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OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH). METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH-in the absence of severe left ventricular dysfunction (LVD), significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism-between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH) was confirmed in 302 patients (78.6%). The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3%) and 178 (81.7%) were diagnosed with PH associated with LVD (PH-LVD) and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001). CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant. .

OBJETIVO: Avaliar o papel do cateterismo de câmaras cardíacas direitas no diagnóstico de hipertensão arterial pulmonar (HAP). MÉTODOS: Entre 2008 e 2013, foram avaliadas as características clínicas, funcionais e hemodinâmicas de todos os pacientes que realizaram cateterismo cardíaco direito por suspeita de HAP em nosso laboratório, depois de afastada a presença de disfunção ventricular esquerda (DVE) grave, de alterações significativas nos testes de função pulmonar ou de resultados de cintilografia pulmonar de inalação/perfusão compatíveis com tromboembolismo pulmonar crônico. RESULTADOS: Durante o período de estudo, 384 pacientes foram submetidos a cateterismo cardíaco diagnóstico. A hipertensão pulmonar (HP) foi confirmada em 302 pacientes (78,6%). A média de idade desses pacientes foi de 48,7 anos. Os pacientes sem HP apresentaram melhor perfil hemodinâmico e menores níveis de peptídio natriurético do tipo B que aqueles diagnosticados com HP. No entanto, 13,8% dos pacientes sem HP apresentavam-se em classe funcional III/IV do New York Heart Association. Dos 218 pacientes que cumpriam os critérios de inclusão, 40 (18,3%) e 178 (81,7%) foram diagnosticados como portadores de HP associada à DVE (HP-DVE) e HAP, respectivamente. O grupo HP-DVE tinha idade significativamente mais avançada que aqueles com HAP (p < 0,0001). CONCLUSÕES: A diferença proporcional entre os grupos HAP e HP-DVE foi bastante significativa, considerando a inexistência de sinais ecocardiográficos sugestivos de DVE importante como parte da investigação que antecedeu ...

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OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH). METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH--in the absence of severe left ventricular dysfunction (LVD), significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism--between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH) was confirmed in 302 patients (78.6%). The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3%) and 178 (81.7%) were diagnosed with PH associated with LVD (PH-LVD) and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001). CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant.

OBJETIVO: Avaliar o papel do cateterismo de câmaras cardíacas direitas no diagnóstico de hipertensão arterial pulmonar (HAP). MÉTODOS: Entre 2008 e 2013, foram avaliadas as características clínicas, funcionais e hemodinâmicas de todos os pacientes que realizaram cateterismo cardíaco direito por suspeita de HAP em nosso laboratório, depois de afastada a presença de disfunção ventricular esquerda (DVE) grave, de alterações significativas nos testes de função pulmonar ou de resultados de cintilografia pulmonar de inalação/perfusão compatíveis com tromboembolismo pulmonar crônico. RESULTADOS: Durante o período de estudo, 384 pacientes foram submetidos a cateterismo cardíaco diagnóstico. A hipertensão pulmonar (HP) foi confirmada em 302 pacientes (78,6%). A média de idade desses pacientes foi de 48,7 anos. Os pacientes sem HP apresentaram melhor perfil hemodinâmico e menores níveis de peptídio natriurético do tipo B que aqueles diagnosticados com HP. No entanto, 13,8% dos pacientes sem HP apresentavam-se em classe funcional III/IV do New York Heart Association. Dos 218 pacientes que cumpriam os critérios de inclusão, 40 (18,3%) e 178 (81,7%) foram diagnosticados como portadores de HP associada à DVE (HP-DVE) e HAP, respectivamente. O grupo HP-DVE tinha idade significativamente mais avançada que aqueles com HAP (p < 0,0001). CONCLUSÕES: A diferença proporcional entre os grupos HAP e HP-DVE foi bastante significativa, considerando a inexistência de sinais ecocardiográficos sugestivos de DVE importante como parte da investigação que antecedeu o cateterismo. Nossos resultados reforçam o papel fundamental do cateterismo cardíaco no diagnóstico da HAP, ainda mais em faixas etárias mais avançadas, nas quais a prevalência de DVE não diagnosticada através de exames não invasivos é particularmente importante.

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O entendimento adequado da resposta cardiovascular ao exercício é fundamental para o manejo e tratamento dos pacientes com hipertensão arterial pulmonar (HAP). Entretanto, dados a respeito da adaptação da mecânica vascular pulmonar e da adaptação do ventrículo direito ao esforço são escassos na literatura. O objetivo desse estudo é caracterizar a resposta cardiovascular ao exercício nos pacientes nesse grupo de pacientes. Foram selecionados 30 pacientes com HAP (Grupo 1 de Dana Point) e 7 controles com hemodinâmica invasiva normal, entre dezembro de 2009 e novembro de 2010, que realizaram protocolo incremental de esforço em cicloergômetro limitado por sintomas. Foram avaliados o comportamento das variáveis hemodinâmicas, da mecânica vascular pulmonar e o comportamento do peptídeo natriurético tipo B durante o esforço nos pacientes e comparado sua resposta com os controles. O grupo HAP apresentou elevação da PAPm superior ao grupo controle, com elevação da Poap e FC semelhantes. A elevação do débito cardíaco no pico do esforço foi inferior nesse grupo, sem variação do volume sistólico. A complacência e resistência vascular pulmonar apresentam queda no grupo HAP, permanecendo com complacência inferior e resistência superior no pico do esforço em relação aos controles. Estas variáveis permanecem inversamente acopladas sob a função C = t / R em repouso e durante todos os passos do esforço, reforçando o poder desse acoplamento e o papel na CVP na avaliação desses pacientes. No entanto, o comportamento da resistência não é uniforme em todos os pacientes com HAP, podendo ser separada em 3 grupos distintos: elevação, manutenção e queda durante o esforço. O BNP apresenta elevação durante o esforço no grupo HAP, mas os valores de BNP basal apresentam correlação inversa superior com o DC de pico do que os valores de BNP no pico do esforço. Na avaliação por curva ROC, o BNP de pico e a variação de BNP pico basal não se mostraram superiores ao BNP basal na...

The proper understanding of the cardiovascular response to exercise has a central role in the management and treatment of patients with pulmonary arterial hypertension (PAH). However, data concerning the evolution of pulmonary vascular mechanics and right ventricular adaptation to effort are scarce. The aim of this study is characterize the cardiovascular response to exercise in these patients. We selected 30 patients with PAH (Group 1 Dana Point) and 7 controls with normal invasive hemodynamics between December 2009 and November 2010 who underwent a symptom-limited exercise protocol on cycle ergometer. Were evaluated the behavior of hemodynamic parameters, pulmonary vascular mechanics and B-type natriuretic peptide during stress in patients and compared their response with controls. The PAH group showed elevation of mPAP greater than the controls, with the same elevated PCWP and HR response between the groups. Cardiac output at peak exercise was lower in the PAH patients with no change of stroke volume during effort. Compliance and pulmonary vascular resistance have decrease in PAH group, remaining with lower compliance and higher resistance at peak exercise compared to controls. These variables are inversely related at rest and during every step of the effort, strengthening the power of this relation and the role of CVP in evaluation of these patients. However, the behavior of resistance is not uniform in all PAH patients and may be separated into three distinct groups: rise, fall and maintenance during exercise. The BNP raises during exercise in PAH group, but the values of baseline BNP already has an stronger inverse correlation with peak CO, better than the correlation of peak exercise BNP with the same variable. BNP at peak exercise and the variation of BNP peak - baseline were not superior to baseline BNP in discriminating the response of the CO during exercise, when evaluated by ROC curves. These findings reinforce the role of baseline BNP in...

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OBJECTIVES: The objective of this study was to evaluate the natural history of untreated schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients as compared to idiopathic pulmonary arterial hypertension (IPAH) with respect to hemodynamics recorded at presentation and 36 months survival. BACKGROUND: Schistosomiasis (Sch) is one of the most prevalent chronic infectious diseases in the world. Nevertheless data regarding one of its most severe clinical complications, pulmonary arterial hypertension (PAH), is scarce. METHODS: We retrospectively analyzed case notes of all consecutive patients diagnosed of Sch-PAH and IPAH referred to the Heart Institute in São Paulo, Brazil, between 2004 and 2008. None of the Sch-PAH received PAH specific treatment whereas all IPAH patients did. RESULTS: Sch-PH patients (n = 54) had less severe pulmonary hypertension as evidenced by lower levels of pulmonary vascular resistance (11.3 +/- 11.3 W vs. 16.7 +/- 10.6 W; p = 0.002) and mean pulmonary artery pressure (56.7 +/- 18.7 mm Hg vs. 64.6 +/- 17.4 mm Hg; p = 0.01) and higher cardiac output (4.62 +/- 1.5 l/min vs. 3.87 +/- 1.5 l/min; p = 0.009) at presentation than IPAH patients (n = 95). None of the Sch-PAH patients demonstrated a positive response to acute vasodilator testing, whereas 16.2% of IPAH patients did (p = 0.015). Survival rates at 1, 2, and 3 years were 95.1%, 95.1%, and 85.9% and 95%, 86%, and 82%, for Sch-PAH and IPAH, respectively (p = 0.49). Both groups had a higher survival rate when compared to IPAH survival as estimated by the NIH equation (71%, 61%, and 52%, respectively). CONCLUSIONS: Sch-PAH has a more benign clinical course than IPAH despite a lack of demonstrable acute vasoreactivity at hemodynamic evaluation.

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Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Surgical biopsy was usually required for diagnostic confirmation. However, the morbidity, mortality and limited benefit of this procedure have generated discussion regarding noninvasive diagnostic techniques. We present the case of a female patient with progressive dyspnea, hypoxemia and pulmonary hypertension, the last diagnosed via catheterization. Computed tomography revealed septal thickening and diffuse micronodules. Bronchoalveolar lavage revealed occult alveolar hemorrhage. Treatment with an endothelin antagonist was started, resulting in symptomatic and functional improvement. Occult alveolar hemorrhage differentiates PVOD from idiopathic pulmonary hypertension. We believe that this finding, in combination with characteristic tomographic findings, is sufficient to establish a diagnosis of PVOD.

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A doença veno-oclusiva pulmonar (DVOP) é uma causa rara de hipertensão pulmonar. A biópsia cirúrgica era usualmente necessária para seu diagnóstico; entretanto, sua morbidade, mortalidade e seu impacto limitado levantou a discussão sobre o diagnóstico não-invasivo. Apresentamos um caso de uma paciente com dispnéia progressiva, hipoxemia e hipertensão pulmonar no cateterismo. A tomografia computadorizada revelou espessamento septal e micronódulos difusos. O lavado broncoalveolar revelou hemorragia alveolar oculta. Iniciou-se tratamento com antagonista da endotelina, que resultou em melhora clínica e funcional. A hemorragia alveolar oculta é uma característica da DVOP capaz de diferenciá-la da hipertensão pulmonar idiopática. Acreditamos que sua presença, associada à tomografia característica, seja suficiente para o diagnóstico de DVOP.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Surgical biopsy was usually required for diagnostic confirmation. However, the morbidity, mortality and limited benefit of this procedure have generated discussion regarding noninvasive diagnostic techniques. We present the case of a female patient with progressive dyspnea, hypoxemia and pulmonary hypertension, the last diagnosed via catheterization. Computed tomography revealed septal thickening and diffuse micronodules. Bronchoalveolar lavage revealed occult alveolar hemorrhage. Treatment with an endothelin antagonist was started, resulting in symptomatic and functional improvement. Occult alveolar hemorrhage differentiates PVOD from idiopathic pulmonary hypertension. We believe that this finding, in combination with characteristic tomographic findings, is sufficient to establish a diagnosis of PVOD.

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Chronic thromboembolic pulmonary hypertension is the only potentially curable form of pulmonary hypertension, assuming that surgical treatment is possible. However, there are hindrances to making a definitive, noninvasive diagnosis. We present the case of a 40-year-old female patient with idiopathic pulmonary arterial hypertension, confirmed in 1994. This patient developed thrombi in pulmonary vessels (as an overlap syndrome) mimicking chronic thromboembolic pulmonary hypertension. The identification of these conditions, which present high intraoperative mortality and unsatisfactory surgical resolution, is quite difficult in clinical practice. We discuss the current approach to candidate selection for surgical treatment of chronic thromboembolic pulmonary hypertension and the possible repercussions of inappropriate selection.

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A hipertensão pulmonar associada ao tromboembolismo pulmonar crônico é a única forma potencialmente curável de hipertensão pulmonar, desde que o tratamento cirúrgico seja possível. Existem, entretanto, limitações para o diagnóstico não-invasivo definitivo. Apresentamos o caso de uma paciente de 40 anos, com diagnóstico confirmado de hipertensão arterial pulmonar idiopática desde 1994, a qual evoluiu com sobreposição de trombos em vasos pulmonares simulando hipertensão pulmonar associada ao tromboembolismo pulmonar crônico. O reconhecimento dessas condições, as quais apresentam alta mortalidade intra-operatória e baixa resolutividade cirúrgica, é muito difícil na prática clínica. Discutiremos a abordagem atual para a seleção de candidatos para o tratamento cirúrgico da hipertensão pulmonar associada ao tromboembolismo pulmonar crônico e as possíveis repercussões da seleção inadequada.

Chronic thromboembolic pulmonary hypertension is the only potentially curable form of pulmonary hypertension, assuming that surgical treatment is possible. However, there are hindrances to making a definitive, noninvasive diagnosis. We present the case of a 40-year-old female patient with idiopathic pulmonary arterial hypertension, confirmed in 1994. This patient developed thrombi in pulmonary vessels (as an overlap syndrome) mimicking chronic thromboembolic pulmonary hypertension. The identification of these conditions, which present high intraoperative mortality and unsatisfactory surgical resolution, is quite difficult in clinical practice. We discuss the current approach to candidate selection for surgical treatment of chronic thromboembolic pulmonary hypertension and the possible repercussions of inappropriate selection.

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