Risk assessment validation in patients with pulmonary arterial hypertension: Data from a Southern Brazil registry (RESPHIRAR study)

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art_SPILIMBERGO_Risk_assessment_validation_in_patients_with_pulmonary_arterial_2023.PDF (1.14 MB)
Citações na Scopus
0
Tipo de produção
article
Data de publicação
2023
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
WILEY
Autores
SPILIMBERGO, Fernanda Brum
RODRIGUES, Roger Pirath
DIAS-PINTO, Marcelo Credidio
BLANCO, Daniela Cavalet
BARBIERI, Glaucia Maria
ANDRADE-LIMA, Marina
FAGUNDES, Ariovaldo Leal
GAZZANA, Marcelo Basso
RONCATO, Gabriela
MELLO, Marcelo Martins
Citação
PULMONARY CIRCULATION, v.13, n.1, article ID e12193, 10p, 2023
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Registries are a valuable tool in the research of rare conditions such as PAH. Moreover, the risk assessment strategy has been validated in European and North American registries and has been reported to provide an accurate prediction of mortality and the clinical advantage of reaching low-risk status. However, there is no available data from Brazil. Thus, the aim of the present study was to describe the characteristics of a sample of PAH from Southern Brazil and to retrospectively validate the risk assessment at our population. The RESPHIRAR is a retrospective and multicentric registry on pulmonary hypertension. With a join collaboration from nine centers in Southern Brazil, demographics, clinical presentation, and hemodynamics data of PAH were collected between 2007 and 2017. Moreover, the REVEAL 2.0 and REVEAL 2.0 Lite risk assessments were validated in our population. Overall, 370 PAH patients were included in the present study. Patients were predominantly female (78.5%) and had a mean age of 41.8 +/- 18.8 years. Most patients (33.4%) had idiopathic PAH, 30.2% had PAH associated with congenital heart disease, and 23.5% had PAH associated with connective tissue disease. The low-risk group showed significantly lower mortality than the intermediated- or high-risk group at diagnosis (p < 0.05). In conclusion, our data suggest that REVEAL 2.0 and REVEAL 2.0 Lite risk assessments can predict mortality risk in PAH patients in Southern Brazil.
Palavras-chave
pulmonary arterial hypertension, risk assessment, Southern Brazil registry
URI
https://observatorio.fm.usp.br/handle/OPI/52884
Referências
  1. Alonso-Gonzalez R, 2015, INT J CARDIOL, V184, P717, DOI 10.1016/j.ijcard.2015.02.031
  2. Alves JL, 2015, CHEST, V147, P495, DOI 10.1378/chest.14-1036
  3. Badesch DB, 2010, CHEST, V137, P376, DOI 10.1378/chest.09-1140
  4. Badlam JB, 2021, CHEST, V159, P311, DOI 10.1016/j.chest.2020.07.088
  5. Benza RL, 2021, CHEST, V159, P337, DOI 10.1016/j.chest.2020.08.2069
  6. Benza RL, 2019, CHEST, V156, P323, DOI 10.1016/j.chest.2019.02.004
  7. Benza RL, 2017, EUR RESPIR J, V50, DOI 10.1183/13993003.01353-2017
  8. Benza RL, 2015, J HEART LUNG TRANSPL, V34, P356, DOI 10.1016/j.healun.2014.09.016
  9. Benza RL, 2012, CHEST, V141, P354, DOI 10.1378/chest.11-0676
  10. Benza RL, 2010, CIRCULATION, V122, P164, DOI 10.1161/CIRCULATIONAHA.109.898122
  11. Boucly A, 2017, EUR RESPIR J, V50, DOI 10.1183/13993003.00889-2017
  12. Condliffe R, 2018, PULM CIRC, V8, DOI 10.1177/2045894018792501
  13. D'Alto M, 2012, EUR RESPIR REV, V21, P328, DOI 10.1183/09059180.00004712
  14. DALONZO GE, 1991, ANN INTERN MED, V115, P343, DOI 10.7326/0003-4819-115-5-343
  15. Deshwal H, 2021, J INVEST MED, V69, P1270, DOI 10.1136/jim-2021-002027
  16. Escribano-Subias P, 2012, EUR RESPIR J, V40, P596, DOI 10.1183/09031936.00101211
  17. Farber HW, 2015, CHEST, V148, P1043, DOI 10.1378/chest.15-0300
  18. Galie N, 2019, EUR RESPIR J, V53, DOI 10.1183/13993003.01889-2018
  19. Galie N, 2016, EUR HEART J, V37, P67, DOI 10.1093/eurheartj/ehv317
  20. Gall H, 2017, J HEART LUNG TRANSPL, V36, P957, DOI 10.1016/j.healun.2017.02.016
  21. Hachulla E, 2009, RHEUMATOLOGY, V48, P304, DOI 10.1093/rheumatology/ken488
  22. Hoeper MM, 2017, EUR RESPIR J, V50, DOI 10.1183/13993003.00740-2017
  23. Hoeper MM, 2016, LANCET RESP MED, V4, P306, DOI 10.1016/S2213-2600(15)00543-3
  24. Hoeper MM, 2014, EUR RESPIR REV, V23, P450, DOI 10.1183/09059180.00007814
  25. Hoeper MM, 2013, INT J CARDIOL, V168, P871, DOI 10.1016/j.ijcard.2012.10.026
  26. Humbert M, 2006, AM J RESP CRIT CARE, V173, P1023, DOI 10.1164/rccm.200510-1668OC
  27. Humbert M, 2010, EUR RESPIR J, V36, P549, DOI 10.1183/09031936.00057010
  28. Humbert M, 2018, REV PORT CARDIOL, V37, P759, DOI 10.1016/j.repc.2018.08.003
  29. Humbert M, 2010, CIRCULATION, V122, P156, DOI 10.1161/CIRCULATIONAHA.109.911818
  30. Hurdman J, 2012, EUR RESPIR J, V39, P945, DOI 10.1183/09031936.00078411
  31. Jing ZC, 2007, CHEST, V132, P373, DOI 10.1378/chest.06-2913
  32. Katz N., 2018, FIOCRUZ, P1
  33. Kjellstrom B, 2019, EXPERT REV CARDIOVAS, V17, P1, DOI 10.1080/14779072.2019.1548278
  34. Kylhammar D, 2018, EUR HEART J, V39, P4175, DOI 10.1093/eurheartj/ehx257
  35. Ling Y, 2012, AM J RESP CRIT CARE, V186, P790, DOI 10.1164/rccm.201203-0383OC
  36. Manes A, 2014, EUR HEART J, V35, P716, DOI 10.1093/eurheartj/eht072
  37. Maurer SJ, 2020, J CLIN MED, V9, DOI 10.3390/jcm9124071
  38. McGoon MD, 2013, J AM COLL CARDIOL, V62, pD51, DOI 10.1016/j.jacc.2013.10.023
  39. Radegran G, 2016, SCAND CARDIOVASC J, V50, P243, DOI 10.1080/14017431.2016.1185532
  40. Simonneau G, 2019, EUR RESPIR J, V53, DOI 10.1183/13993003.01913-2018
  41. Sitbon O, 2015, EUR RESPIR J, V46, P152, DOI 10.1183/09031936.00004414
  42. Swinnen K, 2019, EUR RESPIR REV, V28, DOI 10.1183/16000617.0050-2019
  43. Talavera María L, 2014, Rev. am. med. respir., V14, P144
  44. Thenappan T, 2012, CHEST, V141, P642, DOI 10.1378/chest.11-0969
  45. Thomas CA, 2020, PULM THER, V6, P9, DOI 10.1007/s41030-019-00105-5
  46. Valverde AB, 2018, BMC PULM MED, V18, DOI 10.1186/s12890-018-0667-8
  47. Vandenbroucke JP, 2007, ANN INTERN MED, V147, pW163, DOI 10.7326/0003-4819-147-8-200710160-00010-w1
  48. von Elm E, 2008, J CLIN EPIDEMIOL, V61, P344, DOI [10.1016/j.jclinepi.2007.11.008, 10.2471/BLT.07.045120]
  49. Weatherald J, 2018, AM J RESP CRIT CARE, V197, P860, DOI 10.1164/rccm.201709-1840PP

Coleções
Artigos e Materiais de Revistas Científicas - FM/MCP
Artigos e Materiais de Revistas Científicas - HC/InCor
Artigos e Materiais de Revistas Científicas - LIM/09
Artigos e Materiais de Revistas Científicas - ODS/03