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.

Keywords: Hypertension, pulmonary; Cardiac catheterization, Ventricular dysfunction, left.

A pneumonia por Pneumocystis carinii (PPC), que é a infecção oportunista que mais comumente põe em risco a vida de pacientes com síndrome da imunodeficiência adquirida (SIDA), tem sido há bastante tempo associada a outros estados de imunodeficiência. Os relatos iniciais sugeriram que a apresentação clínica da PPC em SIDA difere de sua apresentação em outras situações, embora ambas tenham sofrido modificações nos últimos anos. O objetivo do presente estudo é comparar os achados clínicos e laboratoriais da PPC em SIDA com os de pacientes portadores de PPC sem SIDA. Foram revisados os prontuários médicos de todos os pacientes em nossa instituição que tiveram o diagnóstico confirmado de PPC e SIDA durante o ano de 1994 (17 pacientes) e sem SIDA no período de 1992-1994 (dez pacientes). Quando da apresentação, os pacientes sem SIDA tinham uma duração de sintomas significativamente menor (8,4 ± 7,7 vs 19,5 ± 10,2 dias, p < 0,05), menor PaO2 média a ar ambiente (47 ± 8,2 vs 63 ± 10mmHg, p < 0,05), e mais freqüente infiltrado unilateral no radiograma do tórax (60 vs 6%, p < 0,05). O índice de mortalidade da PPC foi marcadamente maior nos pacientes sem SIDA (40 vs 6%, p < 0,05), e este fato estava associado com retarde no início do tratamento específico nestes pacientes. Devido a seu curso mais agudo, apresentação radiológica atípica e alto índice de mortalidade, concluímos que o diagnóstico de PPC deve ser afastado em pacientes que recebem terapia imunossupressora e que profilaxia anti-PPC deve ser fortemente considerada em pacientes que serão imunossuprimidos.

Keywords: PPC-AIDS. PPC não-AIDS.

Objective: To look for correlations between lung function and cardiac dimension variables in morbidly obese patients, in order to test the hypothesis that the relative size of the small airways is independently correlated with left ventricular hypertrophy. Methods: This was a retrospective study involving 192 medical records containing a clinical protocol employed in candidates for bariatric surgery between January of 2006 and December of 2010. Results: Of the 192 patients evaluated, 39 (10 males and 29 females) met the inclusion criteria. The mean BMI of the patients was 49.2 ± 7.6 kg/m2, and the mean age was 35.5 ± 7.7 years. The FEF25-75/FVC, % correlated significantly with left ventricular posterior wall thickness and relative left ventricular posterior wall thickness, those correlations remaining statistically significant (r = −0.355 and r = −0.349, respectively) after adjustment for weight, gender, and history of systemic arterial hypertension. Stepwise multivariate linear regression analysis showed that FVC and FEV1 were the major determinants of left ventricular mass (in grams or indexed to body surface area). Conclusions: A reduction in the relative size of the small airways appears to be independently correlated with obesity-related cardiac hypertrophy, regardless of factors affecting respiratory mechanics (BMI and weight), gender, or history of systemic arterial hypertension. However, FEV1 and FVC might be important predictors of left ventricular mass in morbidly obese individuals.

Keywords: Obesity; Spirometry; Echocardiography; Body mass index.

Pulmonary hypertension is a common complication of sickle cell anemia. Despite the fact that the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of sickle cell anemia-related pulmonary hypertension is multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients, pulmonary arterial hypertension is the main cause of elevated pulmonary artery pressures. However, pulmonary venous hypertension also plays a role in a subgroup of patients. Specific data on the effects of treatment modalities for pulmonary hypertension in patients with sickle cell anemia are scarce. It is likely that all patients would benefit from maximization of sickle cell anemia therapy, and that patients with the severe form of the disease would benefit from treatment with selective pulmonary vasodilators and antiproliferative agents. Large trials evaluating the effects of treatment for pulmonary hypertension in the sickle cell anemia population are underway.

Keywords: Anemia, Sickle cell; Hemolysis; Hypertension, pulmonary; Nitric oxide.

Recent studies have suggested an association between pulmonary arterial hypertension (PAH) and thyroid diseases (hypothyroidism and hyperthyroidism). This combination has a good prognosis, because the increase in the pulmonary artery pressure is usually slight and reverses after the treatment of the thyroid disease. Although the exact mechanism involved in the pathogenesis of this combination has not yet been established, it has been hypothesized that thyroid hormones and autoimmunity have a direct influence. Due to the high prevalence of thyroid disease in patients with PAH, thyroid function tests should be considered in the investigation of every patient with PAH. In this review, we describe the prevalence of PAH in patients with thyroid diseases and the prevalence of thyroid disease in patients with PAH, as well as addressing the principal effects that thyroid diseases have on the respiratory system. In addition, we report the treatment effects in patients with these diseases.

Keywords: Hypertension, pulmonary; Graves disease; Hyperthyroidism; Hypothyroidism; Thyroid hormones; Echocardiography.

Objetivo: Realizar a tradução para a língua portuguesa e a adaptação cultural para uso no Brasil do Quality of Communication Questionnaire (QOC) para pacientes com DPOC em cuidados paliativos. Métodos: Após a aprovação do autor do questionário e do comitê de ética da instituição, a versão original do QOC com 13 itens foi traduzida, de forma independente, por dois tradutores brasileiros com fluência na língua inglesa. Uma síntese das duas traduções foi realizada por uma médica bilíngue e os dois tradutores, que chegaram a um consenso, gerando uma versão na língua portuguesa. Essa versão foi retraduzida por dois tradutores nativos de países de língua inglesa com fluência na língua portuguesa. Para resolver quaisquer discrepâncias, um comitê de especialistas comparou a versão original com todas as versões produzidas e, assim, obteve-se a versão pré-final do QOC. O pré-teste foi aplicado em 32 pacientes internados em UTIs de três hospitais públicos da grande Florianópolis (SC) para avaliar a clareza e a aceitabilidade cultural da versão pré-final do QOC. Resultados: A média de idade dos pacientes foi de 48,5 ± 18,8 anos. A maioria dos itens foi bem compreendida e aceita, recebendo pontuações ≥ 8. Um item, sobre morte, suscitou dificuldades na compreensão dos participantes do pré-teste. Ao submeter a retradução ao autor do QOC, ele solicitou alterações em dois itens, que foram acatadas. Após essas alterações, a versão final foi aprovada. Conclusões: A tradução e a adaptação cultural do QOC para uso no Brasil foi concluída com sucesso.

Keywords: Doença pulmonar obstrutiva crônica; Inquéritos e questionários; Comunicação; Unidades de terapia intensiva.

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.

Keywords: Hypertension, pulmonary; Exercise test; Hemodynamics.

Idiopathic pulmonary arterial hypertension (PAH) patients with a positive response to acute vasodilator challenge and a clinical response to calcium channel blockers (CCBs) for at least one year are traditionally designated true responders. Nevertheless, little is known about a sustained response to CCBs over longer periods of time. We evaluated the loss of response to CCBs after long-term treatment in a cohort of idiopathic PAH patients previously classified as being true responders. Our data suggest that idiopathic PAH patients can lose clinical response to CCBs even after one year of clinical stability, reinforcing the need for constant multidimensional reevaluation to assess the need for targeted PAH therapies and to classify these patients correctly.

Keywords: Pulmonary hypertension; Vasodilator agents; Calcium channel blockers.