Skeletal muscle abnormalities in pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension is a progressive disease that is characterized by dyspnea and exercise intolerance. Impairment in skeletal muscle has recently been described in PAH, although the degree to which this impairment is solely determined by the hemodynamic profile remains uncertain. The aim of this study was to verify the association of structural and functional skeletal muscle characteristics with maximum exercise in PAH. METHODS: The exercise capacity, body composition, CT area of limb muscle, quality of life, quadriceps biopsy and hemodynamics of 16 PAH patients were compared with those of 10 controls. RESULTS: PAH patients had a significantly poorer quality of life, reduced percentage of lean body mass, reduced respiratory muscle strength, reduced resistance and strength of quadriceps and increased functional limitation at 6MWT and CPET. VO2 max was correlated with muscular variables and cardiac output. Bivariate linear regression models showed that the association between muscular structural and functional variables remained significant even after correcting for cardiac output. CONCLUSION: Our study showed the coexistence of ventilatory and quadriceps weakness in face of exercise intolerance in the same group of PAH patients. More interestingly, it is the first time that the independent association between muscular pattern and maximum exercise capacity is evidenced in PAH, independently of cardiac index highlighting the importance of considering rehabilitation in the treatment strategy for PAH.

Quality of life as a prognostic marker in pulmonary arterial hypertension.

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.

Evaluating compliance to a cardiac rehabilitation program in a private general hospital.

OBJECTIVE: Identify the primary factors that influenced the participant in our cardiovascular rehabilitation program towards missing their therapy sessions, and to correlate those factors with age, cardiovascular risk, and motivation of our population. METHODS: We conducted a retrospective study with 42 patients (69.15±13.93 years) participating in the cardiac rehabilitation program at a general hospital in São Paulo, through the analysis of two scales applied during the initial evaluation: Cardiac Rehabilitation Barriers Scale and scale of the original provision. We used Spearman correlation to relate them to absenteeism, cardiovascular risk age and duration of cardiac rehabilitation. RESULTS: The total score of barriers was 31±6 and the mean score of 1.47±0.31. The main barriers subscales were related to "travel/labor dispute" and "personal problems/family." The percentage of absenteeism was 8.4% in the number of sessions that could be made in the month. The faults and cancellations were positively correlated with cardiovascular risk (p=0.01; r=0.4) and negatively with scale provision of baseline (p=0.03; r=-0.35) and age (p=0.02; r=-0.35). CONCLUSION: "Travel/labor dispute", "personal/ family problems", and low initial provision are the main factors absenteism in a cardiac rehabilitation program in a general hospital in São Paulo.

Avaliação da aderência ao programa de reabilitação cardíaca em um hospital particular geral / Evaluating compliance to a cardiac rehabilitation program in a private general hospital

OBJETIVO: Identificar os principais fatores que influenciaram o participante do nosso programa de reabilitação cardiovascular a faltar em suas terapias e correlacioná-los a idade, risco cardiovascular e motivação de nossa população. MÉTODOS: Realizou-se um estudo retrospectivo transversal com 42 pacientes (69,15±13,93 anos) participantes do programa de reabilitação cardíaca de um hospital geral particular de São Paulo, por meio da análise de duas escalas: a Escala de Barreiras para Reabilitação Cardíaca e a Escala de Disposição. Utilizou-se a correlação de Spearman para relacioná-las com absenteísmo, risco cardiovascular, idade e tempo de reabilitação cardíaca. RESULTADOS: A pontuação total da escala de barreiras foi 31±6 e o escore médio foi de 1,47±0,31. As principais barreiras foram relacionadas às subescalas "viagem/conflito de trabalho" e "problemas pessoais/familiares". A porcentagem de absenteísmo foi 8,4% do número de sessões possíveis de serem realizadas no mês. As faltas e os cancelamentos apresentaram correlação positiva com o risco cardiovascular (p=0,01; r=0,4) e correlações negativas com Escala de Disposição inicial (p=0,03; r=-0,35) e idade (p=0,02; r=-0,35). CONCLUSÃO: "Viagem/conflito de trabalho", "problemas pessoais/familiares" e baixa disposição inicial foram os principais fatores de absenteísmo em um programa de reabilitação cardíaca de um hospital geral particular de São Paulo.

OBJECTIVE: Identify the primary factors that influenced the participant in our cardiovascular rehabilitation program towards missing their therapy sessions, and to correlate those factors with age, cardiovascular risk, and motivation of our population. METHODS: We conducted a retrospective study with 42 patients (69.15±13.93 years) participating in the cardiac rehabilitation program at a general hospital in São Paulo, through the analysis of two scales applied during the initial evaluation: Cardiac Rehabilitation Barriers Scale and scale of the original provision. We used Spearman correlation to relate them to absenteeism, cardiovascular risk age and duration of cardiac rehabilitation. RESULTS: The total score of barriers was 31±6 and the mean score of 1.47±0.31. The main barriers subscales were related to "travel/labor dispute" and "personal problems/family." The percentage of absenteeism was 8.4% in the number of sessions that could be made in the month. The faults and cancellations were positively correlated with cardiovascular risk (p=0.01; r=0.4) and negatively with scale provision of baseline (p=0.03; r=-0.35) and age (p=0.02; r=-0.35). CONCLUSION: "Travel/labor dispute", "personal/ family problems", and low initial provision are the main factors absenteism in a cardiac rehabilitation program in a general hospital in São Paulo.

Avaliação da musculatura estriada de membros inferiores na limitação funcional ao exercício em pacientes com hipertensão arterial pulmonar / Assessment of skeletal muscle of lower limb in functional exercise limitation in patients with pulmonary arterial hypertension

Introdução: A hipertensão arterial pulmonar (HAP) é uma doença progressiva extremamente grave, que evolui com insuficiência cardíaca direita e morte. Apesar do avanço do tratamento farmacológico, o prognóstico permanece reservado com taxa de sobrevida de 86%, 70% e 55% em 1, 3 e 5 anos, respectivamente. A dispnéia progressiva e a intolerância ao exercício são as principais manifestações clínicas e refletem a falência do ventrículo direito. O músculo esquelético periférico parece ser também um dos principais determinantes desta limitação funcional, visto que a redução da oferta de oxigênio e alterações na extração/utilização do oxigênio pelo músculo são diretamente relacionados com a tolerância ao exercício. Existem dois mecanismos potencialmente envolvidos na regulação da oferta de oxigênio, e portanto, na capacidade de exercício: mecanismos centrais (função do coração, pulmão e sistema nervoso autônomo) e mecanismos periféricos (associado ao fluxo sanguíneo periférico e a função do músculo esquelético). Os pacientes com HAP geralmente apresentam baixo débito cardíaco e estado adrenérgico exacerbado. A combinação destas alterações pode resultar em alterações estruturais e funcionais da musculatura estriada periférica. Porém, não existem informações sólidas que nos esclareçam se o acometimento muscular é preditor independente da limitação da capacidade de exercício. Objetivos: (1) Caracterizar o papel da musculatura periférica na limitação funcional em pacientes com HAP. (2) Avaliar o papel do sistema muscular periférico como um fator independente para a limitação ao exercício em HAP. Materiais e métodos: Dezesseis pacientes com HAP foram prospectivamente comparados com 10 indivíduos controle em termos de dados demográficos, qualidade de vida relacionada à saúde e limitação ao exercício, avaliada pelo teste de caminhada de seis minutos, teste cardiopulmonar, dinamometria isocinética e medições de pressão respiratória máxima. Pacientes com HAP também foram...

Introduction: Pulmonary arterial hypertension (PAH) is a relentlessly progressive disease that leads to right heart failure and death. Despite advances in pharmacological treatment, prognosis is still poor with survival rates of 86%, 70% and 55% at 1, 3 and 5 years, respectively. Progressive dyspnea and exercise intolerance are the main clinical manifestations and reflect the impairment of right ventricular function. Peripheral skeletal muscle also seems to be a major determinant of functional limitation, as the reduction of oxygen supply and changes in extraction and utilization of oxygen by the muscle are directly associated to exercise tolerance. There are two potential mechanisms involved in the regulation of oxygen supply and therefore in exercise capacity: central (as a function of heart, lung and autonomic nervous system function) and peripheral (associated to peripheral blood flow and skeletal muscle function). Patients with PAH usually present low cardiac output and exacerbated adrenergic state. The combination of these features might result in changes of peripheral skeletal muscle and structure. However, there is no robust information that clearly clarifies whether the muscle involvement is an independent factor for exercise limitation. Objectives: (1) Characterize the role of the peripheral muscles in functional limitation in patients with PAH. (2) Address the role of the peripheral muscle system as an independent factor in exercise limitation in PAH. Materials and methods: Sixteen PAH patients were prospectively compared to 10 control individuals in terms of demographic data, health related quality of life and exercise limitation, assessed by six-minute walk test, cardiopulmonary test, isokinetic dynamometry and maximum respiratory pressure measurements. PAH patients also were submitted to vastus lateralis biopsy in order to assess structural changes. Results: PAH patients presented poorer quality of life (p <0.001), lower percentage of fat...

Famílias monoparentais: os padrões de interação de uma família singular com pai viúvo

Esta pesquisa se propõe a compreender a dinâmica do funcionamento de uma família monoparental, composta por um pai singular viúvo e seus dois filhos. O enfoque deste estudo está nos padrões interacionais, papéis sexuais e na construção das relações dos sujeitos envolvidos. O fenômeno foi estudado através de Estudo de Caso e também do Genograma e Linha Familiar do Tempo. Foram realizadas duas entrevistas: uma com o pai e outra com os subsistema fraterno. Os resultados sugerem que se trata de uma família em que o papel de nutridor é assumido pelo pai, enquanto o normativo era assumido pela mãe. Foi constatada uma coalisão do pai com os dois filhos. Observou se a não existência de fronteiras entre os subsistemas (parental fraterno), não havia nível hierárquico nítido entre pais e filhos. Constatou se um conflito intergeracional por parte do pai com relação à autoridade, juntamente com uma repetição de padrões interacionais oriundos da família de origem paterna. Por outro lado, pode se observar a construção de vínculos e papéis não estereotipados dentro da família monoparental, ou seja, que não responda a estrutura de funcionamento do modelo clássico nuclear (AU)