Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal
Objectives: This study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center. Methods: We studied PAH and CTEPH patients diagnosed between...
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Elsevier
2018-09-01
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Series: | Revista Portuguesa de Cardiologia (English Edition) |
Online Access: | http://www.sciencedirect.com/science/article/pii/S2174204918303167 |
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DOAJ |
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English |
format |
Article |
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DOAJ |
author |
Mário Santos Ana Gomes Célia Cruz Joana Rocha Miguel Ricardo Fabienne Gonçalves Luísa Carvalho Margarida Vicente Alzira Melo Abílio Reis |
spellingShingle |
Mário Santos Ana Gomes Célia Cruz Joana Rocha Miguel Ricardo Fabienne Gonçalves Luísa Carvalho Margarida Vicente Alzira Melo Abílio Reis Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal Revista Portuguesa de Cardiologia (English Edition) |
author_facet |
Mário Santos Ana Gomes Célia Cruz Joana Rocha Miguel Ricardo Fabienne Gonçalves Luísa Carvalho Margarida Vicente Alzira Melo Abílio Reis |
author_sort |
Mário Santos |
title |
Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal |
title_short |
Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal |
title_full |
Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal |
title_fullStr |
Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal |
title_full_unstemmed |
Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal |
title_sort |
long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: insights from a referral center in portugal |
publisher |
Elsevier |
series |
Revista Portuguesa de Cardiologia (English Edition) |
issn |
2174-2049 |
publishDate |
2018-09-01 |
description |
Objectives: This study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center. Methods: We studied PAH and CTEPH patients diagnosed between January 2005 and December 2016. Cumulative survival was estimated using the Kaplan-Meier method. Survival trends were compared over two periods (2005-2010 vs. 2011-2016). Results: Of the 142 studied PH patients (age 54±18 years; 31% male), 47 had CTEPH and 95 had group 1 PH. Most patients with CTEPH and idiopathic/heritable PAH (I/HPAH) were in NYHA III-IV at diagnosis (64% and 57%, respectively). At the time of death, 31% of patients with connective tissue disease (CTD)-associated PAH (CTD-PAH) and all I/HPAH patients were on double or triple combination therapy. No patient underwent lung transplantation. Pulmonary endarterectomy or angioplasty were performed in 36% of CTEPH patients. Age at diagnosis tended to increase over time in CTD-PAH (53±15 vs. 63±15 years; p=0.13) and I/HPAH (39±15 vs. 51±19 years; p=0.10). The five-year survival estimates for I/HPAH, CTD-PAH and CTEPH patients were 80%, 52%, and 81%, respectively. Over time, CTD-PAH and CTEPH showed better five-year survival (33 vs. 67% and 77 vs. 84%), but I/HPAH did not (84 vs. 75%). Conclusions: Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis. Resumo: Introdução: Este estudo tem como objetivo avaliar a sobrevivência a longo prazo em doentes com hipertensão arterial pulmonar (PAH) e hipertensão pulmonar tromboembólica crónica (CTEPH) seguidos num centro de tratamento. Métodos: Doentes diagnosticados com PAH ou CTEPH entre janeiro 2005 e dezembro 2016 foram incluídos. A sobrevivência cumulativa foi estimada utilizando o método Kaplan-Meier. Compararam-se os padrões de sobrevivência de dois períodos (2005-2010 versus 2011-2016). Resultados: Foram estudados 142 doentes (54 ± 18 anos; 31% homens), 47 diagnosticados com CTEPH e 95 com PAH. A maioria dos doentes com CTEPH e etiologia idiopática/hereditária (I/HPAH) apresentava classe funcional NYHA III-IV ao diagnóstico (64% e 57%, respetivamente). Aquando da morte, 31% dos doentes com doença do tecido conjuntivo (CTD) e todos os doentes com H/IPAH recebiam terapia dupla ou tripla. Nenhum doente realizou transplante pulmonar. Endarterectomia pulmonar (PEA) ou angioplastia foram realizadas em 36% dos doentes com CTEPH. A idade de diagnóstico de H/IPAH (39 ± 15 versus 51 ± 19 anos; p = 0,10) e CTD (53 ± 15 versus 63 ± 15 anos; p = 0.13) tendeu a aumentar. A sobrevivência a cinco anos foi estimada em 80%, 52% e 81% para H/IPAH, CTD e CTEPH, respetivamente. No 2.° período, a sobrevivência a cinco anos melhorou nos doentes com CTD e CTEPH (33% versus 67% e 77% versus 84%), mas não nos I/HPAH (84% versus 75%). Conclusões: Existe uma tendência de melhoria na sobrevivência de doentes com CTD-PAH e CTEPH tratados num centro de referência português. O diagnóstico precoce, o uso de prostanoides parenterais e a disponibilização de tratamentos cirúrgicos poderão traduzir-se em ganhos adicionais de sobrevida. Keywords: Pulmonary hypertension, Pulmonary arterial hypertension, Survival, Chronic thromboembolic pulmonary hypertension, Palavras-chave: Hipertensão pulmonar, Hipertensão arterial pulmonar, Sobrevivência, Hipertensão pulmonar tromboembólica crónica |
url |
http://www.sciencedirect.com/science/article/pii/S2174204918303167 |
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doaj-0b8df9b928224da8b614d3ea1156689c2020-11-25T02:47:37ZengElsevierRevista Portuguesa de Cardiologia (English Edition)2174-20492018-09-01379749757Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in PortugalMário Santos0Ana Gomes1Célia Cruz2Joana Rocha3Miguel Ricardo4Fabienne Gonçalves5Luísa Carvalho6Margarida Vicente7Alzira Melo8Abílio Reis9Unidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Serviço de Cardiologia, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Unidade de Investigação Cardiovascular, Faculdade de Medicina da Universidade do Porto, Porto, Portugal; Corresponding author.Serviço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalServiço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalServiço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalServiço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalUnidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Serviço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalUnidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal; Serviço de Medicina Interna, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalDepartamento de Ciências da Saúde, Universidade de Aveiro, Aveiro, PortugalUnidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalUnidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, PortugalObjectives: This study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center. Methods: We studied PAH and CTEPH patients diagnosed between January 2005 and December 2016. Cumulative survival was estimated using the Kaplan-Meier method. Survival trends were compared over two periods (2005-2010 vs. 2011-2016). Results: Of the 142 studied PH patients (age 54±18 years; 31% male), 47 had CTEPH and 95 had group 1 PH. Most patients with CTEPH and idiopathic/heritable PAH (I/HPAH) were in NYHA III-IV at diagnosis (64% and 57%, respectively). At the time of death, 31% of patients with connective tissue disease (CTD)-associated PAH (CTD-PAH) and all I/HPAH patients were on double or triple combination therapy. No patient underwent lung transplantation. Pulmonary endarterectomy or angioplasty were performed in 36% of CTEPH patients. Age at diagnosis tended to increase over time in CTD-PAH (53±15 vs. 63±15 years; p=0.13) and I/HPAH (39±15 vs. 51±19 years; p=0.10). The five-year survival estimates for I/HPAH, CTD-PAH and CTEPH patients were 80%, 52%, and 81%, respectively. Over time, CTD-PAH and CTEPH showed better five-year survival (33 vs. 67% and 77 vs. 84%), but I/HPAH did not (84 vs. 75%). Conclusions: Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis. Resumo: Introdução: Este estudo tem como objetivo avaliar a sobrevivência a longo prazo em doentes com hipertensão arterial pulmonar (PAH) e hipertensão pulmonar tromboembólica crónica (CTEPH) seguidos num centro de tratamento. Métodos: Doentes diagnosticados com PAH ou CTEPH entre janeiro 2005 e dezembro 2016 foram incluídos. A sobrevivência cumulativa foi estimada utilizando o método Kaplan-Meier. Compararam-se os padrões de sobrevivência de dois períodos (2005-2010 versus 2011-2016). Resultados: Foram estudados 142 doentes (54 ± 18 anos; 31% homens), 47 diagnosticados com CTEPH e 95 com PAH. A maioria dos doentes com CTEPH e etiologia idiopática/hereditária (I/HPAH) apresentava classe funcional NYHA III-IV ao diagnóstico (64% e 57%, respetivamente). Aquando da morte, 31% dos doentes com doença do tecido conjuntivo (CTD) e todos os doentes com H/IPAH recebiam terapia dupla ou tripla. Nenhum doente realizou transplante pulmonar. Endarterectomia pulmonar (PEA) ou angioplastia foram realizadas em 36% dos doentes com CTEPH. A idade de diagnóstico de H/IPAH (39 ± 15 versus 51 ± 19 anos; p = 0,10) e CTD (53 ± 15 versus 63 ± 15 anos; p = 0.13) tendeu a aumentar. A sobrevivência a cinco anos foi estimada em 80%, 52% e 81% para H/IPAH, CTD e CTEPH, respetivamente. No 2.° período, a sobrevivência a cinco anos melhorou nos doentes com CTD e CTEPH (33% versus 67% e 77% versus 84%), mas não nos I/HPAH (84% versus 75%). Conclusões: Existe uma tendência de melhoria na sobrevivência de doentes com CTD-PAH e CTEPH tratados num centro de referência português. O diagnóstico precoce, o uso de prostanoides parenterais e a disponibilização de tratamentos cirúrgicos poderão traduzir-se em ganhos adicionais de sobrevida. Keywords: Pulmonary hypertension, Pulmonary arterial hypertension, Survival, Chronic thromboembolic pulmonary hypertension, Palavras-chave: Hipertensão pulmonar, Hipertensão arterial pulmonar, Sobrevivência, Hipertensão pulmonar tromboembólica crónicahttp://www.sciencedirect.com/science/article/pii/S2174204918303167 |