear, but is estimated to be approximately 3% after acute pulmonary embolism. V˙/Q˙ scintigraphy is the best screening test for CTEPH, with CT scan imaging and other advanced imaging methods now playing a larger role in disease detection and confirmation. Perfusion defects on V˙/Q˙ scintigraphy in the setting of pulmonary hypertension are suggestive of CTEPH, but pulmonary angiography and right heart catheterization are required for confirmation and treatment planning. CTEPH potentially is curative with pulmonary thromboendarterectomy surgery, with mortality rates of approximately 2% at expert centers. Advances in operative techniques are allowing more distal endarterectomies to be performed successfully with favorable outcomes. However, more than one-third of patients may be considered inoperable. Although these patients previously had minimal therapeutic options, effective treatments now are available with pharmacotherapy and balloon pulmonary angioplasty. Diagnosis of CTEPH should be considered in all patients with suspicion of pulmonary hypertension. Treatments for CTEPH have advanced with improvements in outcomes for both operable and inoperable patients. Therapy should be tailored based on multidisciplinary team evaluation to ensure optimal treatment response. Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable form of pulmonary hypertension and right heart failure. CTEPH (group 4 pulmonary hypertension) is caused by persistent organized thromboembolic obstruction of the pulmonary arteries from incompletely resolved acute pulmonary embolism. CTEPH also may present without prior VTE history, which can contribute to its underrecognition. The true incidence of CTEPH is unclear, but is estimated to be approximately 3% after acute pulmonary embolism. V˙/Q˙ scintigraphy is the best screening test for CTEPH, with CT scan imaging and other advanced imaging methods now playing a larger role in disease detection and confirmation. Perfusion defects on V˙/Q˙ scintigraphy in the setting of pulmonary hypertension are suggestive of CTEPH, but pulmonary angiography and right heart catheterization are required for confirmation and treatment planning. CTEPH potentially is curative with pulmonary thromboendarterectomy surgery, with mortality rates of approximately 2% at expert centers. Advances in operative techniques are allowing more distal endarterectomies to be performed successfully with favorable outcomes. However, more than one-third of patients may be considered inoperable. Although these patients previously had minimal therapeutic options, effective treatments now are available with pharmacotherapy and balloon pulmonary angioplasty. Diagnosis of CTEPH should be considered in all patients with suspicion of pulmonary hypertension. Treatments for CTEPH have advanced with improvements in outcomes for both operable and inoperable patients. Therapy should be tailored based on multidisciplinary team evaluation to ensure optimal treatment response. Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of pulmonary hypertension (PH) characterized by unresolved thromboembolic occlusions of the pulmonary arteries.1Delcroix M. Torbicki A. Gopalan D. et al.ERS statement on chronic thromboembolic pulmonary hypertension.Eur Respir J. 2021; 572002828Crossref Scopus (186) Google Scholar,2Kim N.H. Delcroix M. Jais X. et al.Chronic thromboembolic pulmonary hypertension.Eur Respir J. 2019; 531801915Crossref Scopus (388) Google Scholar These chronic obstructions become organized and fibrotic and, along with concomitant remodeling of the pulmonary vasculature, can lead to progressive PH, right heart failure, and death. Since CTEPH was identified nearly 100 years ago, the diagnostic methods and therapeutic foundations of CTEPH have evolved. Advancements in surgical technique have been made, and the roles for balloon pulmonary angioplasty (BPA) and medical therapy have grown. For this review, a literature search was conducted in PubMed using the following terms: CTEPH, chronic thromboembolic pulmonary hypertension, chronic thromboembolic disease, pulmonary thromboendarterectomy, pulmonary endarterectomy, and balloon pulmonary angioplasty. The lead and senior authors screened the search list for relevance to narrow the list to the references provided. All included references were read in full. The aftermath of acute pulmonary embolism (PE) includes a spectrum of outcomes that range from full resolution of symptoms with complete restoration of normal perfusion to residual chronic obstruction that contributes to PH and persistent cardiopulmonary limitations. After acute PE, approximately 30% to 50% of patients will show abnormal perfusion scans after 6 months of anticoagulation.3Sanchez O. Helley D. Couchon S. et al.Perfusion defects after pulmonary embolism: risk factors and clinical significance.J Thromb Haemost. 2010; 8: 1248-1255Abstract Full Text Full Text PDF PubMed Scopus (124) Google Scholar,4Pesavento R. Filippi L. Palla A. et al.Impact of residual pulmonary obstruction on the long-term outcome of patients with pulmonary embolism.Eur Respir J. 2017; 491601980Crossref PubMed Scopus (75) Google Scholar Of those patients, only 10% to 15% will proceed to demonstrate CTEPH.3Sanchez O. Helley D. Couchon S. et al.Perfusion defects after pulmonary embolism: risk factors and clinical significance.J Thromb Haemost. 2010; 8: 1248-1255Abstract Full Text Full Text PDF PubMed Scopus (124) Google Scholar Chronic thromboembolic pulmonary disease (CTEPD) without PH is an increasingly recognized entity on the spectrum of possible outcomes after PE and is characterized by the presence of persistent perfusion defects with associated symptoms, but no resting PH. The true incidence of CTEPH is unknown, and current reported rates after PE range from 0.5% to 9%.5Ende-Verhaar Y.M. Cannegieter S.C. Noordegraaf A.V. et al.Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature.Eur Respir J. 2017; 491601792Crossref PubMed Scopus (286) Google Scholar, 6Pengo V. Lensing A.W.A. Prins M.H. et al.Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.N Engl J Med. 2004; 350: 2257-2264Crossref PubMed Scopus (1517) Google Scholar, 7Valerio L. Mavromanoli A.C. Barco S. et al.Chronic thromboembolic pulmonary hypertension and impairment after pulmonary embolism: the FOCUS study.Eur Heart J. 2022; 43: 3387-3398Crossref PubMed Scopus (46) Google Scholar This wide variability may reflect referral bias, study design, and screening strategies used, but in clinical practice, an incidence of approximately 3% is likely most relevant. The landmark study by Pengo et al,6Pengo V. Lensing A.W.A. Prins M.H. et al.Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.N Engl J Med. 2004; 350: 2257-2264Crossref PubMed Scopus (1517) Google Scholar which followed up 223 patients prospectively after acute PE for up to 10 years, reported a CTEPH incidence of 3.1%. Similarly, a meta-analysis of 16 studies evaluating the incidence of CTEPH found the rate to be 3.2% in survivors of PE.5Ende-Verhaar Y.M. Cannegieter S.C. Noordegraaf A.V. et al.Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature.Eur Respir J. 2017; 491601792Crossref PubMed Scopus (286) Google Scholar Still, CTEPH likely remains underdiagnosed. An estimated 300,000 of acute PE in the A. et disease and a from the Heart PubMed Scopus Google Scholar with of CTEPH, that lead to approximately to CTEPH in those without a of prior However, CTEPH are in the with only pulmonary endarterectomies performed for chronic thromboembolic pulmonary 2021; PubMed Scopus Google Scholar Although CTEPH is by of the larger pulmonary is the of PH. of disease also in and in patients with chronic thromboembolic pulmonary Full Text Full Text PDF PubMed Scopus Google Scholar this in of patients with and and to those in patients with pulmonary hypertension. The for this is the of from the vasculature, in and in those However, this was in both the and studies identified the and pulmonary arteries and which now are to be in the of distal to S. et disease in chronic thromboembolic pulmonary a role for pulmonary and Respir J. PubMed Scopus Google Scholar Although these to perfusion to distal to the of the pulmonary to the of the can to of the PE are associated with the of PE, a diagnostic of and right at of PE were found to be of O. M. et of a clinical for chronic thromboembolic pulmonary hypertension after acute pulmonary Thromb Haemost. Full Text Full Text PDF PubMed Scopus Google Scholar the PE a also was associated with an risk of CTEPH, with study that more than of patients with CTEPH had a of V. Lensing A.W.A. Prins M.H. et al.Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.N Engl J Med. 2004; 350: 2257-2264Crossref PubMed Scopus (1517) Google D. S. et factors for chronic thromboembolic pulmonary hypertension.Eur Respir J. PubMed Scopus Google Scholar However, up to of patients with CTEPH a of J. Delcroix M. et al.Chronic thromboembolic pulmonary hypertension from an PubMed Scopus Google Scholar medical are associated with a risk of CTEPH Although VTE is a risk for CTEPH, most of the that acute VTE risk are associated with M. et risk factors in pulmonary hypertension.Eur Respir J. PubMed Scopus Google Scholar The is the presence of which is associated with of D. S. et factors for chronic thromboembolic pulmonary hypertension.Eur Respir J. PubMed Scopus Google Scholar study that approximately of patients with CTEPH the presence of M. et risk factors in pulmonary hypertension.Eur Respir J. PubMed Scopus Google Scholar and abnormal and also have been associated with and risk factors for chronic thromboembolic pulmonary Full Text Full Text PDF PubMed Scopus Google Scholar patients with CTEPH were more likely to have and D. S. et factors for chronic thromboembolic pulmonary hypertension.Eur Respir J. PubMed Scopus Google D. J. et the risk of chronic thromboembolic pulmonary Haemost. PubMed Scopus Google Scholar with and also are at risk of CTEPH D. S. et factors for chronic thromboembolic pulmonary hypertension.Eur Respir J. PubMed Scopus Google Scholar CTEPH is chronic is present in the pulmonary arteries in the setting of PH, which is pulmonary of pulmonary of and pulmonary of M. et for the and treatment of pulmonary hypertension.Eur Respir J. 2022; Scholar The in CTEPH is the in patients for PH. The and been a diagnostic for CTEPH and that a of suspicion is required to N.H. Delcroix M. et al.Chronic thromboembolic pulmonary PubMed Scopus Google Scholar both the and CTEPH the from to was 10 and J. Delcroix M. et al.Chronic thromboembolic pulmonary hypertension from an PubMed Scopus Google et from the Chronic and 2021; Full Text Full Text PDF PubMed Scopus Google Scholar in CTEPH with associated with Barco S. et of diagnostic in chronic thromboembolic pulmonary from the CTEPH Respir J. PubMed Scopus Google Scholar are and can be in other cardiopulmonary patients will and progressive other of right heart failure, and can also can in CTEPH, likely to the Similarly, the can the disease caused by pulmonary can be in approximately 30% of N.H. in patients with operable chronic thromboembolic pulmonary hypertension are associated with and of Scholar is but may show pulmonary can show normal in CTEPH, but both may be also can the of with pulmonary disease have a on cardiopulmonary that can and J. S. in pulmonary 2017; PubMed Scopus Google Scholar The role of cardiopulmonary and in the of without PH may be M. N.H. Chronic thromboembolic with cardiopulmonary and for 2021; Full Text Full Text PDF Scopus Google Scholar of of this the of possible PH from of and estimated right However, both can and right PH in up to 30% of the will show normal at in of without J. et of pulmonary a from the of PubMed Scopus Google Scholar Still, remains to and and presence of V˙/Q˙ scintigraphy remains the imaging test of for screening for chronic thromboembolic D. Delcroix M. M. Diagnosis of chronic thromboembolic pulmonary hypertension.Eur Respir 2017; PubMed Scopus Google Scholar Although V˙/Q˙ CT scans can be more than scans in perfusion technique can be for The presence of persistent perfusion defects is a in CTEPH V˙/Q˙ scan CTEPH with a of to and of to et scintigraphy is more than in chronic thromboembolic pulmonary disease a treatable of pulmonary Med. PubMed Scopus Google Scholar However, V˙/Q˙ scintigraphy can the of pulmonary obstruction in Perfusion scan the of and in chronic thromboembolic pulmonary Full Text Full Text PDF PubMed Scopus Google Scholar the presence of a perfusion should to the of V˙/Q˙ scan also can be in other disease acute PE, pulmonary pulmonary and pulmonary S. R. of chronic thromboembolic pulmonary hypertension on imaging a PubMed Scopus Google Scholar Although CT pulmonary angiography is the test of for acute PE, should be the test for the evaluation of have rates of in CTEPH from to this wide variability likely of CTEPH than a of the imaging et scintigraphy is more than in chronic thromboembolic pulmonary disease a treatable of pulmonary Med. PubMed Scopus Google J. et of chronic thromboembolic pulmonary of and pulmonary angiography with pulmonary PubMed Scopus Google Scholar can the suggestive of CTEPH and However, these can be acute PE can be by with Gopalan D. chronic thromboembolic disease on 2019; PubMed Scopus Google Scholar of to the and to for D. Delcroix M. M. Diagnosis of chronic thromboembolic pulmonary hypertension.Eur Respir 2017; PubMed Scopus Google Scholar CT scan have a of of of and D. Delcroix M. M. Diagnosis of chronic thromboembolic pulmonary hypertension.Eur Respir 2017; PubMed Scopus Google Scholar The of perfusion a for CTEPH with that of V˙/Q˙ M. J. et CT perfusion in pulmonary rate with scintigraphy in chronic thromboembolic pulmonary hypertension PubMed Scopus Google Scholar also been in the evaluation of CTEPH and in PH, but is M. Torbicki A. Gopalan D. et al.ERS statement on chronic thromboembolic pulmonary hypertension.Eur Respir J. 2021; 572002828Crossref Scopus (186) Google Scholar of CTEPH right heart catheterization and, pulmonary D. Delcroix M. M. Diagnosis of chronic thromboembolic pulmonary hypertension.Eur Respir 2017; PubMed Scopus Google Scholar heart catheterization is required to PH and can disease pulmonary angiography is performed to of the with CTEPH complete and Although pulmonary angiography been to the imaging in CTEPH may be required in all patients. patients, a with without perfusion may be for both and evaluation of All patients with CTEPH should a multidisciplinary team evaluation to the optimal and treatment N.H. Delcroix M. 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