Hipertensão arterial pulmonar – Experiência do Centro Hospitalar de Vila Nova de Gaia

Pulmonary arterial hypertension – Experience of Centro Hospitalar de Vila Nova de Gaia

 

Ana Oliveira¹

Daniela Ferreira¹

António Caiado²

Susana Ferreira¹

Paula Ferreira¹

Lino Santos³

Manuel Gonçalves⁴

Teresa Shiang⁵

 

Resumo

Introdução: A hipertensão arterial pulmonar (HAP) é uma doença grave e progressiva potencialmente fatal em poucos anos. Até à década de 90, o único tratamento eficaz para esta entidade era o transplante pulmonar/cardiopulmonar. Nos últimos anos assistiu-se ao aparecimento de novos fármacos que vieram alterar o curso da doença.

Objectivos: Caracterização dos doentes com HAP observados no Centro Hospitalar de VN Gaia no período de 2000 a 2006. Alertar para esta entidade.

Material e métodos: Análise retrospectiva de todos os doentes com HAP ou doença tromboembólica crónica em tratamento no CHVN Gaia.

Resultados: No referido período foram observados 11 doentes, 7 mulheres e 4 homens, com média etária de 43 anos. Cinco doentes com HAP idiopática, três com doença tromboembólica crónica, dois com HAP associada a comunicação interauricular e um com HAP associada a S. CREST. Ao diagnóstico, a maioria encontrava-se em classe funcional NYHA/OMS III/IV, a pressão sistólica média da artéria pulmonar foi de 98±32mmHg e a distância média percorrida na 6-MWT foi de 401m. Terapêutica inicial: Três doentes iloprost por via inalatória; dois treprostinil subcutâneo; três bosentan per os; um sildenafil per os e dois doentes foram submetidos a cirurgia. O tempo médio de follow-up é de 28,1±20.3 meses. Cinco doentes melhoraram, três mantiveram-se estáveis, um agravou e dois faleceram.

Conclusão: A HAP é uma doença grave que requer uma abordagem multidisciplinar com necessidade de terapêuticas complexas e dispendiosas. O diagnóstico em estádios precoces é essencial, de modo a permitir uma terapêutica atempada.

 

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a serious and progressive disease, potentially fatal in a few years. Until the 1990s the only effective treatment for this disease was pulmonary/cardiopulmonary transplant. Over the last few years several drugs have emerged that have modified the course of the disease.

Aims: To characterise patients with PAH followed at Centro Hospitalar de V.N.Gaia between 2000 and 2006. To raise awareness of this entity.

Material and methods: Retrospective analysis of all patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH) followed at our Hospital.

Results: In this period 11 patients were observed, 7 women and 4 men, with a mean age of 43 y. Five patients had idiopathic PAH, three had CTEPH, two had PAH associated with interauricular communication and one had PAH associated with CREST Syndrome. At the time of diagnosis most patients were in NYHA//WHO Functional Class III/IV, mean systolic pulmonary artery pressure was 98±32mmHg and mean distance walked in the 6-minute walk test was 401m. Initial treatment: Three patients had inhaled iloprost, two subcutaneous treprostinil, three oral bosentan, one oral sildenafil and two patients underwent surgery. Mean follow-up time was 28.1 months. Five patients recovered, three are stable, one worsened and two died.

Conclusions: PAH is a serious illness which requires a multidisciplinary approach with the need for complex and expensive therapies. Diagnosis in initial stages is essential for an effective treatment.

 

Texto completo disponível apenas em PDF.

Full text only available in PDF format.

 

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¹ Interna Complementar de Pneumologia / Pulmonolgy resident

² Assistente Hospitalar de Pneumologia / Pulmonolgy consultant

³ Assistente Hospitalar de Cardiologia / Cardiology consultant

⁴ Assistente Hospitalar Graduado de Medicina Interna / Internal Medicine specialist consultant

⁵ Assistente Hospitalar Graduada de Pneumologia / Pulmonolgy specialist consultant

 

Serviço de Pneumologia. Directora: Dra. Bárbara Parente

Serviço de Medicina Interna. Director: Dr. Rosas Vieira

Serviço de Cardiologia. Director: Dr. Vasco Gama Ribeiro

 

Centro Hospitalar de Vila Nova de Gaia

Rua Conceição Fernandes

4430 Vila Nova de Gaia

 

Recebido para publicação/received for publication: 06.10.04

Aceite para publicação/accepted for publication: 07.01.02