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Medical treatment of pulmonary hypertension in adults with congenital heart disease. updated and extended results from the International COMPERA-CHD registry
- Source :
- Kaemmerer, A-S, Gorenflo, M, Huscher, D R, Pittrow, D, Ewert, P, Pausch, C, Delcroix, M, Ghofrani, H A, Hoeper, M M, Kozlik-Feldmann, R, Skride, A, Stähler, G, Vizza, C D, Jureviciene, E, Jancauskaite, D, Gumbiene, L, Ewert, R, Dähnert, I, Held, M, Halank, M, Skowasch, D, Klose, H, Wilkens, H, Milger, K, Jux, C, Koestenberger, M, Scelsi, L, Brunnemer, E, Hofbeck, M, Ulrich, S, Noordegraaf, A V, Lange, T J, Bruch, L, Konstantinides, S, Claussen, M, Löffler-Ragg, J, Wirtz, H, Apitz, C, Neidenbach, R, Freilinger, S, Nemes, A, Opitz, C, Grünig, E & Rosenkranz, S 2021, ' Medical treatment of pulmonary hypertension in adults with congenital heart disease : updated and extended results from the International COMPERA-CHD Registry ', Cardiovascular Diagnosis and Therapy, vol. 11, no. 6, pp. 1255-1268 . https://doi.org/10.21037/cdt-21-351, Cardiovascular Diagnosis and Therapy, 11(6), 1255-1268. AME Publishing Company, Cardiovascular diagnosis and therapy, Hong Kong : AME Publication co, 2021, vol. 11, no. 6, p. 1255-1268, Cardiovasc Diagn Ther
- Publication Year :
- 2021
- Publisher :
- AME Publishing Company, 2021.
-
Abstract
- BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. RESULTS: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. CONCLUSIONS: Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. TRIAL REGISTRATION: www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216. ispartof: CARDIOVASCULAR DIAGNOSIS AND THERAPY vol:11 issue:6 pages:1255-1268 ispartof: location:China status: published
- Subjects :
- congenital heart disease (CHD)
eisenmenger syndrome
pulmonary hypertension
registry
targeted treatment
Pediatrics
medicine.medical_specialty
Cardiac & Cardiovascular Systems
Heart disease
Eisenmenger syndrome
GUIDELINES
THERAPIES
ANTICOAGULATION
MANAGEMENT
medicine
EPIDEMIOLOGY
cardiovascular diseases
Science & Technology
Medical treatment
business.industry
Congenital heart disease (CHD)
medicine.disease
Pulmonary hypertension
ddc
PREVALENCE
Original Article on Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV
CLINICAL-ASPECTS
Cardiovascular System & Cardiology
ARTERIAL-HYPERTENSION
Cardiology and Cardiovascular Medicine
business
Life Sciences & Biomedicine
SINGLE-CENTER
Subjects
Details
- Language :
- English
- ISSN :
- 22233652 and 22233660
- Database :
- OpenAIRE
- Journal :
- Kaemmerer, A-S, Gorenflo, M, Huscher, D R, Pittrow, D, Ewert, P, Pausch, C, Delcroix, M, Ghofrani, H A, Hoeper, M M, Kozlik-Feldmann, R, Skride, A, Stähler, G, Vizza, C D, Jureviciene, E, Jancauskaite, D, Gumbiene, L, Ewert, R, Dähnert, I, Held, M, Halank, M, Skowasch, D, Klose, H, Wilkens, H, Milger, K, Jux, C, Koestenberger, M, Scelsi, L, Brunnemer, E, Hofbeck, M, Ulrich, S, Noordegraaf, A V, Lange, T J, Bruch, L, Konstantinides, S, Claussen, M, Löffler-Ragg, J, Wirtz, H, Apitz, C, Neidenbach, R, Freilinger, S, Nemes, A, Opitz, C, Grünig, E & Rosenkranz, S 2021, ' Medical treatment of pulmonary hypertension in adults with congenital heart disease : updated and extended results from the International COMPERA-CHD Registry ', Cardiovascular Diagnosis and Therapy, vol. 11, no. 6, pp. 1255-1268 . https://doi.org/10.21037/cdt-21-351, Cardiovascular Diagnosis and Therapy, 11(6), 1255-1268. AME Publishing Company, Cardiovascular diagnosis and therapy, Hong Kong : AME Publication co, 2021, vol. 11, no. 6, p. 1255-1268, Cardiovasc Diagn Ther
- Accession number :
- edsair.doi.dedup.....88baec5ab4d7af1e57b5f245217e14f6