TY - JOUR AB - We present a 30-year-old man with pulmonary hypertension after pulmonary embolism. Pulmonary angiography showed multiple stenosis in the pulmonary vascular tree. We treated four of these stenoses by balloon angioplasty in three sessions. Pulmonary artery pressure was reduced from 90/25 mm Hg (mean 46) to 78/13 mm Hg (mean 35) with concomitant increase of aortic pressure from 105/60 mm Hg (mean 75) to 134/68 mm Hg (mean 90). Pulmonary perfusion scintigraphy showed increase of perfusion in the treated segments. Two procedures were followed by transient segmental pulmonary edema, but no other complications were noted. We conclude that balloon angioplasty is a promising method of lowering pulmonary artery pressure and improving pulmonary perfusion in suitable cases of pulmonary hypertension secondary to pulmonary embolism. AU - Voorburg, J. A.I. AU - Manger Cats, V. AU - Buis, B. AU - Bruschke, A. V.G. DO - 10.1378/chest.94.6.1249 IS - 6 PB - The American College of Chest Physicians PY - 1988 SP - 1249 EP - 1253 TI - Balloon angioplasty in the treatment of pulmonary hypertension caused by pulmonary embolism T2 - Chest UR - http://dx.doi.org/10.1378/chest.94.6.1249 VL - 94 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. Methods and Results-A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. Conclusions-The long-term prognosis of operated patients currently is excellent and better than the outcome of notoperated patients. AU - Delcroix, Marion AU - Lang, Irene AU - Pepke-Zaba, Joanna AU - Jansa, Pavel AU - D'Armini, Andrea M. AU - Snijder, Repke AU - Bresser, Paul AU - Torbicki, Adam AU - Mellemkjaer, Sören AU - Lewczuk, Jerzy AU - Simkova, Iveta AU - Barberà, Joan A. AU - De Perrot, Marc AU - Hoeper, Marius M. AU - Gaine, Sean AU - Speich, Rudolf AU - Gomez-Sanchez, Miguel A. AU - Kovacs, Gabor AU - Jais, Xavier AU - Ambroz, David AU - Treacy, Carmen AU - Morsolini, Marco AU - Jenkins, David AU - Lindner, Jaroslav AU - Dartevelle, Philippe AU - Mayer, Eckhard AU - Simonneau, Gérald DO - 10.1161/CIRCULATIONAHA.115.016522 IS - 9 KW - Endarterectomy KW - hypertension, pulmonary KW - pulmonary embolism KW - survival KW - thromboembolism PY - 2016 SP - 859 EP - 871 TI - Long-Term Outcome of Patients with Chronic Thromboembolic Pulmonary Hypertension : Results from an International Prospective Registry T2 - Circulation VL - 133 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension is an underdiagnosed condition. Patients typically present with the symptoms of right heart failure. Diagnosis is usually done by radionuclide ventilation/perfusion (VQ) scan, high-quality multidetector computed tomography (CT) or pulmonary angiography at expert centers. Pulmonary endarterectomy remains the corner stone in management of chronic thromboembolic pulmonary hypertension. Deep hypothermic circulatory arrest is commonly used for the operation at most centers. In-hospital mortality ranges from 1.7 to 14.2%. Pulmonary hemorrhage, reperfusion lung injury, and right ventricular failure remain major early post-operative concerns. Five-year survival is reported to be 76 to 89%. Long-term outcome depends on residual pulmonary hypertension. Balloon pulmonary angioplasty and medical management play an adjunctive role. Here, we provide a comprehensive review on surgical management of chronic thromboembolic pulmonary hypertension. AU - Mangukia, Chirantan AU - Rali, Parth AU - Desai, Parag AU - Ku, Tse Shuen Jade AU - Brann, Stacey AU - Patel, Shrey AU - Sunagawa, Gengo AU - Minakata, Kenji AU - Kehara, Hiromu AU - Toyoda, Yoshiya DO - 10.1007/s12055-021-01208-y KW - Chronic thromboembolic pulmonary hypertension KW - Pulmonary endarterectomy KW - Pulmonary thromboendarterectomy PB - Indian Journal of Thoracic and Cardiovascular Surgery PY - 2021 SP - 14 EP - 20 TI - Pulmonary endarterectomy T2 - Indian Journal of Thoracic and Cardiovascular Surgery ER - TY - GEN AB - Riociguat is a first-in-class soluble guanylate cyclase stimulator, approved for the treatment of adults with pulmonary arterial hypertension (PAH), inoperable chronic thromboembolic pulmonary hypertension (CTEPH), or persistent or recurrent CTEPH after pulmonary endarterectomy. Approval was based on the results of the phase III PATENT-1 (PAH) and CHEST-1 (CTEPH) studies, with significant improvements in the primary endpoint of 6-minute walk distance vs placebo of +36 m and +46 m, respectively, as well as improvements in secondary endpoints such as pulmonary vascular resistance and World Health Organization functional class. Riociguat acts as a stimulator of cyclic guanosine monophosphate synthesis rather than as an inhibitor of cGMP metabolism. As with other approved therapies for PAH, riociguat has antifibrotic, antiproliferative and anti-inflammatory effects, in addition to vasodilatory properties. This has led to further clinical studies in patients who do not achieve a satisfactory clinical response with phosphodiesterase type-5 inhibitors. Riociguat has also been evaluated in patients with World Health Organization group 2 and 3 pulmonary hypertension, and other conditions including diffuse cutaneous systemic sclerosis, Raynaud's phenomenon and cystic fibrosis. This review evaluates the results of the original clinical trials of riociguat for the treatment of PAH and CTEPH, and summarises the body of work that has examined the safety and efficacy of riociguat for the treatment of other types of pulmonary hypertension. AU - Klinger, James R. AU - Chakinala, Murali M. AU - Langleben, David AU - Rosenkranz, Stephan AU - Sitbon, Olivier DA - 2021/7// DO - 10.1111/bcp.14676 IS - 7 KW - drug information KW - pharmacotherapy KW - therapeutics PB - John Wiley and Sons Inc PY - 2021 SP - 2645 EP - 2662 TI - Riociguat: Clinical research and evolving role in therapy T2 - British Journal of Clinical Pharmacology VL - 87 ER - TY - JOUR AU - Galiè, Nazzareno AU - Humbert, Marc AU - Vachiery, Jean Luc AU - Gibbs, Simon AU - Lang, Irene AU - Torbicki, Adam AU - Simonneau, Gérald AU - Peacock, Andrew AU - Noordegraaf, Anton Vonk AU - Beghetti, Maurice AU - Ghofrani, Ardeschir AU - Sanchez, Miguel Angel Gomez AU - Hansmann, Georg AU - Klepetko, Walter AU - Lancellotti, Patrizio AU - Matucci, Marco AU - McDonagh, Theresa AU - Pierard, Luc A. AU - Trindade, Pedro T. AU - Zompatori, Maurizio AU - Hoeper, Marius DO - 10.1183/13993003.01032-2015 IS - 4 PY - 2015 SP - 903 EP - 975 TI - 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension T2 - European Respiratory Journal UR - http://dx.doi.org/10.1183/13993003.01032-2015 VL - 46 ER - TY - GEN AB - Objective: To evaluate clinical literature for direct oral anticoagulants (DOACs) therapy for non–Food and Drug Administration approved indications. Data Sources: Articles from MEDLINE, Cochrane Library, Google Scholar, and OVID databases were reviewed from 1946 through September 4, 2020. Study Selection and Data Extraction: Fully published studies assessing DOACs for atrial fibrillation (AF) with valvular heart disease (VHD), heart failure (HF), left ventricular thrombus (LVT), superficial vein thrombosis (SVT), or pulmonary hypertension (PH) were evaluated. Data Synthesis: Our review showed that DOACs are safe to use in patients with AF and VHD except for mitral stenosis or mechanical heart valve. Rivaroxaban 2.5 mg twice daily should be used with caution in patients with HF with reduced ejection fraction until further evaluation is performed. Four retrospective studies for DOAC use in patients with LVT showed conflicting results. One phase 3 randomized controlled trial showed noninferiority of rivaroxaban to fondaparinux for SVT treatment. The use of DOACs for pulmonary arterial hypertension was not evaluated in any clinical study, but 2 retrospective studies for the use of DOACs in patients with chronic thromboembolic PH (CTEPH) showed similar efficacy between DOACs and warfarin. Relevance to Patient Care and Clinical Practice: This review provides clinicians with a comprehensive literature review surrounding DOAC use in common off-label indications. Conclusion: DOACs can be considered for AF complicated by VHD except for mitral stenosis or mechanical valve replacement. DOACs (especially rivaroxaban) are considered as an alternative therapy for SVT and CTEPH. Further prospective studies for DOAC uses are needed for HF or LVT. AU - Brokmeier, Hannah AU - Kido, Kazuhiko DA - 2021/8// DO - 10.1177/1060028020970618 IS - 8 KW - atrial fibrillation KW - direct oral anticoagulants KW - heart failure KW - left ventricular thrombus KW - pulmonary hypertension KW - superficial vein thrombosis PB - SAGE Publications Inc. PY - 2021 SP - 995 EP - 1009 TI - Off-label Use for Direct Oral Anticoagulants: Valvular Atrial Fibrillation, Heart Failure, Left Ventricular Thrombus, Superficial Vein Thrombosis, Pulmonary Hypertension—a Systematic Review T2 - Annals of Pharmacotherapy VL - 55 ER - TY - JOUR AU - Hoole, Stephen P. AU - Jenkins, David P. DO - 10.1136/heartjnl-2019-316291 IS - 19 KW - pulmonary vascular disease KW - thromboembolic pulmonary vascular disease PY - 2020 SP - 1525 EP - 1531 TI - Chronic thromboembolic pulmonary hypertension: Interventional approaches T2 - Heart VL - 106 ER - TY - JOUR AB - Background Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. Methods The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II–IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm5 and a walk distance of 150–450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo. Treatment with phosphodiesterase type-5 inhibitors and oral or inhaled prostanoids was permitted for WHO functional class III/IV patients. The primary endpoint was resting PVR at week 16, expressed as percentage of PVR measured at baseline. Analyses were done in all patients who were randomly assigned to treatment; safety analyses were done in all patients who received at least one dose of the study drug. This study is registered with ClinicalTrials.gov, number NCT02021292. Findings Between April 3, 2014, and March 17, 2016, we screened 186 patients for eligibility at 48 hospitals across 20 countries. Of these, 80 patients in 36 hospitals were randomly assigned to treatment (40 patients to macitentan, 40 patients to placebo). At week 16, geometric mean PVR decreased to 73·0% of baseline in the macitentan group and to 87·2% in the placebo group (geometric means ratio 0·84, 95% CI 0·70–0·99, p=0·041). The most common adverse events in the macitentan group were peripheral oedema (9 [23%] of 40 patients) and decreased haemoglobin (6 [15%]). Interpretation In MERIT-1, macitentan significantly improved PVR in patients with inoperable CTEPH and was well tolerated. Funding Actelion Pharmaceuticals Ltd. AU - Ghofrani, Hossein Ardeschir AU - Simonneau, Gérald AU - D'Armini, Andrea M. AU - Fedullo, Peter AU - Howard, Luke S. AU - Jaïs, Xavier AU - Jenkins, David P. AU - Jing, Zhi Cheng AU - Madani, Michael M. AU - Martin, Nicolas AU - Mayer, Eckhard AU - Papadakis, Kelly AU - Richard, Dominik AU - Kim, Nick H. AU - Lang, Irene AU - Kähler, Christian AU - Delcroix, Marion AU - Bshouty, Zoheir AU - Varela, Pablo Sepulveda AU - Jing, Zhi Cheng AU - Yang, Yuanhua AU - Liu, Jinming AU - Zhang, Gangcheng AU - Zhang, Nuofu AU - Mi, Yuhong AU - Zhu, Xianyang AU - Jansa, Pavel AU - Jaïs, Xavier AU - Prévot, Grégoire AU - Bouvaist, Hélène AU - Sanchez, Olivier AU - Grimminger, Friedrich AU - Held, Matthias AU - Wilkens, Heinrike AU - Rosenkranz, Stephan AU - Grünig, Ekkehard AU - Karlócai, Kristóf AU - Temesvári, András AU - Edes, Istvan AU - Aidietienė, Sigita AU - Miliauskas, Skaidrius AU - Zamudio, Tomas Rene Pulido AU - Sanchez, Carlos Jerjes AU - Noordegraaf, Anton Vonk AU - Lewczuk, Jerzy AU - Podolec, Piotr AU - Kasprzak, Jarosław AU - Mularek-Kubzdela, Tatiana AU - Grzywna, Ryszard AU - Dheda, Keertan AU - Moiseeva, Olga AU - Chernyavskiy, Alexander AU - Shipulin, Vladimir AU - Barbarash, Olga AU - Martynyuk, Tamila AU - Kim, Hyung Kwan AU - Park, Jun Bean AU - Lee, Jae Seung AU - Speich, Rudolf AU - Ulrich, Silvia AU - Aubert, John David AU - Phrommintikul, Arintaya AU - Jaimchariyatam, Nattapong AU - Sompradeekul, Suree AU - Onen, Zeynep Pinar AU - Okumus, Gulfer AU - Solovey, Lyubomyr AU - Gavrysyuk, Volodymyr AU - Howard, Luke AU - Pepke-Zaba, Joanna AU - Condliffe, Robin AU - McConnell, John AU - Kerr, Kim AU - Nguyen, Lan Hieu AU - Pham, Nguyen Vinh DA - 2017/10// DO - 10.1016/S2213-2600(17)30305-3 IS - 10 PB - Lancet Publishing Group PY - 2017 SP - 785 EP - 794 TI - Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study T2 - The Lancet Respiratory Medicine VL - 5 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension (PH). The disease is still underdiagnosed, and the true prevalence is unknown. CTEPH is characterized by intraluminal non-resolving thrombus organization and fibrous stenosis, or complete obliteration of pulmonary arteries, promoted by progressive remodeling of the pulmonary vasculature. One consequence of this is an increase in pulmonary vascular resistance and pressure, resulting in PH and progressive right heart failure, leading to death if left untreated. Endovascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for CTEPH patients. PEA surgery is the only technique that can potentially cure CTEPH disease, especially in patients with fresh or organized thrombi of the proximal branches of pulmonary arteries. However, not all patients are eligible for PEA surgery. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) and targeted medical therapy as additional promising available treatments options for inoperable CTEPH and recurrent/persistent PH after PEA surgery. Studies on BPA have shown it to improve pulmonary hemodynamics, symptoms, exercise capacity and RV function in inoperable CTEPH. Subsequently, BPA has developed into an essential component of the modern era of CTEPH treatment. Large randomized controlled trials have demonstrated varying significant improvements with targeted medical therapy in technically inoperable CTEPH patients. Thus, treatment of CTEPH requires a comprehensive multidisciplinary assessment, including an experienced PEA surgeon, PH specialist, BPA interventionist and CTEPH-trained radiologist at expert centers. In this comprehensive review, we address the latest developments in the fast-evolving field of CTEPH. These include advancements in imaging modalities and developments in operative and interventional techniques, which have widened the range of patients who may benefit from these procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed. As the treatment options for CTEPH improve, hybrid management involving multiple treatments in the same patient may become a viable option in the near future. AU - Gerges, Mario AU - Yacoub, Magdi DO - 10.21542/GCSP.2020.11 IS - 1 PY - 2020 TI - Chronic thromboembolic pulmonary hypertension - Still evolving T2 - Global Cardiology Science and Practice VL - 2020 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention. AU - Kim, Nick H. AU - Delcroix, Marion AU - Jais, Xavier AU - Madani, Michael M. AU - Matsubara, Hiromi AU - Mayer, Eckhard AU - Ogo, Takeshi AU - Tapson, Victor F. AU - Ghofrani, Hossein Ardeschir AU - Jenkins, David P. DO - 10.1183/13993003.01915-2018 IS - 1 PY - 2019 TI - Chronic thromboembolic pulmonary hypertension T2 - European Respiratory Journal UR - http://dx.doi.org/10.1183/13993003.01915-2018 VL - 53 ER - TY - JOUR AB - Objective: To estimate the prevalence and incidence of pulmonary hypertension (PH) in Colombia between the period between 2010 and 2014. Methodology: Cross-sectional study using the Data Warehouse of the Social Protection Information System (SISPRO) of the Ministry of Health and Social Protection (MSPS). We included all the nationwide patients who had registered the diagnoses of Primary Pulmonary Hypertension (ICD-10 I270) during the period established for the study. Results: It was estimated that the prevalence and incidence of PH is approximately 52 and 20 cases per million inhabitants, respectively. It predominates in the female sex and in the age groups over 60 years. Conclusion: Epidemiological estimates of PH in Colombia are consistent with the definition of orphan or rare diseases. AU - Miranda, Pablo A.M. AU - Guzmán Sáenz, Roberto C. AU - Ivan, Baños A. AU - Álvaro, Alvarez B. DO - 10.14482/sun.34.3.614.4 IS - 3 KW - Epidemiology KW - Incidence KW - Prevalence KW - Pulmonary hypertension PY - 2018 SP - 607 EP - 624 TI - Epidemiology of pulmonary hypertension in Colombia T2 - Salud Uninorte VL - 34 ER - TY - JOUR AB - El presente documento resume el marco de colaboración denominado “Red Colombiana de Hipertensión Pulmonar” entre miembros de diversas instituciones y especialidades médicas y paramédicas que tienen intereses académicos y científicos en el diagnóstico, tratamiento y/o investigación en áreas diversas de la Hipertensión Pulmonar. Esta iniciativa denominada con el acrónimo “HAPredco”, se justifica ampliamente por la relevancia científica de viabilizar un intercambio gremial, transdisciplinar y continuo mediante una Plataforma de Acción Continuada (PAC) de gestión de la información, experiencias, bases de datos y experticias alrededor de la hipertensión pulmonar. AU - Orozco-Levi, Mauricio AU - Conde Camacho, Rafael AU - Conrado Pacheco, Manuel AU - Londoño Villegas, Alejandro DO - 10.30789/rcneumologia.v33.n1.2021.545 IS - 1 PY - 2021 SP - 43 EP - 49 TI - La Red Colombiana de Hipertensión Pulmonar: Elementos de Gestión y Gobernanza Científica T2 - Revista Colombiana de Neumología VL - 33 ER - TY - JOUR AB - This systematic review of literature and online reports critically appraised incidence and prevalence estimates of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension to identify the most accurate estimates. Medline® and Embase® databases were searched for articles published between 1 January 2003 and 31 August 2020. Studies were grouped according to whether they were registries (population-based estimates), clinical databases (hospital-based estimates) or claims/administrative databases. Registries were classified into systematic and non-systematic registries, according to whether every national centre participated. Of 7309 publications identified, 5414 were screened after removal of duplicates and 33 were included. Inclusion was based on study type, availability of a clear numerator (diagnosed population) and a population- or hospital-based denominator, or all primary data required to calculate estimates. Only the most recent publication from a database was included. Most studies were based on European data and very few included children. In adults, the range of estimates per million was approximately 20-fold for pulmonary arterial hypertension incidence (1.5–32) and prevalence (12.4–268) and of similar magnitude for chronic thromboembolic pulmonary hypertension incidence (0.9–39) and prevalence (14.5–144). Recent (≤5 years) national systematic registry data from centralised healthcare systems provided the following ranges in adult estimates per million: approximately 5.8 for pulmonary arterial hypertension incidence, 47.6–54.7 for pulmonary arterial hypertension prevalence, 3.1–6.0 for chronic thromboembolic pulmonary hypertension incidence and 25.8–38.4 for chronic thromboembolic pulmonary hypertension prevalence. These estimates were considered the most reliable and consistent for the scientific community to plan for resource allocation and improve detection rates. AU - Leber, Laurence AU - Beaudet, Amélie AU - Muller, Audrey DO - 10.1177/2045894020977300 IS - 1 KW - epidemiology KW - pulmonary arterial hypertension KW - pulmonary hypertension KW - registries PB - SAGE Publications Ltd PY - 2021 TI - Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review T2 - Pulmonary Circulation VL - 11 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolismis observed inmost patientswith CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possiblemisdiagnosis ofCTEPHas acute pulmonaryembolism. A complete resolution of thrombi is usually not achieved after acute pulmonary embolism, and the clinical relevance of a "postpulmonary embolism syndrome" with persistent perfusion defects and exercise intolerance is discussed. Risk factors most consistently associated with CTEPH are circulating anti-phospholipid antibodies or lupus anticoagulant, increased factorVIII, non-Oblood groups, and chronic inflammatory diseases. There is no female predominance, and it is a disease of older age. Survival in the absence of specific surgical or medical treatment is poor and depends on the hemodynamic severity. AU - Delcroix, Marion AU - Kerr, Kim AU - Fedullo, Peter DO - 10.1513/AnnalsATS.201509-621AS KW - Epidemiology KW - Pulmonary Embolism KW - Pulmonary Hypertension KW - Risk Factors PY - 2016 SN - 0000000183949 SP - S201 EP - S206 TI - Chronic thromboembolic pulmonary hypertension: Epidemiology and risk factors T2 - Annals of the American Thoracic Society VL - 13 ER - TY - JOUR AB - The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is relevant for management decisions but is currently unknown. We performed a meta-analysis of studies including consecutive PE patients followed for CTEPH. Study cohorts were predefined as "all comers", "survivors" or "survivors without major comorbidities". CTEPH incidences were calculated using random effects models. We selected 16 studies totalling 4047 PE patients who were mostly followed up for >2-years. In 1186 all comers (two studies), the pooled CTEPH incidence was 0.56% (95% CI 0.1-1.0). In 999 survivors (four studies) CTEPH incidence was 3.2% (95% CI 2.0-4.4). In 1775 survivors without major comorbidities (nine studies), CTEPH incidence was 2.8% (95% CI 1.5-4.1). Both recurrent venous thromboembolism and unprovoked PE were significantly associated with a higher risk of CTEPH, with odds ratios of 3.2 (95% CI 1.7-5.9) and 4.1 (95% CI 2.1-8.2) respectively. The pooled CTEPH incidence in 12 studies that did not use right heart catheterisation as the diagnostic standard was 6.3% (95% CI 4.1-8.4). The 0.56% incidence in the all-comer group probably provides the best reflection of the incidence of CTEPH after PE on the population level. The ∼3% incidences in the survivor categories may be more relevant for daily clinical practice. Studies that assessed CTEPH diagnosis by tests other than right heart catheterisation provide overestimated CTEPH incidences. AU - Ende-Verhaar, Yvonne M. AU - Cannegieter, Suzanne C. AU - Noordegraaf, Anton Vonk AU - Delcroix, Marion AU - Pruszczyk, Piotr AU - Mairuhu, Albert T.A. AU - Huisman, Menno V. AU - Klok, Frederikus A. DO - 10.1183/13993003.01792-2016 IS - 2 PY - 2017 TI - Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: A contemporary view of the published literature T2 - European Respiratory Journal UR - http://dx.doi.org/10.1183/13993003.01792-2016 VL - 49 ER - TY - JOUR AB - BACKGROUND Chronic thromboembolic pulmonary hypertension (CTPH) is associated with considerable morbidity and mortality. Its incidence after pulmonary embolism and associated risk factors are not well documented. METHODS We conducted a prospective, long-term, follow-up study to assess the incidence of symptomatic CTPH in consecutive patients with an acute episode of pulmonary embolism but without prior venous thromboembolism. Patients with unexplained persistent dyspnea during follow-up underwent transthoracic echocardiography and, if supportive findings were present, ventilation-perfusion lung scanning and pulmonary angiography. CTPH was considered to be present if systolic and mean pulmonary-artery pressures exceeded 40 mm Hg and 25 mm Hg, respectively; pulmonary-capillary wedge pressure was normal; and there was angiographic evidence of disease. RESULTS The cumulative incidence of symptomatic CTPH was 1.0 percent (95 percent confidence interval, 0.0 to 2.4) at six months, 3.1 percent (95 percent confidence interval, 0.7 to 5.5) at one year, and 3.8 percent (95 percent confidence interval, 1.1 to 6.5) at two years. No cases occurred after two years among the patients with more than two years of follow-up data. The following increased the risk of CTPH: a previous pulmonary embolism (odds ratio, 19.0), younger age (odds ratio, 1.79 per decade), a larger perfusion defect (odds ratio, 2.22 per decile decrement in perfusion), and idiopathic pulmonary embolism at presentation (odds ratio, 5.70). CONCLUSIONS CTPH is a relatively common, serious complication of pulmonary embolism. Diagnostic and therapeutic strategies for the early identification and prevention of CTPH are needed. AU - Pengo, Vittorio AU - Lensing, Anthonie W.A. AU - Prins, Martin H. AU - Marchiori, Antonio AU - Davidson, Bruce L. AU - Tiozzo, Francesca AU - Albanese, Paolo AU - Biasiolo, Alessandra AU - Pegoraro, Cinzia AU - Iliceto, Sabino AU - Prandoni, Paolo DO - 10.1056/nejmoa032274 IS - 22 PY - 2004 SP - 2257 EP - 2264 TI - Incidence of Chronic Thromboembolic Pulmonary Hypertension after Pulmonary Embolism T2 - New England Journal of Medicine VL - 350 ER - TY - JOUR AB - BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. METHODS AND RESULTS-: The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. CONCLUSIONS-: Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments. © 2011 American Heart Association, Inc. AU - Pepke-Zaba, Joanna AU - Delcroix, Marion AU - Lang, Irene AU - Mayer, Eckhard AU - Jansa, Pavel AU - Ambroz, David AU - Treacy, Carmen AU - D'Armini, Andrea M. AU - Morsolini, Marco AU - Snijder, Repke AU - Bresser, Paul AU - Torbicki, Adam AU - Kristensen, Bent AU - Lewczuk, Jerzy AU - Simkova, Iveta AU - Barberà, Joan A. AU - De Perrot, Marc AU - Hoeper, Marius M. AU - Gaine, Sean AU - Speich, Rudolf AU - Gomez-Sanchez, Miguel A. AU - Kovacs, Gabor AU - Hamid, Abdul Monem AU - Jaïs, Xavier AU - Simonneau, Gérald DO - 10.1161/CIRCULATIONAHA.110.015008 IS - 18 KW - chronic disease KW - endarterectomy KW - hypertension KW - pulmonary PY - 2011 SP - 1973 EP - 1981 TI - Chronic thromboembolic pulmonary hypertension (CTEPH): Results from an international prospective registry T2 - Circulation VL - 124 ER - TY - JOUR AB - Objectives: To describe the characteristics of patients who undergo balloon pulmonary angioplasty (BPA) for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) and report the mid-term outcomes. Background: BPA has been recently introduced in Latin America. Mid-term results have not been published. Methods: Prospective Chilean Registry of inoperable CTEPH patients who underwent BPA. Clinical variables were analyzed at baseline, after each procedure and at follow-up. Hemodynamic variables were recorded before and after the last BPA. Results: Between August 2016 and September 2019, 22 patients (17 women), 59 ± 12.7 years, underwent 81 BPA and were followed for as long as 33.1 months (mean 17.3 ± 7.5). Mean pulmonary artery pressure decreased by 17.4% (51.1 ± 12 vs. 42.2 ± 13 mmHg, p =.001), pulmonary vascular resistance by 23.9% (766.7 ± 351 vs. 583 ± 346 dynes/s/cm−5, p =.001), cardiac index increased by 8% (2.3 ± 0.54 vs. 2.5 ± 0.54 L/min/m2, p =.012), N-terminal pro-B-type natriuretic peptide decreased by 73.8% (1,685 ± 1,045 vs. 441.8 ± 276 pg/dl, p =.006), and 6-min walk distance improved by 135 m (316.7 ± 94 vs. 451.1 ± 113 m, p =.001). One patient (4.5%) developed lung reperfusion injury and four patients (18.2%) had minor bleeding (hemoptysis), after the procedure. There was no mortality associated with BPA. Conclusions: Our results confirm that BPA for inoperable CTEPH is a relatively safe procedure that improves clinical and hemodynamic parameters in the mid-term. This therapy should be considered as an alternative, mainly in places where access to PAH therapy or surgery is restricted. AU - Sepúlveda, Pablo AU - Hameau, René AU - Backhouse, Christian AU - Charme, Gustavo AU - Pacheco, Francisco AU - Ramírez, Pablo A. AU - Fuensalida, Alberto J. AU - Quitral, Jorge AU - Martínez, Gonzalo AU - Martínez, José A. DO - 10.1002/ccd.29322 IS - 6 KW - angioplasty KW - chronic thromboembolic pulmonary hypertension KW - pulmonary hypertension KW - registry PY - 2021 SP - E748 EP - E757 TI - Mid-term follow-up of balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: An experience in Latin America T2 - Catheterization and Cardiovascular Interventions VL - 97 ER - TY - JOUR AU - Mahmud, Ehtisham AU - Maolo, Alessandro AU - Poch, David AU - Ang, Lawrence AU - Patel, Mitul AU - Kim, Nick DO - 10.1016/j.jacc.2018.08.1145 IS - 13 PB - Elsevier PY - 2018 SP - B25 EP - B26 TI - TCT-57 Hemodynamic and Functional Improvement Following Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Initial Experience at a High Volume Pulmonary Thromboendarterectomy Center T2 - Journal of the American College of Cardiology UR - http://dx.doi.org/10.1016/j.jacc.2018.08.1145 VL - 72 ER - TY - JOUR AB - Background-Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for pulmonary endarterectomy-the standard therapy. Currently, most reported results of BPA are from relatively small cohorts treated at single centers. The present study evaluated the safety and efficacy of BPA for chronic thromboembolic pulmonary hypertension based on a multicenter registry. Methods and Results-A total of 308 patients (62 men and 246 women; mean age, 61 years) underwent 1408 procedures at 7 institutions in Japan. Data were retrospectively reviewed to evaluate clinical outcome and complications. Hemodynamics were significantly improved in 249 patients in whom BPA was terminated, most often because of improvement in mean pulmonary arterial pressure or symptomatic improvement after 1154 procedures. In 196 patients who underwent followup right heart catheterization, improvement of hemodynamic parameters was maintained. Mean pulmonary arterial pressure decreased from 43.2±11.0 to 24.3±6.4 mm Hg after final BPA and 22.5±5.4 mm Hg at follow-up, with significant reduction of concomitant use of pulmonary hypertension-targeted therapy and oxygen supplementation. Complications occurred in 511 (36.3%), including pulmonary injury (17.8%), hemoptysis (14.0%), and pulmonary artery perforation (2.9%). Twelve patients (3.9%) died during follow-up, including 8 patients who died within 30 days after BPA. The leading causes of death were right heart failure, multiorgan failure, and sepsis. Overall survival was 96.8% (95% confidence interval, 93.7%-98.4%) at 1 and 2 years and 94.5% (95% confidence interval, 89.3%-97.3%) at 3 years, respectively, after the initial BPA procedure for all 308 patients. Conclusions-This multicenter registry suggested improved hemodynamic results after BPA. Complication rates were high, but overall survival was comparable with pulmonary endarterectomy. BPA may be an important therapeutic option in patients with chronic thromboembolic pulmonary hypertension. AU - Ogawa, Aiko AU - Satoh, Toru AU - Fukuda, Tetsuya AU - Sugimura, Koichiro AU - Fukumoto, Yoshihiro AU - Emoto, Noriaki AU - Yamada, Norikazu AU - Yao, Atsushi AU - Ando, Motomi AU - Ogino, Hitoshi AU - Tanabe, Nobuhiro AU - Tsujino, Ichizo AU - Hanaoka, Masayuki AU - Minatoya, Kenji AU - Ito, Hiroshi AU - Matsubara, Hiromi DO - 10.1161/CIRCOUTCOMES.117.004029 IS - 11 KW - Angioplasty KW - Hypertension KW - Lung injury KW - Pulmonary KW - Survival PY - 2017 SP - 1 EP - 7 TI - Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension results of a multicenter registry T2 - Circulation: Cardiovascular Quality and Outcomes VL - 10 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature. Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual’s operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team. Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an “expert centre” for the management of this condition. AU - Jenkins, David AU - Madani, Michael AU - Fadel, Elie AU - D’Armini, Andrea Maria AU - Mayer, Eckhard DO - 10.1183/16000617.0111-2016 IS - 143 PY - 2017 TI - Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension T2 - European Respiratory Review UR - http://dx.doi.org/10.1183/16000617.0111-2016 VL - 26 ER - TY - JOUR AB - Chronic thromboembolic disease (CTEPH) is one of the causes for developing pulmonary hypertension (PH). PH is characterized by an increase in pulmonary vascular pressure and resistance, ultimately leading to chronic overload. This study describes the clinical, functional, and hemodynamic characteristics as well as the established treatment strategy for a cohort of patients diagnosed with CTEPH in Bucaramanga, Colombia. In Colombia, PH is considered as an orphan disease with limited epidemiological data. We aim to provide useful information in order to help guide future clinical decisions for PH treatment and prevention. We conducted a cross-sectional study, obtaining clinical data from patients under follow-up, over 18 years of age, with hemodynamic confirmation of CTEPH in two pulmonary outpatient centers in Bucaramanga, Colombia between 2012 and 2018. 35 patients with diagnosis of CTEPH were included. Mean age was 52.3 ± 17.9 years. The mean time between the onset of symptoms to diagnosis was 14 months. 71% had a previous thrombotic event and 69% had functional class III and IV according to the world health organization (WHO) criteria. Most of the patients were classified as at high risk of mortality according to the European Society of Cardiology (ESC) and the European Respiratory Society (ERS/ESC) criteria and 60% were referred to undergo thromboendarterectomy. Most of the patients were under monotherapy treatment with Bosentan, the most prescribed medication in both monotherapy and dual therapy. This study identified a high number of patients in advanced stages of CETPH due to late diagnosis, related to health care limitations. This resulted in worse prognosis and quality of life. In addition, low adherence to non-pharmacological interventions was evidenced in patients who were not candidates for thromboendarterectomy despite the onset of pharmacological therapy. AU - Fajardo-Rivero, Javier Enrique AU - Mogollón, Melissa AU - García-Bohórquez, Diego Fernando AU - Villabona-Rueda, Andrés AU - Mendoza-Herrera, Tania AU - Ramírez-Sarmiento, Alba AU - Bolívar-Grimaldos, Fabio AU - Orozco-Levi, Mauricio DO - 10.3390/jor1020012 IS - 2 PY - 2021 SP - 105 EP - 113 TI - Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study) T2 - Journal of Respiration VL - 1 ER - TY - JOUR AU - Payares, Carlos DO - http://dx.doi.org/10.1016/j.rccar.2017.08.008 IS - 1 PY - 2017 SP - 48 EP - 54 TI - Diagnóstico y tratamiento de la hipertensión pulmonar tromboembólica crónica T2 - Revista Colombiana de Cardiología VL - 24 ER - TY - JOUR AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially fatal and frequently undiagnosed form of pulmonary hypertension (PH), classified within group 4 by the World Health Organization (WHO). It is a type of precapillary PH, which uncommonly develops as a peculiar sequel of acute pulmonary embolism due to the partial resolution of the mechanically obstructing thrombus with a coexisting inflammatory response from pulmonary vessels. CTEPH is one of the potentially treatable forms of PH whose current standard of care is surgical pulmonary endarterectomy. Medical therapy with few drugs in non-operable disease is approved and has shown improvement in patients' hemodynamic condition and functional ability. Recently, balloon pulmonary angioplasty (BPA) has shown promising results as a treatment option for technically inoperable patients, those with unacceptable risk-to-benefit ratio and in a case of residual PH after endarterectomy. Lack of meticulous CTEPH screening programs in post-pulmonary embolism patients leading to underdiagnosis of this condition, complex operability assessment, and diversity in BPA techniques among different institutions are still the issues that need to be addressed. In this paper, we review the recent achievements in the management of non-operable CTEPH, their outcome and safety, based on available data. AU - Rotzinger, David C. AU - Rezaei-Kalantari, Kiara AU - Aubert, John David AU - Qanadli, Salah D. DO - 10.1016/j.ejrad.2021.109562 IS - January KW - Chronic thromboembolic pulmonary hypertension KW - Endovascular procedure KW - Pulmonary angioplasty KW - Pulmonary embolism PB - Elsevier B.V. PY - 2021 SP - 109562 EP - 109562 TI - Pulmonary angioplasty: A step further in the continuously changing landscape of chronic thromboembolic pulmonary hypertension management T2 - European Journal of Radiology UR - https://doi.org/10.1016/j.ejrad.2021.109562 VL - 136 ER - TY - JOUR AB - Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention. BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%. BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk-benefit assessments. AU - Olsson, Karen M. AU - Wiedenroth, Christoph B. AU - Kamp, Jan Christopher AU - Breithecker, Andreas AU - Fuge, Jan AU - Krombach, Gabriele A. AU - Haas, Moritz AU - Hamm, Christian AU - Kramm, Thorsten AU - Guth, Stefan AU - Ghofrani, Hossein Ardeschir AU - Hinrichs, Jan B. AU - Cebotari, Serghei AU - Meyer, Katrin AU - Hoeper, Marius M. AU - Mayer, Eckhard AU - Liebetrau, Christoph AU - Meyer, Bernhard C. DO - 10.1183/13993003.02409-2016 IS - 6 PY - 2017 TI - Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: The initial German experience T2 - European Respiratory Journal UR - http://dx.doi.org/10.1183/13993003.02409-2016 VL - 49 ER - TY - JOUR AB - Background —Although pulmonary thromboendarterectomy is increasingly successful for the definitive treatment of chronic thromboembolic pulmonary hypertension (CTEPH), not all patients have surgically accessible disease. Others are poor surgical candidates because of comorbid illness. Therefore, for selected patients, we defined and implemented an alternative interventional strategy of balloon pulmonary angioplasty (BPA). AU - Feinstein, Jeffrey A. AU - Goldhaber, Samuel Z AU - Lock, James E AU - Ferndandes, Susan M AU - Landzberg, Michael J DA - 2001/1// DO - 10.1161/01.CIR.103.1.10 IS - 1 KW - balloon ⅲ angioplasty ⅲ KW - cteph KW - definitive treatment of chronic KW - embolism ⅲ thrombus ⅲ KW - increas- KW - ingly successful for the KW - lthough pulmonary thromboendarterectomy is KW - not all KW - pulmonary heart disease KW - thromboembolic pulmonary hypertension PY - 2001 SP - 10 EP - 13 TI - Balloon Pulmonary Angioplasty for Treatment of Chronic Thromboembolic Pulmonary Hypertension T2 - Circulation UR - https://www.ahajournals.org/doi/10.1161/01.CIR.103.1.10 VL - 103 ER - TY - JOUR AB - Background-Although balloon pulmonary angioplasty (BPA) for inoperable patients with chronic thromboembolic pulmonary hypertension was first reported over a decade ago, its clinical application has been restricted because of limited efficacy and complications. We have refined the procedure of BPA to maximize its clinical efficacy. Methods and Results-Sixty-eight consecutive patients with inoperable chronic thromboembolic pulmonary hypertension underwent BPA. We evaluated pulmonary artery diameters and determined the appropriate balloon size by using intravascular ultrasound. We performed BPA in a staged fashion over multiple, separate procedures to maximize efficacy and reduce the risk of reperfusion pulmonary injury. A total of 4 (2-8) sessions were performed in each patient, and the number of vessels dilated per session was 3 (1-14). The World Health Organization functional class improved from 3 to 2 (P<0.01), and mean pulmonary arterial pressure was decreased from 45.4±9.6 to 24.0±6.4 mm Hg (P<0.01). One patient died because of right heart failure 28 days after BPA. During follow-up for 2.2±1.4 years after the final BPA, another patient died of pneumonia, and the remaining 66 patients are alive. In 57 patients who underwent right heart catheterization at follow-up, improvement of mean pulmonary arterial pressure was maintained (24.0±5.8 mm Hg at 1.0±0.9 years). Forty-one patients (60%) developed reperfusion pulmonary injury after BPA, but mechanical ventilation was required in only 4 patients. Conclusions-Our refined BPA procedure improves clinical status and hemodynamics of inoperable patients with chronic thromboembolic pulmonary hypertension, with a low mortality. A refined BPA procedure could be considered as a therapeutic approach for patients with inoperable chronic thromboembolic pulmonary hypertension. © 2012 American Heart Association, Inc. AU - Mizoguchi, Hiroki AU - Ogawa, Aiko AU - Munemasa, Mitsuru AU - Mikouchi, Hiroshi AU - Ito, Hiroshi AU - Matsubara, Hiromi DO - 10.1161/CIRCINTERVENTIONS.112.971077 IS - 6 KW - Peripheral vascular disease KW - Pulmonary hypertension KW - Reperfusion KW - Revascularization PY - 2012 SP - 748 EP - 755 TI - Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension T2 - Circulation: Cardiovascular Interventions VL - 5 ER - TY - JOUR AU - Galiè, Nazzareno AU - Humbert, Marc AU - Vachiery, Jean Luc AU - Gibbs, Simon AU - Lang, Irene AU - Torbicki, Adam AU - Simonneau, Gérald AU - Peacock, Andrew AU - Vonk Noordegraaf, Anton AU - Beghetti, Maurice AU - Ghofrani, Ardeschir AU - Gomez Sanchez, Miguel Angel AU - Hansmann, Georg AU - Klepetko, Walter AU - Lancellotti, Patrizio AU - Matucci, Marco AU - McDonagh, Theresa AU - Pierard, Luc A. AU - Trindade, Pedro T. AU - Zompatori, Maurizio AU - Hoeper, Marius AU - Aboyans, Victor AU - Vaz Carneiro, Antonio AU - Achenbach, Stephan AU - Agewall, Stefan AU - Allanore, Yannick AU - Asteggiano, Riccardo AU - Paolo Badano, Luigi AU - Albert Barberà, Joan AU - Bouvaist, Hélène AU - Bueno, Héctor AU - Byrne, Robert A. AU - Carerj, Scipione AU - Castro, Graça AU - Erol, Çetin AU - Falk, Volkmar AU - Funck-Brentano, Christian AU - Gorenflo, Matthias AU - Granton, John AU - Iung, Bernard AU - Kiely, David G. AU - Kirchhof, Paulus AU - Kjellstrom, Barbro AU - Landmesser, Ulf AU - Lekakis, John AU - Lionis, Christos AU - Lip, Gregory Y.H. AU - Orfanos, Stylianos E. AU - Park, Myung H. AU - Piepoli, Massimo F. AU - Ponikowski, Piotr AU - Revel, Marie Pierre AU - Rigau, David AU - Rosenkranz, Stephan AU - Völler, Heinz AU - Luis Zamorano, Jose AU - Myftiu, Sokol AU - Bonderman, Diana AU - Firdovsi, Ibrahimov AU - Lazareva, Irina AU - De Pauw, Michel AU - Sokolović, Šekib AU - Velchev, Vasil AU - Čikeš, Maja AU - Moutiris, Josef Antoniou AU - Jansa, Pavel AU - Nielsen-Kudsk, Jens Erik AU - Anton, Ly AU - Jääskeläinen, Pertti AU - Bauer, Fabrice AU - Chukhrukidze, Archil AU - Opitz, Christian AU - Giannakoulas, George AU - Karlócai, Kristóf AU - Oddsson, Hjörtur AU - Gaine, Sean AU - Menachemi, Doron AU - Emdin, Michele AU - Sooronbaev, Talant AU - Rudzītis, Ainars AU - Gumbiene, Lina AU - Lebrun, Frederic AU - Micallef, Josef AU - Botnaru, Victor AU - Oukerraj, Latifa AU - Andreassen, Arne K. AU - Kurzyna, Marcin AU - Leite Baptista, Maria João Ribeiro AU - Coman, Ioan Mircea AU - Moiseeva, Olga AU - Stefanović, Branislav S. AU - Šimková, Iveta AU - Wikström, Gerhard AU - Schwerzmann, Markus AU - Srbinovska-Kostovska, Elizabeta AU - van Dijk, Arie P.J. AU - Mahdhaoui, Abdallah AU - Kaymaz, Cihangir AU - Coghlan, Gerry AU - Sirenko, Yuriy DO - 10.1093/eurheartj/ehv317 IS - 1 KW - Chronic thromboembolic pulmonary hypertension KW - Congenital heart disease KW - Connective tissue disease KW - Endothelin receptor antagonists KW - Guidelines KW - Heart failure KW - Left heart disease KW - Lung disease KW - Phosphodiesterase type 5 inhibitors KW - Prostacyclin analogues KW - Pulmonary arterial hypertension KW - Pulmonary hypertension KW - Respiratory failure PY - 2016 SP - 67 EP - 119 TI - 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension T2 - European Heart Journal VL - 37 ER - TY - JOUR AB - Background: The potential role of new oral anticoagulants in antiphospholipid antibody syndrome (APS) remains uncertain. Objective: To determine whether rivaroxaban is noninferior to dose-adjusted vitamin K antagonists (VKAs) for thrombotic APS. Design: 3-year, open-label, randomized noninferiority trial. (EU Clinical Trials Register: EUDRA [European Union Drug Regulatory Authorities] code 2010-019764-36) Setting: 6 university hospitals in Spain. Participants: 190 adults (aged 18 to 75 years) with thrombotic APS. Intervention: Rivaroxaban (20 mg/d or 15 mg/d, according to renal function) versus dose-adjusted VKAs (target international normalized ratio, 2.0 to 3.0, or 3.1 to 4.0 in patients with a history of recurrent thrombosis). Measurements: The primary efficacy outcome was the proportion of patients with new thrombotic events; the primary safety outcome was major bleeding. The prespecified noninferiority margin for risk ratio (RR) was 1.40. Secondary outcomes included time to thrombosis, type of thrombosis, changes in biomarker levels, cardiovascular death, and nonmajor bleeding. Results: After 3 years of follow-up, recurrent thrombosis occurred in 11 patients (11.6%) in the rivaroxaban group and 6 (6.3%) in the VKA group (RR in the rivaroxaban group, 1.83 [95% CI, 0.71 to 4.76]). Stroke occurred more commonly in patients receiving rivaroxaban (9 events) than in those receiving VKAs (0 events) (corrected RR, 19.00 [CI, 1.12 to 321.9]). Major bleeding occurred in 6 patients (6.3%) in the rivaroxaban group and 7 (7.4%) in the VKA group (RR, 0.86 [CI, 0.30 to 2.46]). Post hoc analysis suggested an increased risk for recurrent thrombosis in rivaroxaban-treated patients with previous arterial thrombosis, livedo racemosa, or APS-related cardiac valvular disease. Limitation: Anticoagulation intensity was not measured in the rivaroxaban group. Conclusion: Rivaroxaban did not show noninferiority to dose-adjusted VKAs for thrombotic APS and, in fact, showed a non-statistically significant near doubling of the risk for recurrent thrombosis. AU - Ordi-Ros, Josep AU - Sáez-Comet, Luis AU - Pérez-Conesa, Mercedes AU - Vidal, Xavier AU - Riera-Mestre, Antoni AU - Castro-Salomó, Antoni AU - Cuquet-Pedragosa, Jordi AU - Ortiz-Santamaria, Vera AU - Mauri-Plana, Montserrat AU - Solé, Cristina AU - Cortés-Hernández, Josefina DA - 2019/11// DO - 10.7326/M19-0291 IS - 10 PB - American College of Physicians PY - 2019 SP - 685 EP - 694 TI - Rivaroxaban versus Vitamin K antagonist in antiphospholipid syndrome a randomized noninferiority trial T2 - Annals of Internal Medicine VL - 171 ER - TY - JOUR AB - Objectives: Our goal was to investigate the effect of treatment with the oral dual endothelin receptor antagonist bosentan on the hemodynamics and exercise capacity of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Background: CTEPH is characterized by vascular obstruction and remodeling, leading to increased pulmonary vascular resistance (PVR). Although pulmonary endarterectomy (PEA) is potentially curative, medical therapy is needed in patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. Methods: The BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension) study was a double-blind, randomized, placebo-controlled study in CTEPH including patients with either inoperable CTEPH or persistent/recurrent pulmonary hypertension after PEA (>6 months after PEA). Independent coprimary end points were change in PVR as a percentage of baseline and change from baseline in 6-min walk distance after 16 weeks of treatment with bosentan or placebo. Secondary end points included change from baseline in World Health Organization functional class and other hemodynamic parameters. Results: One hundred fifty-seven patients were enrolled and randomized: 80 to placebo, 77 to bosentan. A statistically significant treatment effect (TE) of bosentan over placebo on PVR was demonstrated: -24.1% of baseline (95% confidence interval [CI]: -31.5% to -16.0%; p < 0.0001). Total pulmonary resistance (TE: -193 dyn·s·cm-5; 95% CI: -283 to -104 dyn·s·cm-5; p < 0.0001) and cardiac index (TE: 0.3 l·min-1·m-2; 95% CI: 0.14 to 0.46 l·min-1·m-2; p = 0.0007) improved. Mean TE on 6-min walk distance was +2.2 m (95% CI: -22.5 to 26.8 m; p = 0.5449). Bosentan treatment was well tolerated. Conclusions: This study demonstrated a positive TE of bosentan on hemodynamics in this patient population. No improvement was observed in exercise capacity. Further trials are needed to define the role of medical therapy in patients with CTEPH (Bosentan Effects in Inoperable Forms of Chronic Thromboembolic Pulmonary Hypertension; NCT00313222). © 2008 American College of Cardiology Foundation. AU - Jaïs, Xavier AU - D'Armini, Andrea M. AU - Jansa, Pavel AU - Torbicki, Adam AU - Delcroix, Marion AU - Ghofrani, Hossein A. AU - Hoeper, Marius M. AU - Lang, Irene M. AU - Mayer, Eckhard AU - Pepke-Zaba, Joanna AU - Perchenet, Loïc AU - Morganti, Adele AU - Simonneau, Gérald AU - Rubin, Lewis J. DO - 10.1016/j.jacc.2008.08.059 IS - 25 KW - bosentan KW - hemodynamics KW - operability KW - pulmonary endarterectomy PY - 2008 SP - 2127 EP - 2134 TI - Bosentan for Treatment of Inoperable Chronic Thromboembolic Pulmonary Hypertension. BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a Randomized, Placebo-Controlled Trial T2 - Journal of the American College of Cardiology VL - 52 ER - TY - JOUR AB - BACKGROUND Riociguat, a member of a new class of compounds (soluble guanylate cyclase stimulators), has been shown in previous clinical studies to be beneficial in the treatment of chronic thromboembolic pulmonary hypertension. METHODS In this phase 3, multicenter, randomized, double-blind, placebo-controlled study, we randomly assigned 261 patients with inoperable chronic thromboembolic pulmonary hypertension or persistent or recurrent pulmonary hypertension after pulmonary endarterectomy to receive placebo or riociguat. The primary end point was the change from baseline to the end of week 16 in the distance walked in 6 minutes. Secondary end points included changes from baseline in pulmonary vascular resistance, N-terminal pro-brain natriuretic peptide (NT-proBNP) level, World Health Organization (WHO) functional class, time to clinical worsening, Borg dyspnea score, quality-of-life variables, and safety. RESULTS By week 16, the 6-minute walk distance had increased by a mean of 39 m in the riociguat group, as compared with a mean decrease of 6 m in the placebo group (least-squares mean difference, 46 m; 95% confidence interval [CI], 25 to 67; P<0.001). Pulmonary vascular resistance decreased by 226 dyn·sec·cm(-5) in the riociguat group and increased by 23 dyn·sec·cm(-5) in the placebo group (least-squares mean difference, -246 dyn·sec·cm(-5); 95% CI, -303 to -190; P<0.001). Riociguat was also associated with significant improvements in the NT-proBNP level (P<0.001) and WHO functional class (P=0.003). The most common serious adverse events were right ventricular failure (in 3% of patients in each group) and syncope (in 2% of the riociguat group and in 3% of the placebo group). CONCLUSIONS Riociguat significantly improved exercise capacity and pulmonary vascular resistance in patients with chronic thromboembolic pulmonary hypertension. (Funded by Bayer HealthCare; CHEST-1 and CHEST-2 ClinicalTrials.gov numbers, NCT00855465 and NCT00910429, respectively.) AU - Ghofrani, Hossein-Ardeschir AU - D'Armini, Andrea M. AU - Grimminger, Friedrich AU - Hoeper, Marius M. AU - Jansa, Pavel AU - Kim, Nick H. AU - Mayer, Eckhard AU - Simonneau, Gerald AU - Wilkins, Martin R. AU - Fritsch, Arno AU - Neuser, Dieter AU - Weimann, Gerrit AU - Wang, Chen DA - 2013/7// DO - 10.1056/nejmoa1209657 IS - 4 PB - New England Journal of Medicine (NEJM/MMS) PY - 2013 SP - 319 EP - 329 TI - Riociguat for the Treatment of Chronic Thromboembolic Pulmonary Hypertension T2 - New England Journal of Medicine VL - 369 ER - TY - JOUR AB - Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention. BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%. BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk-benefit assessments. AU - Olsson, Karen M. AU - Wiedenroth, Christoph B. AU - Kamp, Jan Christopher AU - Breithecker, Andreas AU - Fuge, Jan AU - Krombach, Gabriele A. AU - Haas, Moritz AU - Hamm, Christian AU - Kramm, Thorsten AU - Guth, Stefan AU - Ghofrani, Hossein Ardeschir AU - Hinrichs, Jan B. AU - Cebotari, Serghei AU - Meyer, Katrin AU - Hoeper, Marius M. AU - Mayer, Eckhard AU - Liebetrau, Christoph AU - Meyer, Bernhard C. DO - 10.1183/13993003.02409-2016 IS - 6 PY - 2017 TI - Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: The initial German experience T2 - European Respiratory Journal UR - http://dx.doi.org/10.1183/13993003.02409-2016 VL - 49 ER - TY - JOUR AB - BACKGROUND: Balloon pulmonary angioplasty (BPA) is a promising therapy for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy. AIMS: The present study aimed to evaluate the safety and efficacy of BPA for CTEPH using the first multicentre registry of a single European country. METHODS: Data were obtained from the Database of Pulmonary Hypertension in the Polish Population (NCT03959748), a prospective, multicentre registry of adult and paediatric pulmonary arterial hypertension (PAH) and CTEPH, for a total of 236 patients with confirmed CTEPH (124 women; mean age 67 years) who underwent 1,056 BPA procedures at eight institutions in Poland. RESULTS: In 156 patients who underwent follow-up assessments after a median of 5.9 (IQR: 3.0-8.0) months after final BPA, the mean pulmonary arterial pressure decreased from 45.1±10.7 to 30.2±10.2 mmHg (p<0.001) and pulmonary vascular resistance from 642±341 to 324±183 dynes (p<0.001), and the six-minute walking test (6MWT) improved from 341±129 to 423±136 m (p<0.001). Pulmonary injury related to the BPA procedure occurred in 6.4% of all sessions. Eighteen patients (7.6%) died during follow-up, including 4 (1.7%) who died within 30 days after BPA. Overall survival was 92.4% (95% confidence interval [CI]: 87.6%-94.9%) three years after the initial BPA procedure. CONCLUSIONS: This multicentre registry confirmed significant improvement of haemodynamic, functional, and biochemical parameters after BPA. Complication rates were low and overall survival comparable to the results of another registry. Therefore, BPA may be an important therapeutic option in patients with CTEPH in Poland. AU - Darocha, Szymon AU - Roik, Marek AU - Kopeć, Grzegorz AU - Araszkiewicz, Aleksander AU - Furdal, Michał AU - Lewandowski, Maciej AU - Jacheć, Wojciech AU - Grabka, Marek AU - Banaszkiewicz, Marta AU - Piertrasik, Arkadiusz AU - Pietura, Radosław AU - Stępniewski, Jakub AU - Waligóra, Marcin AU - Magoń, Wojciech AU - Jonas, Kamil AU - Łabyk, Andrzej AU - Potępa, Michał AU - Fudryna, Aleksandra AU - Jankiewicz, Stanislaw AU - Sławek-Szmyt, Sylwia AU - Mularek-Kubzdela, Tatiana AU - Lesiak, Maciej AU - Mroczek, Ewa AU - Orłowska, Joanna AU - Peregud-Pogorzelska, Małgorzata AU - Tomasik, Andrzej AU - Mizia-Stec, Katarzyna AU - Przybylski, Roman AU - Podolec, Piotr AU - Zieliński, Dariusz AU - Biederman, Andrzej AU - Torbicki, Adam AU - Pruszczyk, Piotr AU - Kurzyna, Marcin DO - 10.4244/EIJ-D-21-00230 PY - 2021 TI - Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension: A Multicenter Registry. T2 - EuroIntervention : journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology ER - TY - JOUR AB - Purpose: Right ventricular (RV) function is an important factor in the prognosis of chronic thromboembolic pulmonary hypertension (CTEPH) in patients. In our study, we aimed to evaluate the timing and magnitude of regional RV function before and after balloon pulmonary angioplasty (BPA) using speckle tracking echocardiography (STE) and their relation to clinical and hemodynamic parameters in patients with CTEPH. Material and Method: We enrolled 20 CTEPH patients and 19 healthy subjects in our study. Enrolled patients underwent echocardiography, right heart catheterization (RHC), and 6-minute walk distance (6MWD) test at baseline and after the BPA. Results: In hemodynamic RHC measurements and clinical evaluations, mean pulmonary artery pressure (median: 53.5 mm Hg vs 37.0 mm Hg, P =.001) and pulmonary vascular resistance (median: 12 Wood units [WU] vs 7 WU, P =.001) and pro-brain natriuretic peptide level decreased and 6MWD increased after BPA sessions. There was no statistically significant difference between before and after the BPA sessions in conventional echocardiographic measurements. In STE analysis, the electromechanical delay (EMD) between RV free wall (RVF) and LV lateral wall (LVL) (median: 65 ms vs 47.5 ms, P =.01) and RV peak systolic strain dispersion index (52 ms vs 29 ms, P =.001) were higher in patients with CTEPH than healthy controls before the BPA. Both these parameters decreased significantly after BPA. Conclusion: Chronic thromboembolic pulmonary hypertension was associated with RV electromechanical delay and dispersion based on the STE analysis. Balloon pulmonary angioplasty might have an important impact on the improvement of both RV function and hemodynamics. AU - Kanar, Batur Gonenc AU - Mutlu, Bülent AU - Atas, Halil AU - Akaslan, Dursun AU - Yıldızeli, Bedrettin DA - 2019/11// DO - 10.1111/echo.14503 IS - 11 KW - balloon pulmonary angioplasty KW - chronic thromboembolic pulmonary hypertension KW - echocardiography KW - electromechanical delay KW - strain PB - Blackwell Publishing Inc. PY - 2019 SP - 2050 EP - 2056 TI - Improvements of right ventricular function and hemodynamics after balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension T2 - Echocardiography VL - 36 ER - TY - JOUR AB - Objective: To examine the interpretation of the verbal anchors used in the Borg rating of perceived exertion (RPE) scales in different clinical groups and a healthy control group. Design: Prospective experimental study. Setting: Rehabilitation center. Participants: Nineteen subjects with brain injury, 16 with chronic low back pain (CLBP), and 20 healthy controls. Interventions: Not applicable. Main Outcome Measures: Subjects used a visual analog scale (VAS) to rate their interpretation of the verbal anchors from the Borg RPE 6-20 and the newer 10-point category ratio scale. Results: All groups placed the verbal anchors in the order that they occur on the scales. There were significant within-group differences (P>.05) between VAS scores for 4 verbal anchors in the control group, 8 in the CLBP group, and 2 in the brain injury group. There was no significant difference in rating of each verbal anchor between the groups (P>.05). Conclusions: All subjects rated the verbal anchors in the order they occur on the scales, but there was less agreement in rating of each verbal anchor among subjects in the brain injury group. Clinicians should consider the possibility of small discrepancies in the meaning of the verbal anchors to subjects, particularly those recovering from brain injury, when they evaluate exercise perceptions. © 2005 by American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation. AU - Dawes, Helen N. AU - Barker, Karen L. AU - Cockburn, Janet AU - Roach, Neil AU - Scott, Oona AU - Wade, Derick DA - 2005/5// DO - 10.1016/j.apmr.2004.10.043 IS - 5 KW - Exercise KW - Exertion KW - Perception KW - Rehabilitation KW - Semantics PY - 2005 SP - 912 EP - 916 TI - Borg's rating of perceived exertion scales: Do the verbal anchors mean the same for different clinical groups? T2 - Archives of Physical Medicine and Rehabilitation VL - 86 ER - TY - JOUR AB - Background-Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for pulmonary endarterectomy-the standard therapy. Currently, most reported results of BPA are from relatively small cohorts treated at single centers. The present study evaluated the safety and efficacy of BPA for chronic thromboembolic pulmonary hypertension based on a multicenter registry. Methods and Results-A total of 308 patients (62 men and 246 women; mean age, 61 years) underwent 1408 procedures at 7 institutions in Japan. Data were retrospectively reviewed to evaluate clinical outcome and complications. Hemodynamics were significantly improved in 249 patients in whom BPA was terminated, most often because of improvement in mean pulmonary arterial pressure or symptomatic improvement after 1154 procedures. In 196 patients who underwent followup right heart catheterization, improvement of hemodynamic parameters was maintained. Mean pulmonary arterial pressure decreased from 43.2±11.0 to 24.3±6.4 mm Hg after final BPA and 22.5±5.4 mm Hg at follow-up, with significant reduction of concomitant use of pulmonary hypertension-targeted therapy and oxygen supplementation. Complications occurred in 511 (36.3%), including pulmonary injury (17.8%), hemoptysis (14.0%), and pulmonary artery perforation (2.9%). Twelve patients (3.9%) died during follow-up, including 8 patients who died within 30 days after BPA. The leading causes of death were right heart failure, multiorgan failure, and sepsis. Overall survival was 96.8% (95% confidence interval, 93.7%-98.4%) at 1 and 2 years and 94.5% (95% confidence interval, 89.3%-97.3%) at 3 years, respectively, after the initial BPA procedure for all 308 patients. Conclusions-This multicenter registry suggested improved hemodynamic results after BPA. Complication rates were high, but overall survival was comparable with pulmonary endarterectomy. BPA may be an important therapeutic option in patients with chronic thromboembolic pulmonary hypertension. AU - Ogawa, Aiko AU - Satoh, Toru AU - Fukuda, Tetsuya AU - Sugimura, Koichiro AU - Fukumoto, Yoshihiro AU - Emoto, Noriaki AU - Yamada, Norikazu AU - Yao, Atsushi AU - Ando, Motomi AU - Ogino, Hitoshi AU - Tanabe, Nobuhiro AU - Tsujino, Ichizo AU - Hanaoka, Masayuki AU - Minatoya, Kenji AU - Ito, Hiroshi AU - Matsubara, Hiromi DO - 10.1161/CIRCOUTCOMES.117.004029 IS - 11 KW - Angioplasty KW - Hypertension KW - Lung injury KW - Pulmonary KW - Survival PY - 2017 SP - 1 EP - 7 TI - Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension results of a multicenter registry T2 - Circulation: Cardiovascular Quality and Outcomes VL - 10 ER -