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OBJETIVO: Elaborar e validar um protocolo para teste de caminhada de seis minutos em esteira (TC6est) para a avaliação de pacientes com hipertensão arterial pulmonar (HAP). MÉTODOS: A população do estudo foi composta por 73 pacientes com HAP diagnosticados através de cateterismo cardíaco direito, com ou sem inalação de NO. Todos os pacientes realizaram um TC6 em solo e três TC6est baseados em um protocolo de incremento de velocidade pré-determinado e intercalados por um período de repouso. Os pacientes que haviam realizado o teste hemodinâmico com inalação de NO realizaram o terceiro TC6est com a inalação da mesma dose de NO utilizada durante o cateterismo. RESULTADOS: Os resultados mostraram uma correlação da distância caminhada no TC6est com os dados hemodinâmicos, assim como com a classe funcional e com a distância caminhada no solo. Além disso, a distância percorrida no TC6est apresentou uma correlação significativa com a sobrevida, confirmando, portanto, sua correlação com a gravidade da doença. A inalação de NO durante o TC6est levou a variações compatíveis com as variações hemodinâmicas frente à mesma dose de NO, sugerindo que o protocolo em questão pode refletir o efeito de intervenções terapêuticas. CONCLUSÕES: Concluímos que a distância percorrida no TC6est é um marcador funcional e prognóstico na avaliação de rotina de pacientes com HAP. OBJECTIVE: To develop and validate a protocol for the treadmill six-minute walk test (tread6MWT) to evaluate patients with pulmonary arterial hypertension (PAH). METHODS:The study population comprised 73 patients with PAH, diagnosed by means of right heart catheterization, with or without NO inhalation. All patients performed a hallway 6MWT and three tread6MWTs based on a pre-determined incremental speed protocol and interposed by a rest period. The patients who had been submitted to hemodynamic testing using NO performed the third tread6MWT while inhaling the same dose of NO that had been used during the catheterization. RESULTS: We found that the treadmill six-minute walk distance (tread6MWD) correlated with hemodynamic data, functional class and the hallway six-minute walk distance (6MWD). In addition, the tread6MWD correlated significantly with survival, thereby confirming the correlation with disease severity. Inhalation of NO during the tread6MWT led to variations that were consistent with the hemodynamic changes induced by the same dose of inhaled NO, suggesting that the protocol developed can reflect the effect of therapeutic interventions. CONCLUSIONS: We conclude that the tread6MWD is a useful prognostic and functional marker for the routine evaluation of PAH patients.

In recent years new therapeutic options have been developed for the management of pulmonary arterial hypertension (PAH). Sitaxsentan is an oral, once daily, highly selective endothelin A receptor antagonist that recently demonstrated a positive effect on functional capacity and haemodynamics of PAH patients. The aim of this study is to evaluate the effect of sitaxsentan in the quality of life (QOL) of PAH patients. Twenty-three patients with idiopathic PAH or PAH associated to collagen vascular diseases were evaluated at baseline and after 16 weeks of treatment with sitaxsentan 100 mg orally, once daily. 6-min walk test distance (6MWD) and QOL questionnaire (QOLQ) (SF-36) were obtained at baseline and at week 16. There was a significant improvement in functional capacity evaluated by 6MWD (472 m vs. 490 m, p = 0.03) and also in the physical component of the QOLQ (p < 0.01). Evaluating each of the domains of the SF-36 QOLQ, those more related to physical capacity presented a significant increase while the domains related to the mental component presented a trend of improvement, without reaching statistical significance. Sitaxsentan improves QOL in patients with PAH mainly through the domains related to functional capacity.

A hipertensão arterial pulmonar é caracterizada pela elevação anormal dos níveis pressóricos do território arterial pulmonar com conseqüente falência da função ventricular direita. A doença é altamente incapacitante, conforme demonstrado pela limitação nos testes de avaliação funcional, como o teste de caminhada de seis minutos. Entretanto, o impacto da doença na qualidade de vida dos pacientes não é totalmente conhecido. O objetivo desse estudo foi avaliar a qualidade de vida de pacientes com HAP através do questionário genérico SF-36, correlacionando os achados com os demais marcadores de gravidade da doença, assim como com a sobrevida. Os resultados mostraram que a QV apresenta forte correlação com TC6M e Classe Funcional e tem melhora significativa após tratamento específico. O Componente Físico do SF-36 teve significante associação com a sobrevida, permitindo concluir que a avaliação de qualidade de vida deve fazer parte da avaliação rotineira de pacientes com HAP Pulmonary arterial hypertension (PAH) is a rare but insidious disease characterized by progressive pulmonary vascular remodelling with increased pulmonary vascular resistance ultimately leading to right ventricular failure e progressive dyspnea, leading to profound functional limitations, how is demonstrated by the six minute walk test. However, there is limited information about quality of life (QoL) in patients with PAH. The aim of this study was to evaluate the quality of life of PAH patients with the SF-36 generic questionnaire and compare the scores with the surrogate markers and identify the prognostic implication of this assessment. The results showed there was a good correlation with six minute walk test and functional class and there was a significant improvement in QoL after specific treatment. The physical component summary (PCS) of the SF36 QoLQ was significantly related to survival. Conclusion is that QoL is a useful tool for routine evaluation of PAH patients once it correlates with other surrogate markers, reflects treatment effect and predicts survival

OBJETIVOS: Descrever as características clínicas dos pacientes com hipertensão pulmonar de dois centros de referência para tratamento de HP no país. MÉTODOS: Análise retrospectiva de prontuários. RESULTADOS: Foram incluídos no estudo 123 pacientes com diagnóstico de HP; 62% destes apresentavam classe funcional III e IV (NYHA). A média da pressão sistólica do ventrículo direito (PSVD) encontrada nos pacientes estudados foi de 83,48±24,61 mmHg. Não houve correlação entre a classe funcional dos pacientes e o valor da PSVD. Cerca de 50% dos pacientes tinham o diagnóstico de HAPI, 30% tinham doença associada à esquistossomose e 10% tinham HP associada a doença do tecido conectivo. O tempo de dispnéia até o diagnóstico foi variável e não houve correlação com a classe funcional dos pacientes (p>0,05). Apesar das diferentes etiologias, os pacientes apresentaram características semelhantes. CONCLUSÃO: Baseando-se em nossos achados, reforça-se a necessidade de investigação ativa para o diagnóstico definitivo antes da instituição de quaisquer alternativas terapêuticas para o tratamento da hipertensão pulmonar. Ressalta-se, ainda, a necessidade de um maior entendimento da hipertensão pulmonar associada à esquistossomose, em virtude da prevalência desse diagnóstico em nosso meio.OBJECTIVES: Describe the clinical profile of PH patients from two pulmonary hypertension centers. METHODS: Retrospective chart analysis. RESULTS: One hundred and twenty three PH patients were included in the study; 62% of these presented functional class III or IV (NYHA). Mean right ventricle systolic pressure (RVSP) was 83.48±24.61 mmHg. There was no correlation between functional class and RVSP. About 50% of the patients were diagnosed as IPAH; 30% as pulmonary hypertension associated to schistosomiasis; 10% as PH associated to connective tissue diseases. Mean time of dyspnea until diagnosis was variable with no correlation with functional class (p>0.05). No difference was found in the clinical presentation in spite of the diverse etiologies. CONCLUSION: Based upon our findings, we stressed the need for an active investigation of PH patients prior to administration of any therapeutic alternative. We emphasized that a better understanding of PH related to schistosomiasis is needed due to the high prevalence of this condition among PH patients as shown in the Brazilian population.

Background: Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. the objective of this study was to evaluate the health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment.Methods: Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a single PAH reference center were included. Patients were evaluated at baseline for clinical and hemodynamic parameters, and they subsequently received first-line therapy with either an endothelin receptor antagonist or a phosphodiesterase-5 inhibitor. After 16 weeks of specific PAH therapy, all patients were re-evaluated using a 6MWT and a SF 36 questionnaire, and then they were followed up for at least 36 months.Results: After treatment, the patients demonstrated an improved 6MWT (414 +/- 124 m vs. 440 +/- 113 m, p = 0.001). Specific PAH therapy also improved the HRQL scores.Patients with a baseline Physical Component Score (PCS) higher than 32 had a better survival rate than those who had a score under 32 (p = 0.04). Similarly, patients with a PCS of at least a 38 after the 16 week therapy period had a better survival rate when compared with those who did not achieve this value (p = 0.016). Unlike the absolute PCS values, the post-treatment PCS variability was unable to predict better survival rates (p = 0.58).Conclusions: Our findings suggest that HRQL is associated with prognosis in PAH. Furthermore, achieving pre-determined PCS scores might represent a specific goal to be reached in treatment-to-target strategies. Univ São Paulo, Sch Med, Pulm Dept, Inst Heart, BR-05403000 São Paulo, Brazil Universidade Federal de São Paulo, Rheumatol Dept, São Paulo, Brazil Universidade Federal de São Paulo, Rheumatol Dept, São Paulo, Brazil Web of Science

To develop and validate a protocol for the treadmill six-minute walk test (tread6MWT) to evaluate patients with pulmonary arterial hypertension (PAH).The study population comprised 73 patients with PAH, diagnosed by means of right heart catheterization, with or without NO inhalation. All patients performed a hallway 6MWT and three tread6MWTs based on a pre-determined incremental speed protocol and interposed by a rest period. The patients who had been submitted to hemodynamic testing using NO performed the third tread6MWT while inhaling the same dose of NO that had been used during the catheterization.We found that the treadmill six-minute walk distance (tread6MWD) correlated with hemodynamic data, functional class and the hallway six-minute walk distance (6MWD). In addition, the tread6MWD correlated significantly with survival, thereby confirming the correlation with disease severity. Inhalation of NO during the tread6MWT led to variations that were consistent with the hemodynamic changes induced by the same dose of inhaled NO, suggesting that the protocol developed can reflect the effect of therapeutic interventions.We conclude that the tread6MWD is a useful prognostic and functional marker for the routine evaluation of PAH patients.

Describe the clinical profile of PH patients from two pulmonary hypertension centers.Retrospective chart analysis.One hundred and twenty three PH patients were included in the study; 62% of these presented functional class III or IV (NYHA). Mean right ventricle systolic pressure (RVSP) was 83.48+/-24.61 mmHg. There was no correlation between functional class and RVSP. About 50% of the patients were diagnosed as IPAH; 30% as pulmonary hypertension associated to schistosomiasis; 10% as PH associated to connective tissue diseases. Mean time of dyspnea until diagnosis was variable with no correlation with functional class (p0.05). No difference was found in the clinical presentation in spite of the diverse etiologies.Based upon our findings, we stressed the need for an active investigation of PH patients prior to administration of any therapeutic alternative. We emphasized that a better understanding of PH related to schistosomiasis is needed due to the high prevalence of this condition among PH patients as shown in the Brazilian population.

OBJETIVOS: Descrever as características clínicas dos pacientes com hipertensão pulmonar de dois centros de referência para tratamento de HP no país. MÉTODOS: Análise retrospectiva de prontuários. RESULTADOS: Foram incluídos no estudo 123 pacientes com diagnóstico de HP; 62% destes apresentavam classe funcional III e IV (NYHA). A média da pressão sistólica do ventrículo direito (PSVD) encontrada nos pacientes estudados foi de 83,48±24,61 mmHg. Não houve correlação entre a classe funcional dos pacientes e o valor da PSVD. Cerca de 50% dos pacientes tinham o diagnóstico de HAPI, 30% tinham doença associada à esquistossomose e 10% tinham HP associada a doença do tecido conectivo. O tempo de dispnéia até o diagnóstico foi variável e não houve correlação com a classe funcional dos pacientes (p>0,05). Apesar das diferentes etiologias, os pacientes apresentaram características semelhantes. CONCLUSÃO: Baseando-se em nossos achados, reforça-se a necessidade de investigação ativa para o diagnóstico definitivo antes da instituição de quaisquer alternativas terapêuticas para o tratamento da hipertensão pulmonar. Ressalta-se, ainda, a necessidade de um maior entendimento da hipertensão pulmonar associada à esquistossomose, em virtude da prevalência desse diagnóstico em nosso meio. OBJECTIVES: Describe the clinical profile of PH patients from two pulmonary hypertension centers. METHODS: Retrospective chart analysis. RESULTS: One hundred and twenty three PH patients were included in the study; 62% of these presented functional class III or IV (NYHA). Mean right ventricle systolic pressure (RVSP) was 83.48±24.61 mmHg. There was no correlation between functional class and RVSP. About 50% of the patients were diagnosed as IPAH; 30% as pulmonary hypertension associated to schistosomiasis; 10% as PH associated to connective tissue diseases. Mean time of dyspnea until diagnosis was variable with no correlation with functional class (p>0.05). No difference was found in the clinical presentation in spite of the diverse etiologies. CONCLUSION: Based upon our findings, we stressed the need for an active investigation of PH patients prior to administration of any therapeutic alternative. We emphasized that a better understanding of PH related to schistosomiasis is needed due to the high prevalence of this condition among PH patients as shown in the Brazilian population.

The aim of this study was to evaluate the effect of the oral dual ET(A)/ET(B) receptor antagonist bosentan on different surrogate markers in patients with pulmonary arterial hypertension (PAH).Prospective, open label, uncontrolled study in a University Hospital in Brazil.Fifteen PAH patients (11 females) with mean age of 40 +/- 11 years (5 in WHO functional class II, 10 in class III).All patients were investigated at baseline and after 16 weeks of bosentan treatment. We used the following surrogate markers for patients' evaluation: 6-min walk test, quality of life questionnaire (Short Form SF-36) and N-terminal proBNP (B type natriuretic peptide) fraction levels in blood.Between the evaluation at baseline and week 16, the 6-min walk test distance changed from 396 +/- 135 to 434 +/- 137 m (p0.05). Each of the eight domains of the SF-36 was significantly improved. Mean NT-proBNP levels were decreased from a mean of 1670 pg/mL to 1010 pg/mL (p = 0.01).The study suggests that bosentan treatment results in the improvement of different kinds of surrogate markers independently of their specificity to reflect functional capacity, quality of life and myocardial stress. It is concluded that the combined use of these different markers may be an alternative endpoint for future short duration clinical trials.