Commentary: Chronic thromboembolic pulmonary hypertension in Austria and Japan: The Sound of Music meets Madame Butterfly
2019; Elsevier BV; Volume: 158; Issue: 2 Linguagem: Inglês
10.1016/j.jtcvs.2019.01.080
ISSN1097-685X
AutoresChiao‐Yun Lin, Michael J. Reardon,
Tópico(s)Vascular Anomalies and Treatments
ResumoCentral MessageCTEPH is treated primarily by surgical endarterectomy in Austria and by balloon angioplasty in Japan. Disease anatomy and cause may help explain this.See Article page 604. CTEPH is treated primarily by surgical endarterectomy in Austria and by balloon angioplasty in Japan. Disease anatomy and cause may help explain this. See Article page 604. Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon condition characterized by obliteration of the major pulmonary arteries and associated with pulmonary hypertension. Symptoms can be nonspecific, and CTEPH remains underdiagnosed. Untreated, CTEPH can lead to heart failure and death and represents a potentially curable form of pulmonary hypertension. While in the past the only treatment available was surgical pulmonary endarterectomy (PEA), today 3 different treatment options are available: (1) surgical PEA1Thistlethwaite P.A. Mo M. Madani M.M. Deutsch R. Blanchard D. Kapelanski D.P. et al.Operative classification of thromboembolic disease determines outcome after pulmonary endarterectomy.J Thorac Cardiovasc Surg. 2002; 124: 1203-1211Abstract Full Text Full Text PDF PubMed Scopus (235) Google Scholar; (2) balloon pulmonary angioplasty2Kawakami T. Ogawa A. Miyaji K. Mizoguchi H. Shimokawahara H. Naito T. et al.Novel angiographic classification of each vascular lesion in chronic thromboembolic pulmonary hypertension based on selective angiogram and results of balloon pulmonary angioplasty.Circ Cardiovasc Interv. 2016; 9Crossref PubMed Scopus (123) Google Scholar; and (3) medical therapy, especially riociguat,3Ghofrani H.A. D'Armini A.M. Grimminger F. Hoeper M.M. Jansa P. Kim N.H. et al.Riociguat for the treatment of chronic thromboembolic pulmonary hypertension.N Engl J Med. 2013; 369: 319-329Crossref PubMed Scopus (932) Google Scholar with a combination of these therapies sometimes being used to optimize more complex cases. Surgical PEA has been the predominant form of intervention in Europe and the United States, whereas balloon pulmonary angioplasty has dominated in Japan, in part related to the anatomic distribution of disease: In 1 single-center report from Japan, 42% of patients were found to have subsegmental and peripheral disease,4Ogino H. Ando M. Matsuda H. Minatoya K. Sasaki H. Nakanishi N. et al.Japanese single-center experience of surgery for chronic thromboembolic pulmonary hypertension.Ann Thorac Surg. 2006; 82: 630-636Abstract Full Text Full Text PDF PubMed Scopus (94) Google Scholar whereas type I and II disease make up more than 75% of lesions in another series from University of California San Diego,1Thistlethwaite P.A. Mo M. Madani M.M. Deutsch R. Blanchard D. Kapelanski D.P. et al.Operative classification of thromboembolic disease determines outcome after pulmonary endarterectomy.J Thorac Cardiovasc Surg. 2002; 124: 1203-1211Abstract Full Text Full Text PDF PubMed Scopus (235) Google Scholar which has important implications for clinical outcomes after PEA. According to the annual reports of the Japanese Association for Thoracic Surgery, in-hospital mortality was 4.8%, 9.8%, and 0% for 2013, 2014, and 2015, respectively, with approximately 60 cases performed annually,5Masuda M. Okumura M. Doki Y. Endo S. Hirata Y. Kobayashi J. et al.Thoracic and cardiovascular surgery in Japan during 2014.Gen Thorac Cardiovasc Surg. 2016; 64: 665-697Crossref PubMed Scopus (184) Google Scholar, 6Masuda M. Kuwano H. Okumura M. Arai H. Endo S. Doki Y. et al.Committee for Scientific AffairsThe Japanese Association for Thoracic SurgeryThoracic and cardiovascular surgery in Japan during 2013: annual report by the Japanese Association for Thoracic Surgery.Gen Thorac Cardiovasc Surg. 2015; 63: 670-701Crossref PubMed Scopus (87) Google Scholar, 7Masuda M. Endo S. Natsugoe S. Shimizu H. Doki Y. Hirata Y. et al.Thoracic and cardiovascular surgery in Japan during 2015.Gen Thorac Cardiovasc Surg. 2018; 66: 581-615Google Scholar despite the typical distal nature of disease in Japan. Why the distribution of disease and therefore treatment approaches and outcomes should differ between Europe and the United States versus Japan is unclear. Understanding any differences might help clinicians understand the treatment options choices better. In this issue of the Journal, Chausheva and colleagues8Chausheva S, Naito A, Ogawa A, Seidl V, Winter MP, Sharma S, et al. Chronic thromboembolic pulmonary hypertension in Austria and Japan. J Thorac Cardiovasc Surg. 2019;158:604-14.e2.Google Scholar tackle this question of different treatment use and outcomes in Austria versus Japan in an attempt to better understand why these choices are made. They studied 20 consecutive patients with CTEPH consenting to the trial and receiving surgical PEA in Austria and Japan, and 20 consecutive nonoperated patients with CTEPH in Austria and Japan. They used 20 consecutive World Health Organization Group I patients with pulmonary arterial hypertension from Austria and Japan as controls. The study objective was to evaluate the clinical characteristics of the patients, the PEA surgical specimens, and the hemodynamics in both groups. The diagnosis of CTEPH was made using guideline definitions.9Galie N. Humbert M. Vachiery J.L. Gibbs S. Lang I. Torbicki A. 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 (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).Eur Heart J. 2016; 37: 67-119Crossref PubMed Scopus (4018) Google Scholar The decision for surgical PEA was made by the site considering the technical feasibility, risk, and patient consent. Surgical PEA was done as a complete, bilateral endarterectomy using median sternotomy and profound hypothermia with circulatory arrest as previously described.10Mahmud E. Madani M.M. Kim N.H. Poch D. Ang L. Behnamfar O. et al.Chronic thromboembolic pulmonary hypertension: evolving therapeutic approaches for operable and inoperable disease.J Am Coll Cardiol. 2018; 71: 2468-2486Crossref PubMed Scopus (90) Google Scholar All cases were managed at established CTEPH centers. Austrian patients were younger and larger than Japanese patients. A history of pulmonary embolism was present in 11 of 20 and 4 of 20 operated patients and 18 of 20 and 7 of 20 nonoperated patients from Austria and Japan, respectively. Analysis of the surgical specimens revealed a large, more central specimen, with more red thrombus in the Austrian patients possibly explaining the more common use of surgery. The Austrian patients also had more inflammatory markers than the Japanese patients. Because operability of CTEPH is an individual site decision that tends to be favored by a larger, more central obstruction burden, these findings may help explain the increased use of surgical PEA in Austria compared with Japan. Although Maria von Trapp and Cio-Cio San both married naval officers from Western countries in the context of musical theater, their final outcomes were markedly different but predictable before the second act. In contrast, recognizing and treating CTEPH variations related to clinical and cultural context, patient ethnic/genetic background, and even molecular pathophysiology remain a challenging and important target. Additional complexity arises from caring for patients from polyglot communities like the Houston metropolitan area, where any distribution of type I to IV disease can be seen on any given pulmonary angiography session. We believe that CTEPH ideally should be evaluated and treated at CTEPH centers of excellence. Our CTEPH center combines the expertise of the Houston Methodist DeBakey Heart & Vascular Center with the Houston Methodist Lung Center and adoption of best techniques of PEA, balloon pulmonary angioplasty, and medical therapy, learned from some of the very authors of the present article. The global collaboration demonstrated in the featured article represents the best side of the teaching and knowledge sharing that occur in the CTEPH community and contemporary medicine. Chronic thromboembolic pulmonary hypertension in Austria and JapanThe Journal of Thoracic and Cardiovascular SurgeryVol. 158Issue 2PreviewChronic thromboembolic pulmonary hypertension (CTEPH) is characterized by one or more of the following features: intraluminal thrombus organization, fibrous stenosis, and complete obliteration of major pulmonary arteries, amenable to significant improvement by pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty, and medical treatments with vasodilators. Because treatment practices and outcomes differ in Europe versus Japan, we hypothesized that population-based characteristics of pulmonary vascular phenotypes may exist in Austria compared with Japan. Full-Text PDF Open Archive
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