Open AccessJournal Article
Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension
Koichiro Tatsumi,Norifumi Nakanishi,Nobuhiro Tanabe,Yasunori Kasahara,Keishi Kubo,Toyohiro Hirai,Michiaki Mishima +6 more
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TL;DR: The Respiratory Failure Research Group, with grant support from the Ministry of Health, Labour and Welfare, changed the diagnostic names of PPH and CTE-PH to "pulmonary arterial hypertension" and "chronic thromboembolic pulmonary hypertension" in keeping with recent worldwide research progress.
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Abstract: The Ministry of Health, Labour and Welfare (Japan) has approved research into primary pulmonary hypertension (PPH) and pulmonary hypertension due to chronic thromboembolic and/or embolic disease (CTE-PH) to examine their epidemiology, pathophysiology, and develop new therapeutic strategies. The Respiratory Failure Research Group, with grant support from the Ministry of Health, Labour and Welfare, changed the diagnostic names of PPH and CTE-PH. The Specific Diseases Control Division in the Health Service Bureau of the Ministry of Health, Labour and Welfare supported our proposal. One of the major purposes of The Respiratory Failure Research Group has been to maintain and, if possible, promote patient quality of life and prognosis in cases of intractable respiratory diseases. The name PPH has been changed to "pulmonary arterial hypertension (PAH)", and the name CTE-PH has been changed to "chronic thromboembolic pulmonary hypertension (CTEPH)", in keeping with recent worldwide research progress in this field. PAH should be subdivided into different pathophysiologic conditions, such as idiopathic and hereditary PAH, PAH associated with connective tissue diseases, portal hypertension, congenital heart disease, persistent pulmonary hypertension in newborn babies, pulmonary veno-occlusive disease etc. Different therapeutic strategies may be adopted for different subgroups. Pulmonary hypertension due to left heart disease, lung disease and/or hypoxia and CTEPH should be excluded from PAH. Continuous monitoring of PAH and CTEPH is required in patients with these conditions, even if the degree of pulmonary hypertension is improved by therapeutic intervention, because these diseases are incurable.
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Citations
My life with pulmonary arterial hypertension: a patient perspective
Pisana Ferrari,Hall Skåra +1 more
TL;DR: The role of patient advocacy in improving PAH care and future roles for patient associations and patient representatives in the design of clinical trials and development of new treatment guidelines are discussed.
Current trends in the management of pulmonary hypertension associated with respiratory disease in institutions approved by the Japanese Respiratory Society.
Nobuhiro Tanabe,Hiroyuki Taniguchi,Ichizo Tsujino,Fumio Sakamaki,Noriaki Emoto,Hiroshi Kimura,Katsumasa Miyaji,Kei Takamura,Shinichi Hayashi,Masayuki Hanaoka,Koichiro Tatsumi +10 more
TL;DR: Although few pulmonologists perform RHC for R-PH diagnosis in Japan, more than half consider using R HC for patients before initiating PAH-specific therapy, indicating that the current diagnostic and treatment modalities forR-PH in Japan are satisfactory.
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A retrospective observational study investigating the factors associated with right heart failure in patients with primary acute pulmonary embolism and deep vein thrombosis.
TL;DR: The relationship between the risk of right heart failure in primary acute pulmonary embolism after embolization and the residual thrombus sites in the pelvis and lower limbs is not clear.
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Screening for Pulmonary Hypertension in Systemic Sclerosis-A Primer for Cardio-Rheumatology Clinics.
Adrian Giucă,Carina Mihai,Carina Mihai,Ciprian Jurcuț,Ana Maria Gheorghiu,Laura Groșeanu,Alina Dima,Adrian Saftoiu,I M Coman,Bogdan A. Popescu,Ruxandra Jurcuț +10 more
- 01 Jun 2021
TL;DR: In this paper, the authors discuss the risk factors for systemic sclerosis associated pulmonary hypertension (SSc-PAH) and include a critical description of current detection algorithms, as a primer for clinicians working in the field of cardio-rheumatology.
Chronic thromboembolic pulmonary hypertension: the evolving treatment landscape.
Nick H. Kim,Eckhard Mayer +1 more
TL;DR: PEA is the treatment of choice for operable CTEPH and Riociguat is the first medical therapy for inoperableCTEPH.
References
Updated clinical classification of pulmonary hypertension.
Gérald Simonneau,Michael A. Gatzoulis,Michael A. Gatzoulis,Ian Adatia,Ian Adatia,David S. Celermajer,David S. Celermajer,Christopher P. Denton,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,R. Krishna Kumar,Michael J. Landzberg,Roberto F. Machado,Horst Olschewski,Ivan M. Robbins,Rogiero Souza +15 more
TL;DR: In this paper, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches, and the main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups.
4.5K
Pulmonary arterial hypertension in France: results from a national registry.
Marc Humbert,Olivier Sitbon,Ari Chaouat,Michèle Bertocchi,Gilbert Habib,Virginie Gressin,Azzedine Yaici,Emmanuel Weitzenblum,Jean-François Cordier,François Chabot,Claire Dromer,Christophe Pison,Martine Reynaud-Gaubert,Alain Haloun,Marcel Laurent,Eric Hachulla,Gérald Simonneau +16 more
TL;DR: It is shown that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise, and this contemporary registry highlights current practice.
1.9K
Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism.
Vittorio Pengo,Anthonie W. A. Lensing,Martin H. Prins,Antonio Marchiori,Bruce L. Davidson,Francesca Tiozzo,Paolo Albanese,Alessandra Biasiolo,Cinzia Pegoraro,Sabino Iliceto,Paolo Prandoni +10 more
TL;DR: D diagnostic and therapeutic strategies for the early identification and prevention of CTPH are needed and a relatively common, serious complication of pulmonary embolism.
1.9K
Treatment of Pulmonary Arterial Hypertension
TL;DR: The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in the understanding of the mechanisms mediating pulmonary arterial hypertension.
Chronic thromboembolic pulmonary hypertension.
TL;DR: The first surgical attempt to remove the adherent thrombus from the vessel wall occurred in 1958, and this operation provided the conceptual foundation for the distinction between acute and chronic thromboembolic disease of the pulmonary vascular bed.
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