ABSTRACT
PURPOSE: To evaluate the frequency of sleep-disordered breathing (SDB) and predictors of the presence of nocturnal desaturation in adults with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. METHODS: Outpatients with a hemodynamic diagnosis of precapillary pulmonary hypertension who underwent portable polysomnography were evaluated. Diagnosis and severity of SDB were assessed using three well-established respiratory disturbance index (RDI) thresholds: 5.0/h, 15.0/h, and 30.0/h, while nocturnal hypoxemia was defined by the average oxygen saturation (SpO2) < 90%. Multiple linear regression analysis evaluated the potential relationships among explanatory variables with the dependent variable (average SpO2 values), with comparisons based on the standardized regression coefficient (ß). The R-squared (R2; coefficient of determination) was used to evaluate the goodness-of-fit measure for the linear regression model. RESULTS: Thirty-six adults were evaluated (69.4% females). The majority of the participants (75.0%) had SDB (26 with obstructive sleep apnea [OSA] and one with central sleep apnea [CSA]); while 50% of them had nocturnal hypoxemia. In the linear regression model (R2 = 0.391), the mean pulmonary artery pressure [mPAP] (ß - 0.668; p = 0.030) emerged as the only independent parameter of the average SpO2. CONCLUSION: Our study found that the majority of the participants had some type of SDB with a marked predominance of OSA over CSA, while half of them had nocturnal desaturation. Neither clinical and hemodynamic parameters nor the RDI was a predictor of nocturnal desaturation, except for mPAP measured during a right heart catheterization, which emerged as the only independent and significant predictor of average SpO2.
Subject(s)
Hypertension, Pulmonary , Sleep Apnea Syndromes , Sleep Apnea, Obstructive , Adult , Female , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/etiology , Hypoxia/diagnosis , Hypoxia/epidemiology , Hypoxia/etiology , Male , Prevalence , Sleep Apnea Syndromes/diagnosis , Sleep Apnea Syndromes/epidemiology , Sleep Apnea, Obstructive/diagnosisABSTRACT
Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.
Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Brazil , Chronic Disease , Endarterectomy/adverse effects , Endarterectomy/methods , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Pulmonary Artery/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapyABSTRACT
ABSTRACT Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.
RESUMO A hipertensão pulmonar tromboembólica crônica (HPTEC) é uma doença grave e debilitante, causada pela oclusão do leito arterial pulmonar por êmbolos hemáticos e por material fibroso induzido pela presença desses êmbolos. Essa oclusão eleva a resistência vascular e, por consequência, a pressão do território arterial pulmonar, caracterizando a presença de hipertensão pulmonar. Esse aumento da carga imposta ao ventrículo direito leva a progressiva insuficiência do mesmo e, finalmente, ao óbito. No entanto, ao contrário das outras formas de hipertensão pulmonar, a HPTEC possui uma particularidade muito significativa: a existência de tratamento potencialmente curativo através da tromboendarterectomia pulmonar. Dessa forma, o objetivo primordial do manejo deve ser a avaliação do potencial cirúrgico do paciente em um centro de referência em HPTEC. Entretanto, nem todos os pacientes podem ser submetidos à cirurgia. Para esses pacientes outras alternativas terapêuticas viáveis são a angioplastia de artérias pulmonares e o tratamento farmacológico. Nestas recomendações, discutir-se-ão as bases fisiopatológicas para o surgimento de HPTEC, a partir da embolia pulmonar aguda, bem como o quadro clínico apresentado pelo paciente, o algoritmo diagnóstico a ser seguido e as alternativas terapêuticas disponíveis.
ABSTRACT
BACKGROUND: No studies have described and evaluated the association between hemodynamics, physical limitations and quality of life in patients with pulmonary hypertension (PH) without concomitant cardiovascular or respiratory disease. OBJECTIVE: To describe the hemodynamic profile, quality of life and physical capacity of patients with PH from groups I and IV and to study the association between these outcomes. METHODS: Cross-sectional study of patients with PH from clinical groups I and IV and functional classes II and III undergoing the following assessments: hemodynamics, exercise tolerance and quality of life. RESULTS: This study assessed 20 patients with a mean age of 46.8 ± 14.3 years. They had pulmonary capillary wedge pressure of 10.5 ± 3.7 mm Hg, 6-minute walk distance test (6MWDT) of 463 ± 78 m, oxygen consumption at peak exercise of 12.9 ± 4.3 mLO2.kg-1.min-1 and scores of quality of life domains < 60%. There were associations between cardiac index (CI) and ventilatory equivalent for CO2 (r=-0.59, p <0.01), IC and ventilatory equivalent for oxygen (r=-0.49, p<0.05), right atrial pressure (RAP) and 'general health perception' domain (r=-0.61, p<0.01), RAP and 6MWTD (r=-0.49, p<0.05), pulmonary vascular resistance (PVR) and 'physical functioning' domain (r=-0.56, p<0.01), PVR and 6MWTD (r=-0.49, p<0.05) and PVR index and physical capacity (r=-0.51, p<0.01). CONCLUSION: Patients with PH from groups I and IV and functional classes II and III exhibit a reduction in physical capacity and in the physical and mental components of quality of life. The hemodynamic variables CI, diastolic pulmonary arterial pressure, RAP, PVR and PVR index are associated with exercise tolerance and quality of life domains.
Subject(s)
Exercise Tolerance/physiology , Exercise/physiology , Hemodynamics/physiology , Hypertension, Pulmonary/physiopathology , Quality of Life , Adult , Blood Pressure/physiology , Cross-Sectional Studies , Exercise Test/methods , Female , Humans , Male , Middle Aged , Oxygen Consumption , Prognosis , Reference Values , Respiratory Function Tests , Statistics, Nonparametric , Surveys and Questionnaires , Time Factors , Walking/physiologyABSTRACT
Abordar as evidências mais recentes quanto às diferentes metas terapêuticas a serem atingidas na HAP e como fazer esse seguimento clínico para um maior impacto na sobrevida. Achados mais recentes: As últimas recomendações oriundas do 5o Simpósio Mundial de HP ocorrido em 2013 sugerem o alcance de metas terapêuticas a serem alcançadas para uma maior sobrevida dos pacientes. A agregação de critérios clínicos, funcionais e de avaliação da função ventricular direita torna-se de suma importância para o sucesso terapêutico. Entretanto, evidências oriundas de registros clínicos também surgiram como elementos mais proximos da realidade dos centros de referência. Resumo: Considera-se hoje como metas mais relevantes: manutenção de CF I / CF II; normalização ou quase normalização da função e dimensão do VD; PAD média < 8 mmHg e IC > 2,5 a 3,0 L/min/m2; TC6M acima de 380 a 440 metros; VO2 de pico > 15 ml/min/kg e/ou EqCO2 < 45 l/min/l/min; e normalização nos níveis de BNP. Ainda não se encontram contempladas metas psicossociais, nutricionais ou de qualidade de vida. A avaliação seriada deve ser a cada 3 a 6 meses baseando em terapia combinada sequencial preferencialmente, considerando-se a terapia combinada simultânea em casos selecionados...
Discuss the latest evidence related to therapeutic goals that need to be reached in PAH and how to make this serial assessment in order to improve survival. Most recent findings: Last recommendations from the 5th World Symposium on Pulmonary Hypertension held in 2013 suggest that therapeutic goals need to be reached in order to improve survival of the patients. The use of clinical and functional criteria associated to right ventricle function analysis has gained a greater importance to achieve therapeutic success. Nevertheless, data from clinical registries generated evidence that represent clinical practice similar to what is done at Pulmonary Hypertension centres.Summary: The most relevant therapeutic goals that need to be reached are: maintainance of WHO functional class I / II; normal or near normal right ventricle function and dimensions; RAP < 8 mmHg and CI > 2,5 a 3,0 L/min/m2; 6MWD > 380 440 m; peak VO2 > 15 ml/min/kg or EqCO2 < 45 l/min/l/min; normalization of BNP levels. There are no estabilished goals related to psychosocial, nutritional ou quality of life aspects. Serial assessment should be made each 3 to 6 months based preferentially on sequential combined therapy. Upfront combination therpy can be considered in selected cases...
Subject(s)
Humans , Male , Female , Hypertension, Pulmonary/therapy , Practice Guidelines as Topic , Survival RateABSTRACT
FUNDAMENTO: A hipertensão arterial pulmonar é uma doença grave e progressiva. O maior desafio clínico é seu diagnóstico precoce. OBJETIVO: Avaliar a presença e a extensão do realce tardio miocárdico pela ressonância magnética cardíaca bem como verificar se o percentual da massa de fibrose miocárdica é indicador de gravidade. MÉTODOS: Estudo transversal com 30 pacientes com hipertensão arterial pulmonar dos grupos I e IV, submetidos às avaliações clínica, funcional e hemodinâmica, e à ressonância magnética cardíaca. RESULTADOS: A média de idade dos pacientes foi de 52 anos, com predomínio do gênero feminino (77%). Dentre os pacientes, 53% apresentavam insuficiência ventricular direita ao diagnóstico, e 90% encontravam-se em classe funcional II/III. A média do teste de caminhada de 6 minutos foi de 395 m. No estudo hemodinâmico com o cateterismo direito, a média da pressão arterial pulmonar foi de 53,3 mmHg, do índice cardíaco de 2,1 L/min.m², e a mediana da pressão atrial direita foi de 13,5 mmHg. Realce tardio do miocárdio pela ressonância magnética cardíaca foi encontrado em 28 pacientes. A mediana da massa de fibrose foi 9,9 g e do percentual da massa de fibrose de 6,17%. A presença de classe funcional IV, insuficiência ventricular direita ao diagnóstico, teste de caminhada de 6 minutos < 300 metros e pressão atrial direita > 15 mmHg, com índice cardíaco < 2,0 L/min.m², teve associação significativa com maior percentual de fibrose miocárdica. CONCLUSÃO: O percentual da massa de fibrose miocárdica mostra-se um marcador não invasivo com perspectivas promissoras na identificação do paciente portador de hipertensão pulmonar com fatores de alto risco.
BACKGROUND: Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge. OBJECTIVE: To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor. METHODS: Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance. RESULTS: The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3mmHg, of the cardiac index of 2.1L/ min.m², and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast-enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure > 15mmHg, with cardiac index < 2.0L/ min.m², there was a relevant association with the increased percentage of myocardial fibrosis. CONCLUSION: The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Contrast Media , Hypertension, Pulmonary/diagnosis , Magnetic Resonance Imaging, Cine/methods , Ventricular Dysfunction, Right/physiopathology , Cross-Sectional Studies , Early Diagnosis , Exercise Test , Hypertension, Pulmonary/physiopathology , Risk Assessment , Risk Factors , ROC Curve , Severity of Illness Index , Statistics, Nonparametric , Time FactorsABSTRACT
BACKGROUND: Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge. OBJECTIVE: To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor. METHODS: Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance. RESULTS: The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395 m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3 mmHg, of the cardiac index of 2.1 L/min.m(2), and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast-enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure > 15 mmHg, with cardiac index < 2.0 L/min.m(2), there was a relevant association with the increased percentage of myocardial fibrosis. CONCLUSION: The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension.
Subject(s)
Contrast Media , Hypertension, Pulmonary/diagnosis , Magnetic Resonance Imaging, Cine/methods , Ventricular Dysfunction, Right/physiopathology , Adult , Aged , Cross-Sectional Studies , Early Diagnosis , Exercise Test , Familial Primary Pulmonary Hypertension , Female , Humans , Hypertension, Pulmonary/physiopathology , Male , Middle Aged , ROC Curve , Risk Assessment , Risk Factors , Severity of Illness Index , Statistics, Nonparametric , Time FactorsABSTRACT
Os autores descrevem dois casos de hipertensão arterial pulmonar secundária a defeito do septo atrial. Em seguida, apresentam uma revisão da literatura discutindo os aspectos mais importantes para o diagnóstico e para a terapêutica desta condição.
The authors describe two cases of secondary pulmonary hypertension due to atrial septal sefect. Following they present a review of literature discussing important aspects for the diagnosis and for the therapeutic of this condition.
