Changes in the Characteristics and Initial Treatments of Pulmonary Hypertension Between 2008 and 2020 in Japan

Overview

Journal JACC Asia

Date 2022 Nov 7

PMID 36338395

Authors

Yuichi Tamura

Hiraku Kumamaru

Takumi Inami

Hiromi Matsubara

Ken-Ichi Hirata

Ichizo Tsujino

Rika Suda

Hiroaki Miyata

Shiori Nishimura

Byron Sigel

Masashi Takano

Koichiro Tatsumi

Affiliations

Soon will be listed here.

Abstract

Background: Pulmonary arterial hypertension (PAH) is a rare, progressive disease. The treatment landscape for PAH in Japan has evolved considerably in recent years, but there is limited knowledge of the changes in treatment practices or patient characteristics.

Objectives: The aim of this study was to evaluate the changes in characteristics and initial treatments for PAH in Japan over time.

Methods: This study used data from the Japan Pulmonary Hypertension Registry (JAPHR) to compare patient characteristics and treatment practices between 2008-2015 (n = 316) and 2016-2020 (n = 315).

Results: The mean ± standard deviation age at diagnosis increased from 47.9 ± 16.7 years in 2008-2015 to 52.7 ± 16.9 years in 2016-2020. The mean pulmonary arterial pressure decreased from 45.4 ± 15.0 to 38.6 ± 13.1 mm Hg. Idiopathic/hereditary PAH was the most common etiology in both periods (50.0% and 51.1%, respectively). The proportion of patients prescribed oral/inhaled combination therapies increased from 47.8% to 57.5%. Oral/inhaled combination therapies were frequently prescribed to patients with congenital heart disease-related PAH (81.8%). There was no significant trend in prescribing practices based on French low-risk criteria: among patients with 0, 1, 2, 3, or 4 criteria, 53.8%, 68.8%, 52.8%, 66.7%, and 39.4% were prescribed oral/inhaled combination therapies, and 0%, 16.7%, 27.0%, 17.3%, and 15.2% were prescribed oral/inhaled monotherapies. Macitentan, tadalafil, selexipag, and epoprostenol were the most frequently prescribed drugs.

Conclusions: The severity of PAH decreased over time in Japan. Oral/inhaled combination therapies were generally preferred. Physicians generally prescribed therapies after considering the patients' hemodynamics and clinical severity. (Japan Pulmonary Hypertension Registry [JAPHR]; UMIN000026680).

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References

Sitbon O, Cottin V, Canuet M, Clerson P, Gressin V, Perchenet L . Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension. Eur Respir J. 2020; 56(3). PMC: 7469971. DOI: 10.1183/13993003.00673-2020. View

Kaemmerer H, Gorenflo M, Huscher D, Pittrow D, Apitz C, Baumgartner H . Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry. J Clin Med. 2020; 9(5). PMC: 7290703. DOI: 10.3390/jcm9051456. View

Almaaitah S, Highland K, Tonelli A . Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis. Integr Blood Press Control. 2020; 13:15-29. PMC: 7125406. DOI: 10.2147/IBPC.S232038. View

Coghlan J, Galie N, Barbera J, Frost A, Ghofrani H, Hoeper M . Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis. 2017; 76(7):1219-1227. PMC: 5530350. DOI: 10.1136/annrheumdis-2016-210236. View

Fukuda K, Date H, Doi S, Fukumoto Y, Fukushima N, Hatano M . Guidelines for the Treatment of Pulmonary Hypertension (JCS 2017/JPCPHS 2017). Circ J. 2019; 83(4):842-945. DOI: 10.1253/circj.CJ-66-0158. View

Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V . Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006; 173(9):1023-30. DOI: 10.1164/rccm.200510-1668OC. View

Frost A, Badesch D, Barst R, Benza R, Elliott C, Farber H . The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest. 2010; 139(1):128-37. DOI: 10.1378/chest.10-0075. View

DAlonzo G, Barst R, Ayres S, Bergofsky E, Brundage B, Detre K . Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991; 115(5):343-9. DOI: 10.7326/0003-4819-115-5-343. View

Benza R, Miller D, Gomberg-Maitland M, Frantz R, Foreman A, Coffey C . Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010; 122(2):164-72. DOI: 10.1161/CIRCULATIONAHA.109.898122. View

10.

Foderaro A, Ventetuolo C . Pulmonary Arterial Hypertension and the Sex Hormone Paradox. Curr Hypertens Rep. 2016; 18(11):84. DOI: 10.1007/s11906-016-0689-7. View

11.

Farber H, Miller D, Poms A, Badesch D, Frost A, Muros-Le Rouzic E . Five-Year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015; 148(4):1043-54. DOI: 10.1378/chest.15-0300. View

12.

Bai Y, Sun L, Hu S, Wei Y . Combination therapy in pulmonary arterial hypertension: a meta-analysis. Cardiology. 2012; 120(3):157-65. DOI: 10.1159/000334431. View

13.

Hoeper M, Humbert M, Souza R, Idrees M, Kawut S, Sliwa-Hahnle K . A global view of pulmonary hypertension. Lancet Respir Med. 2016; 4(4):306-22. DOI: 10.1016/S2213-2600(15)00543-3. View

14.

Kozu K, Sugimura K, Ito M, Hirata K, Node K, Miyamoto T . Current status of long-term prognosis among all subtypes of pulmonary hypertension in Japan. Int J Cardiol. 2019; 300:228-235. DOI: 10.1016/j.ijcard.2019.11.139. View

15.

Song S, Lee S, Oh S, Jeon S, Sung J, Park J . Demographics, treatment trends, and survival rate in incident pulmonary artery hypertension in Korea: A nationwide study based on the health insurance review and assessment service database. PLoS One. 2018; 13(12):e0209148. PMC: 6300275. DOI: 10.1371/journal.pone.0209148. View

16.

Boucly A, Weatherald J, Savale L, Jais X, Cottin V, Prevot G . Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017; 50(2). DOI: 10.1183/13993003.00889-2017. View

17.

Benza R, Miller D, Barst R, Badesch D, Frost A, McGoon M . An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012; 142(2):448-456. DOI: 10.1378/chest.11-1460. View

18.

van Riel A, Schuuring M, van Hessen I, van Dijk A, Hoendermis E, Yip J . Treatment of pulmonary arterial hypertension in congenital heart disease in Singapore versus the Netherlands: age exceeds ethnicity in influencing clinical outcome. Neth Heart J. 2016; 24(6):410-416. PMC: 4887299. DOI: 10.1007/s12471-016-0820-z. View

19.

Peacock A, Murphy N, McMurray J, Caballero L, Stewart S . An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007; 30(1):104-9. DOI: 10.1183/09031936.00092306. View

20.

Galie N, Simonneau G . The Fifth World Symposium on Pulmonary Hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl):D1-3. DOI: 10.1016/j.jacc.2013.10.030. View