A 30-Year-Old Woman With Arthralgia, Progressive Dyspnea, and a Syncope Episode.

CASE PRESENTATION: A 30-year-old White woman with presumed rheumatoid arthritis accompanied by CT scan evidence of eosinophilic pneumonitis was referred to the ED by her rheumatologist for an investigation of the progression of dyspnea. Approximately 6 months before, the patient reported experiencing diffuse interphalangeal arthralgias (both proximal and distal) that affected the wrists, knees, and feet. These symptoms were accompanied by Modified Medical Research Council scale grade 2 dyspnea. During the initial assessment, the patient exhibited slight pallor and had no indications of inflammatory activity in the joints. Furthermore, the cardiovascular physical examination and the auto-antibody laboratory profile yielded normal results. However, a wrist ultrasound scan revealed evidence of active synovitis; a chest CT scan displayed multifocal bilateral ground-glass opacities and mild thickening of the interlobular septa. These findings suggested the presence of eosinophilic disease or an acute interstitial process related to collagen vascular disease. Consequently, the patient's treatment commenced with a weekly dose of methotrexate (10 mg). Despite the intervention, 2 months later, the patient returned, reporting persistent arthralgia and a worsening of dyspnea, now classified as Modified Medical Research Council scale grade 3. Subsequently, the rheumatologist referred her to the ED for further assessment. During the initial emergency evaluation, the patient experienced a syncope episode accompanied by orthostatic prodromal symptoms that included dizziness, nausea, and malaise.

Estimation of pulmonary artery systolic pressure in hemodialysis patients and its association with cardiorespiratory fitness and pulmonary function.

PURPOSE: To evaluate the estimated pulmonary arterial systolic pressure (PASP) through transthoracic echocardiography in hemodialysis (HD) patients and associate it with cardiorespiratory fitness and pulmonary function. METHODS: This study was a cross-sectional analysis of HD patients that performed evaluations of cardiac function, cardiorespiratory fitness, and pulmonary function, through transthoracic echocardiography, cardiopulmonary exercise test, spirometry, and manovacuometry, respectively. All patients underwent the evaluations on a non-dialysis day. RESULTS: Thirty-five HD patients were evaluated and separated according to the presence of probable pulmonary hypertension (PH) (estimated PASP ≥ 35 mmHg) or not (estimated PASP < 35 mmHg). Those HD patients with probable PH had the worst cardiorespiratory fitness, evaluated by the peak oxygen consumption (VO2peak) (17.11 ± 4.40 versus 12.90 ± 2.73 mL/kg/min; p = 0.011), and pulmonary function, evaluated by absolute and predicted of forced vital capacity (FVC) (85.52 ± 12.29 versus 71.38 ± 11.63%; p = 0.005) and absolute and predicted of forced expiratory volume in the first second (FEV1) (83.37 ± 14.98 versus 69.21 ± 13.48%; p = 0.017). The secondary analysis showed that estimated PASP was correlated with VO2peak (r = - 0.508; p = 0.002), FVC (r = - 0.450; p = 0.007), and FEV1 (r = - 0.361; p = 0.033). Moreover, the adjusted odds ratio by HD vintage, dry weight and gender showed that increments in VO2peak (OR 1.62; CI 95% 1.04-2.54; p = 0.034), FVC (OR 39.67; CI 95% 1.74-902.80; p = 0.021), and FEV1 (OR 39.54; CI 95% 1.89-826.99; p = 0.018) were associated with 1-fold and 39-fold higher chance, respectively, for not having PH. However, all these associations were lost when age was included in the analysis. CONCLUSIONS: The HD patients with probable PH had the worst cardiorespiratory fitness and pulmonary function. Exploratory analyses showed that greater cardiopulmonary fitness was associated with better cardiac function. Moreover, increments in cardiorespiratory fitness and pulmonary function may increase the chance of not having PH.

Clinical Outcomes and Prognostic Factors in a Cohort of Adults With Cystic Fibrosis: A 7-Year Follow-Up Study.

BACKGROUND: Due to the heterogeneity of cystic fibrosis (CF), the longer survival observed in cohorts of adult subjects, and inter-population variations, there is a clear need to seek further information about clinical outcomes and prognostic factors in different cohorts of subjects with CF. Our objectives were to evaluate clinical outcomes and prognostic factors in a cohort of adult subjects with CF after a 7-y follow-up period and investigate longitudinal changes in clinical scores, spirometry, 6-min walk test performance, and pulmonary artery systolic pressure as assessed by Doppler echocardiography. METHODS: A cohort of clinically stable subjects (≥16 y old) who were enrolled in an adult CF program in 2004-2005 underwent clinical evaluation. Outcome was classified as good (survival) or poor (survival with lung transplantation or death). In 2011-2012, survivors were re-examined. RESULTS: Of 40 subjects with CF evaluated in 2004-2005, 32 (80%) survived, 2 (5%) survived with lung transplantation, and 6 (15%) died. Logistic regression analysis showed that a low percent-of-predicted FEV1 was associated with poor outcome. An FEV1 cut-off value of ≤30% and pulmonary artery systolic pressure of ≥42 mm Hg predicted poor outcome with high sensitivity, specificity, and positive and negative predictive values. Deterioration was observed in clinical scores (P = .03), FVC (P = .02), FEV1 (P < .001), distance walked in the 6-min walk test (P = .002), baseline SpO2 (P < .001), and final SpO2 (P < .001). CONCLUSIONS: After 7 y of follow-up, 20% of subjects with CF had a poor outcome. Pulmonary artery systolic pressure of ≥42 mm Hg and FEV1 of ≤30% were the most significant prognostic predictors of poor outcome. Clinical and functional deterioration was observed in survivors.

Sleep findings and predictors of sleep desaturation in adult cystic fibrosis patients.

PURPOSE: Questions remain about the polysomnographic findings and the predictors for sleep desaturation in cystic fibrosis (CF) patients. Our study aimed to evaluate sleep parameters in a sample of adult CF patients comparing them with healthy controls and to determine the best predictors of sleep desaturation in CF patients with awake resting peripheral oxygen saturation (SpO(2)) ≥90%. METHODS: In a cross-sectional study, with data collected prospectively, 51 clinically stable adult CF patients (mean age 25.1 ± 6.7 years) and 25 age-matched controls underwent an overnight polysomnography and answered sleep questionnaires. CF patients had their pulmonary function, 6-min walk test, and echocardiography assessed. RESULTS: CF patients and control subjects had similar sleep architecture. However, CF patients had impaired subjective sleep quality and a higher arousal index than controls. The apnea-hypopnea index was similar in both groups, and only two CF patients (3.9%) fulfilled the criteria for obstructive sleep apnea syndrome. Sleep desaturation was more common in CF patients (29.4% vs 0%; p < 0.001). In a logistic regression model, we observed that awake resting SpO(2) was the single best variable associated with sleep desaturation in CF population (p < 0.001). The awake SpO(2) <94% had a sensitivity, specificity, positive and negative predictive value for sleep desaturation of, respectively, 93.3%, 100%, 100%, and 97.3%. CONCLUSIONS: CF patients had a worse subjective sleep quality and small changes in sleep architecture. In nonhypoxic, adult CF patients, sleep desaturation is common, is not associated with obstructive sleep events, and can be accurately predicted by awake resting SpO(2) <94%.

NT-proBNP levels in systemic sclerosis: association with clinical and laboratory abnormalities.

OBJECTIVE: To identify factors related to NT-proBNP levels in systemic sclerosis (SSc). DESIGN AND METHODS: NT-proBNP was measured in 119 patients with SSc and 20 controls. Patients with transtricuspid gradient (TG) > or =36 mm Hg or > or =31 mmHg plus dyspnea were considered to have suspected systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). RESULTS: Increasing age, NYHA functional class, skin score, history of systemic arterial hypertension (SAH), anticentromere antibodies, diastolic dysfunction, reduced pulmonary diffusing capacity, and TG were positively associated with NT-proBNP. In multivariable linear regression, TG, age, and SAH were independently associated to NT-proBNP levels. An ROC curve analysis (with an area under the curve of 0.89, 95% CI: 0.83-0.95) suggested a cutoff of 157.8pg/mL to identify patients with suspected SScPAH, presenting a sensitivity of 100% (78.1-100) and specificity of 72.3% (62.3-80.5). CONCLUSIONS: NT-proBNP levels are related to clinical and laboratory abnormalities in SSc. The results indicate that NT-proBNP may be a useful tool in the evaluation of SScPAH.

Semiquantitative echocardiographic evaluation of intrapulmonary vascular dilatations: correlation with evaluation of shunt levels and pulmonary function parameters.

OBJECTIVE: To correlate semiquantitative evaluation of intrapulmonary vascular dilatations (IPVD) with quantitative evaluation of shunt levels, as well as to describe clinical and pulmonary function findings in a sample of liver disease patients with IPVD. METHODS: Patients presenting transthoracic echocardiography (TTE) positivity for IPVD underwent clinical evaluation, pulmonary function tests and pulmonary shunt quantification (scintigraphy with technetium-99m-labeled albumin macroaggregates and blood gas analysis after pure oxygen breathing). RESULTS: A total of 28 liver cirrhosis patients were studied (mean age, 47.5 years; 60.7% were Child-Pugh class B). A 4-point, ascending scale was used as a measure of IPVD intensity, which was scored as 1, 2, 3 and 4, respectively, in 13 (46.4%), 9 (32.1%), 2 (7.1%) and 4 (14.3%) of the patients. Patients were divided into a low-intensity group (scores 1 and 2) and a high-intensity group (scores 3 and 4). The mean shunt assessed using scintigraphy was 14.9% in the sample as a whole and was lower in the low-intensity group (11.7% vs. 26.3%; p = 0.01). The mean shunt by blood gas analysis was higher in the high-intensity group (8.3% vs. 16.3%; p < 0.001). Mean PaO2 was lower in the high-intensity group. There was a negative correlation between DLCO and IPVD severity (r = -0.406, p = 0.01). CONCLUSIONS: TTE is a safe, useful tool for assessing IPVD severity in liver disease patients. The IPVD intensity assessed using TTE correlated with the intrapulmonary shunt values obtained through the quantitative methods evaluated, as well as with pulmonary gas exchange abnormalities.

Avaliação semiquantitativa ecocardiográfica de dilatações vasculares intrapulmonares em candidatos a transplante hepático: correlação com avaliação de shunt e parâmetros funcionais pulmonares / Semiquantitative echocardiographic evaluation of intrapulmonary vascular dilatations: correlation with evaluation of shunt levels and pulmonary function parameters

OBJETIVO: Verificar a relação entre a avaliação semiquantitativa de dilatações vasculares intrapulmonares (DVIP) e a avaliação quantitativa de shunt, e descrever achados clínicos e funcionais pulmonares em uma amostra de hepatopatas com DVIP. MÉTODOS: Pacientes com ecocardiografia transtorácica (ETT) positiva para DVIP foram submetidos à avaliação clínica e de função pulmonar assim como à quantificação de shunt intrapulmonar (cintilografia com macroagregados de albumina marcados com tecnécio-99m e por gasometria com oxigênio a 100 por cento). RESULTADOS: Foram estudados 28 pacientes cirróticos (média de idade, 47,5 anos; 60,7 por cento dos casos classificados como Child-Pugh B). Uma escala de 4 pontos, em ordem ascendente, foi utilizada para medir a intensidade das DVIP, classificada de 1 a 4, respectivamente, em 13 (46,4 por cento), 9 (32,1 por cento), 2 (7,1 por cento) e 4 (14,3 por cento) dos pacientes. A amostra foi dividida em grupo baixa intensidade (escores 1 e 2) e grupo alta intensidade (escores 3 e 4). A média de shunt por cintilografia foi 14,9 por cento na amostra total, sendo menor no grupo baixa intensidade (11,7 por cento vs. 26,3 por cento; p = 0,01). O grupo alta intensidade teve maiores valores de shunt através de gasometria (8,3 por cento vs. 16,3 por cento; p < 0.001). A PaO2 média foi inferior no grupo alta intensidade. A intensidade de DVIP e a DLCO correlacionaram-se de forma inversa (r = -0,406, p = 0,01). CONCLUSÕES: A ETT é um método útil e seguro para avaliação da gravidade das DVIP em pacientes com hepatopatia. A classificação ecocardiográfica da intensidade das DVIP se correlacionou com valores de shunt intrapulmonar obtidos pelos métodos quantitativos avaliados, bem como com anormalidades nas trocas gasosas pulmonares.

OBJECTIVE: To correlate semiquantitative evaluation of intrapulmonary vascular dilatations (IPVD) with quantitative evaluation of shunt levels, as well as to describe clinical and pulmonary function findings in a sample of liver disease patients with IPVD. METHODS: Patients presenting transthoracic echocardiography (TTE) positivity for IPVD underwent clinical evaluation, pulmonary function tests and pulmonary shunt quantification (scintigraphy with technetium-99m-labeled albumin macroaggregates and blood gas analysis after pure oxygen breathing). RESULTS: A total of 28 liver cirrhosis patients were studied (mean age, 47.5 years; 60.7 percent were Child-Pugh class B). A 4-point, ascending scale was used as a measure of IPVD intensity, which was scored as 1, 2, 3 and 4, respectively, in 13 (46.4 percent), 9 (32.1 percent), 2 (7.1 percent) and 4 (14.3 percent) of the patients. Patients were divided into a low-intensity group (scores 1 and 2) and a high-intensity group (scores 3 and 4). The mean shunt assessed using scintigraphy was 14.9 percent in the sample as a whole and was lower in the low-intensity group (11.7 percent vs. 26.3 percent; p = 0.01). The mean shunt by blood gas analysis was higher in the high-intensity group (8.3 percent vs. 16.3 percent; p < 0.001). Mean PaO2 was lower in the high-intensity group. There was a negative correlation between DLCO and IPVD severity (r = -0.406, p = 0.01). CONCLUSIONS: TTE is a safe, useful tool for assessing IPVD severity in liver disease patients. The IPVD intensity assessed using TTE correlated with the intrapulmonary shunt values obtained through the quantitative methods evaluated, as well as with pulmonary gas exchange abnormalities.

[Chronic bacterial infection and echocardiographic parameters indicative of pulmonary hypertension in patients with cystic fibrosis]. / Infecção bacteriana crônica e indicadores ecocardiográficos de hipertensão pulmonar em pacientes com fibrose cística.

OBJECTIVES: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). METHODS: A prospective cross-sectional study involving CF patients (>16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 +/- 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. RESULTS: In terms of the following variables, no significant differences were found between P. aeruginosa-positive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV1; p = 0.693), radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). CONCLUSIONS: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

Infecção bacteriana crônica e indicadores ecocardiográficos de hipertensão pulmonar em pacientes com fibrose cística / Chronic bacterial infection and echocardiographic parameters indicative of pulmonary hypertension in patients with cystic fibrosis

OBJETIVO: Determinar as relações entre infecção bacteriana crônica e hipertensão pulmonar, avaliada por ecocardiografia Doppler, em pacientes com fibrose cística (FC). MÉTODOS: Estudo transversal e prospectivo em pacientes com FC (idade > 16 anos) atendidos por um programa para adultos com a doença. O estudo incluiu 40 pacientes com média de idade de 23,7 ± 6,3 anos. Os pacientes foram submetidos a avaliação clínica, ecocardiografia Doppler, testes de função pulmonar, exame radiológico do tórax e exames culturais do escarro de Pseudomonas aeruginosa e Burkholderia cepacia. RESULTADOS: Não foram observadas diferenças entre os casos positivos para P. aeruginosa e os negativos para P. aeruginosa quanto às seguintes variáveis: escore clínico (p = 0,472); volume expiratório forçado no primeiro segundo (VEF1; p = 0,693); escore radiológico (p = 0,760); velocidade de regurgitação tricúspide (VRT, p = 0,330); diâmetro do ventrículo direito (DVD, p = 0,191); e tempo de aceleração sistólica (TAS) do ventrículo direito/artéria pulmonar (VD/AP, p = 0,330). O VEF1 foi significativamente menor nos casos positivos para B. cepacia do que nos casos negativos para B. cepacia (p = 0,011). Não foram observadas diferenças entre os casos positivos para B. cepacia e os casos negativos para B. cepacia quanto às seguintes variáveis: escore clínico (p = 0,080); escore radiológico (p = 0,760); VRT (p = 0,613); DVD (p = 0,429); e TAS do VD/AP (p = 0,149). CONCLUSÕES: Não foi observada relação entre infecção crônica por P. aeruginosa e por B. cepacia com hipertensão pulmonar em pacientes adultos com FC. A função pulmonar foi pior nos pacientes positivos para B. cepacia do que nos pacientes positivos para P. aeruginosa.

OBJECTIVES: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). METHODS: A prospective cross-sectional study involving CF patients (>16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 ± 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. RESULTS: In terms of the following variables, no significant differences were found between P. aeruginosa-positive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV1; p = 0.693), radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). CONCLUSIONS: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

TCRBV20S1 and TCRBV3S1 gene segment polymorphisms in systemic sclerosis.

OBJECTIVE: To compare the frequencies of variants of TCRBV20S1 and TCRBV3S1 gene segments in patients with systemic sclerosis (SSc) and in controls. The null allele (allele 2) of TCRBV20S1 is associated with reduced levels of Vbeta20+ T-cells in the peripheral blood, while allele 1 of TCRBV3S1 is related to a low frequency of Vbeta3.1+ T-cells. METHODS: One hundred thirty patients with SSc and 118 healthy volunteer controls were genotyped for TCRBV20S1, and 117 patients and 85 controls were genotyped for TCRBV3S1 variants by PCR-RFLP. Patients underwent clinical evaluation, serology, pulmonary function tests, high resolution computed tomography, and Doppler echocardiography. RESULTS: The genotypic frequencies of TCRBV20S1 were 0.46 (allele 1/allele 1), 0.43 (allele 1/allele 2), and 0.11 (allele 2/allele 2) in SSc patients; in controls the frequencies were 0.70, 0.26, and 0.04, respectively (p < 0.001). The Mantel-Haenszel odds ratio (stratified by race and sex) of the allele 2 carrier state was 3.88 (95% CI 1.94 to 7.75). The allelic and genotypic frequencies of the TCRBV3S1 gene segment did not differ significantly in patients and controls. However, among patients, allele 1 (TCRBV3S1) carriers had a higher prevalence of interstitial lung disease (adjusted p = 0.032). CONCLUSION: The null allele of the TCRBV20S1 and the allele 1 of TCRBV3S1 gene segments may be considered risk factors for the development of SSc and interstitial lung disease, respectively, suggesting a protective role of Vbeta20+ and Vbeta3.1+ cells in the pathogenic immune responses in SSc.

Prevalence of pulmonary hypertension evaluated by Doppler echocardiography in a population of adolescent and adult patients with cystic fibrosis.

OBJECTIVE: To determine the prevalence of pulmonary hypertension (PH) in patients with cystic fibrosis (CF), to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. METHODS: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older) enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. RESULTS: Tricuspid regurgitant jet velocity (TRV) was obtained in 37 of the 40 patients studied. The prevalence of PH was 49% with a TRV cut-off of 2.5 m/s (18 patients) and 30% with a TRV cut-off of 2.8 m/s (11 patients). Peripheral oxygen saturation (SpO2) at rest, clinical score, forced expiratory volume in one second (FEV1), and forced vital capacity (FVC) were significantly lower in the group with PH. The TRV was found to correlate significantly with SpO2 at rest (p < 0.001), clinical score (p < 0.001), radiographic score (p = 0.030), FEV1 in liters (p < 0.001) and in % of predicted (p < 0.001), and FCV in liters (p = 0.008) and in % of predicted (p = 0.001). The single best predictor of TRV was SpO2 at rest (p < 0.001). CONCLUSION: The high prevalence of PH in the CF patients studied suggests that PH should be considered in the evaluation and follow-up treatment of such patients. The best predictor of PH was SpO2 at rest.

Prevalence of pulmonary hypertension evaluated by Doppler echocardiography in a population of adolescent and adult patients with cystic fibrosis / Prevalência de hipertensão pulmonar avaliada por ecocardiografia Doppler em uma população de pacientes adolescentes e adultos com fibrose cística

OBJETIVO: Determinar a prevalência de hipertensão pulmonar (HP) em pacientes com fibrose cística (FC), comparar características clínicas, escore radiológico, função pulmonar e parâmetros ecocardiográficos nos grupos com e sem HP e correlacionar achados ecocardiográficos com características clínicas, escore radiológico e função pulmonar. MÉTODOS: Estudo transversal prospectivo em pacientes clinicamente estáveis (idade > 16 anos) atendidos por um programa de adultos para FC. Os pacientes foram submetidos a avaliação clínica, ecocardiografia Doppler, testes de função pulmonar e exame radiológico do tórax. RESULTADOS: Obteve-se a velocidade de regurgitação tricúspide (VRT) em 37 dos 40 pacientes estudados. A prevalência de HP foi de 49 por cento com um ponte de corte da VRT de 2,5 m/s (18 pacientes) e de 30 por cento com um ponte de corte da VRT de 2,8 m/s (11 pacientes). Os valores de saturação periférica de oxigênio (SpO2) em repouso, escore clínico, volume expiratório forçado no primeiro segundo (VEF1) e capacidade vital forçada (CVF) foram significativamente menores no grupo com HP. A VRT correlacionou-se significativamente com SpO2 em repouso (p < 0,001), escore clínico (p < 0,001), escore radiológico (p = 0,030), VEF1 em litros (p < 0,001) e em por cento do previsto (p < 0,001) e CVF em litros (p = 0,008) e em por cento do previsto (p = 0,001). A SpO2 em repouso foi o melhor preditor independente da VRT (p < 0,001). CONCLUSÃO: A alta prevalência de HP nos pacientes com FC estudados sugere que a presença de HP seja considerada na avaliação e acompanhamento desses pacientes. O melhor preditor de HP foi a SpO2 em repouso.

OBJECTIVE: To determine the prevalence of pulmonary hypertension (PH) in patients with cystic fibrosis (CF), to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. METHODS: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older) enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. RESULTS: Tricuspid regurgitant jet velocity (TRV) was obtained in 37 of the 40 patients studied. The prevalence of PH was 49 percent with a TRV cut-off of 2.5 m/s (18 patients) and 30 percent with a TRV cut-off of 2.8 m/s (11 patients). Peripheral oxygen saturation (SpO2) at rest, clinical score, forced expiratory volume in one second (FEV1), and forced vital capacity (FVC) were significantly lower in the group with PH. The TRV was found to correlate significantly with SpO2 at rest (p < 0.001), clinical score (p < 0.001), radiographic score (p = 0.030), FEV1 in liters (p < 0.001) and in percent of predicted (p < 0.001), and FCV in liters (p = 0.008) and in percent of predicted (p = 0.001). The single best predictor of TRV was SpO2 at rest (p < 0.001). CONCLUSION: The high prevalence of PH in the CF patients studied suggests that PH should be considered in the evaluation and follow-up treatment of such patients. The best predictor of PH was SpO2 at rest.

Masked hypertension, urinary albumin excretion rate, and echocardiographic parameters in putatively normotensive type 2 diabetic patients.

OBJECTIVE: To evaluate the impact of masked hypertension in normotensive type 2 diabetic patients on microvascular complications and echocardiographic parameters. RESEARCH DESIGN AND METHODS: A cross-sectional study was conducted in 135 normotensive patients with type 2 diabetes. Patients underwent urinary albumin excretion rate (UAER) measurement, echocardiography, and 24-h ambulatory blood pressure monitoring (ABPM). Patients with increased daytime blood pressure levels (> or = 135/85 mmHg) were classified as having masked hypertension. RESULTS: The prevalence of masked hypertension was 30% (n = 41). Normotensive and masked hypertensive subjects, based on ambulatory blood pressure, were not different in terms of age, diabetes duration, smoking status, BMI, waist circumference, serum creatinine, glycemic, or lipid profiles. The office systolic blood pressure was higher in those with masked hypertension (127.8 +/- 7.5 vs. 122.9 +/- 10.2 mmHg, P = 0.003) than in the normotensive group. UAER also was increased in the group with masked hypertension (21.3 microg/min [range 2.5-1,223.5] vs. 8.1 microg/min [1.0-1,143.0], P = 0.001), as was the interventricular septum (1.01 +/- 0.15 vs. 0.94 +/- 0.13 cm, P = 0.015) and posterior wall (0.96 +/- 0.12 vs. 0.90 +/- 0.10 cm, P = 0.006) thickness. After adjustments for diabetes duration, sex, smoking, LDL cholesterol, and A1C values, all associations were sustained for daytime systolic blood pressure but not for office systolic blood pressure. CONCLUSIONS: Type 2 diabetic patients with masked hypertension have higher UAER as well as enlargement of ventricular walls compared with the normotensive patients, according to ABPM. Therefore, ABPM is important to identify this high-risk group so as to be able to take interventionist measures.

Doppler echocardiogram, oxygen saturation and submaximum capacity of exercise in patients with cystic fibrosis.

STUDY OBJECTIVES: To determine the relationship between pulmonary arterial systolic pressure (PASP) and submaximum capacity of exercise, using the six-minute walk test (6MWT) in patients with cystic fibrosis (CF), and to investigate the relation between echocardiographic findings and results of 6MWT, clinical scores, chest radiograph scores and lung function tests. DESIGN: This was a prospective cross-sectional study in patients with CF (16 years and older) with clinical stability, attending the Adult CF Program at the Hospital de Clínicas de Porto Alegre. The patients had Doppler echocardiography and performed a 6MWT. As well as pulmonary function tests and chest roentgenograms, and a clinical score was obtained for all patients. RESULTS: The study included 39 patients with a mean age of 23.7+/-6.3 years. There were no significant correlation between the distance walked and PASP, diameter of the right ventricle (DRV) and pulmonary acceleration time (p>0.05). We observed a significant correlation among PASP and the SpO(2) at rest (r=-0.73; p<0.001), SpO(2) at the end of the 6MWT (r= -0.45; p=0.006), clinical score (r= -0.55; p=0.001), chest radiograph score (r= -0.33; p=0.049), FEV1 (r= -0.63; p< 0.001), and FVC (r=-0.55; p=0.001). Right ventricular outflow tract flow acceleration time (Ac T) was significantly correlated only with the FEV1 (r=0.32; p=0.047). RVD was significantly correlated with SpO(2) at rest (r= -0.44; p=0.005) and clinical score (r= -0.38; p=0.017). The SpO(2) at rest was the single best predictor of PASP and this effect was independent of the relationship between other independent variables (p=0.001). The declining pulmonary function was significantly associated with PASP (p<0.001), SpO(2) at rest (p=0.001), SpO(2) at the end of the 6MWT (p=0.007) and difference between peripheral oxygen saturation at resting and at the end of the 6MWT (p=0.025). CONCLUSION: The PASP was not significantly correlated with the distance walked during the 6MWT in patients with CF. The PASP was strongly correlated with oxygen status at rest. The SpO(2) at rest was the best predictor of PASP. Also, PASP was strongly correlated with Shwachman-Kulczycki score, FEV(1), and FVC in this population.

Diastolic dysfunction and mortality in diabetic patients on hemodialysis: a 4.25-year controlled prospective study.

Among patients on hemodialysis, the mortality rate is higher in individuals with diabetes than in nondiabetic individuals, especially due to cardiovascular causes. The objective of the present study was to evaluate the role of echocardiographic abnormalities to predict mortality in diabetic patients starting hemodialysis. A 4.25-year prospective study was carried out with 40 diabetic and 28 nondiabetic patients starting hemodialysis in five dialysis centers in the metropolitan area of Porto Alegre, Brazil, between August 1996 and June 1999. Cardiovascular status was evaluated based on World Health Organization criteria, resting electrocardiogram (ECG), myocardial scintigraphy (at rest and after dipyridamole administration), and M-mode and Doppler echocardiography. Left ventricular diastolic function was classified into the following filling patterns: normal, impaired relaxation, pseudonormal, or restrictive. The survival rate was analyzed by Kaplan-Meier curves and predictors of death by Cox's proportional-hazards model. At the end of the study, the overall mortality rate was higher in patients with diabetes [19/40 (47.5%)] than in those without diabetes [2/28 (7.1%), P=.0013, log rank test]. Pseudonormal and restrictive filling patterns (HR: 3.2; 95% CI: 1.2-8.8; P=.02) and presence of diabetes (HR: 4.7; 95% CI: 1.03-21.4; P=.04) were associated with mortality. In conclusion, left ventricular diastolic dysfunction (LVDD) was the main predictor of mortality in this cohort of diabetic and nondiabetic patients starting dialysis. Intensive treatment of cardiovascular risk factors before the start of dialysis and during the treatment might reduce the mortality rate in diabetic patients.

Urinary albumin excretion rate is associated with increased ambulatory blood pressure in normoalbuminuric type 2 diabetic patients.

OBJECTIVE: To evaluate the 24-h blood pressure profile in normoalbuminuric type 2 diabetic patients. RESEARCH DESIGN AND METHODS: A cross-sectional study was conducted in 90 type 2 diabetic patients with a urinary albumin excretion rate (UAER) <20 microg/min on two occasions, 6 months apart (immunoturbidimetry). Patients underwent clinical and laboratory evaluations. Ambulatory blood pressure monitoring and echocardiograms were also performed. RESULTS: UAER was found to correlate positively with systolic doctor's office blood pressure measurements (r = 0.243, P = 0.021) and ambulatory blood pressure (24 h: r = 0.280, P = 0.008) and left ventricular posterior wall thickness (r = 0.359, P = 0.010). Patients were divided into four groups according to UAER (<5, > or =5-10, > or =10-15, and > or =15-20 microg/min). Systolic blood pressure parameters for the 1st, 2nd, 3rd, and 4th groups, respectively, were 123.0 +/- 10.6, 132.5 +/- 15.0, 139.0 +/- 23.4, and 130.7 +/- 8.0 mmHg for 24-h blood pressure (ANOVA P = 0.004) and 48.4 +/- 6.0, 54.5 +/- 11.2, 58.8 +/- 15.6, and 57.6 +/- 8.0 mmHg for 24-h pulse pressure (ANOVA P = 0.003). A progressive increase in the prevalence of diabetic retinopathy was observed from the 1st to the 4th UAER group: 27.3, 43.8, 45.5, and 66.7% (P = 0.029 for trend). CONCLUSIONS: In type 2 diabetic patients, UAER in the normoalbuminuric range is positively associated with systolic ambulatory blood pressure indexes, left ventricular posterior wall thickness, and diabetic retinopathy, suggesting that intensive blood pressure treatment may prevent diabetes complications in these patients.

Nailfold capillary microscopy can suggest pulmonary disease activity in systemic sclerosis.

OBJECTIVE: To evaluate the association of capillaroscopic alterations with pulmonary disease activity in systemic sclerosis (SSc). METHODS: Ninety-one patients with SSc were studied by means of interview, physical examination, nailfold capillary microscopy (NCM), serology, pulmonary function tests, esophageal transit scintigraphy, Doppler echocardiography, and pulmonary high resolution computed tomography (HRCT). Pulmonary disease activity was diagnosed by the observation of ground-glass opacities on pulmonary HRCT. Capillary loss on NCM was evaluated using the avascular score: patients with mean score > or = 1 or mean number of megacapillaries per finger > or = 1 were considered to have severe capillaroscopic alterations. RESULTS: Patients with higher skin scores, longer disease duration, signs of peripheral ischemia, esophageal dysfunction, antitopoisomerase I antibodies, and ground-glass opacities had higher mean avascular scores (p < or = 0.05 in all tests). The association between ground-glass opacities and higher avascular scores was particularly strong in patients with disease duration < or = 5 years. Among these patients, ground-glass opacities were present in 14 of 19 patients with severe NCM alterations, but were absent in all patients (n = 8) with mild or no NCM alterations (p < 0.001). ROC curves confirmed the ability of NCM to discriminate between patients with and without ground-glass opacities among those with disease duration < or = 5 years. However, NCM could not predict the presence of reduced pulmonary diffusing capacity. CONCLUSION: The severity of NCM abnormalities is associated with lung disease activity in SSc, particularly when the disease duration is relatively short.

Capilaroscopia periungueal pode sugerir atividade de doença pulmonar na esclerose sistêmica / Nailfold capillaroscopy can predict active pulmonary involvement in systemic sclerosis

Objetivo: Estudar a associação entre alterações capilaroscópicas e lesões em órgãos-alvo na esclerose sistêmica (ES). Métodos: Noventa e um pacientes com ES foram avaliados através de entrevista, exame físico, capilaroscopia periungueal (CPU), sorologia, provas de função pulmonar, cintilografia de trânsito esofágico, ecocardiografia com Doppler e tomografia computadorizada de alta resolução pulmonar (TCAR). A deleção capilar na CPU foi avaliada usando o escore de deleção vascular de Lee; consideram-se alterações severas na CPU um escore médio de deleção maior e igual 1 ou número médio de megacapilares por dedo maior e igual 1 para fins de análise. Hipertensão arterial pulmonar (HAP) foi definida como pressão sistólica na artéria pulmonar maior e igual 40 mmHg. Resultados: Pacientes com alterações capilaroscópicas severas apresentaram maior prevalência de áreas de opacidade em vidro-fosco (OVF) (P=0,016), redução da capacidade difusional pulmonar (P=0,026) e disfunção esofágica (P=0,001). HAP ocorreu somente em pacientes com alterações severas na CPU (P=0,114). Naqueles com duração de doença menor e igual 5 anos, OVF estavam presentes em 14 de 19 pacientes com alterações severas na CPU, mas não estavam presentes nos 8 pacientes com alterações capilaroscópicas leves ou ausentes (<0,001). Nesse subgrupo, nenhuma outra variável clínica ou laboratorial associou-se à presença de OVF. O uso de curvas ROC mostrou uma boa capacidade de CPU em discriminar pacientes com e sem o desfecho combinado de HAP ou doença intersticial pulmonar em pacientes com duração de doença menor e igual 5 anos. Conclusões: A severidade das anormalidade da CPU está associada com dano em órgãos-alvo na ES. A CPU pode indicar a presença de doença pulmonar ativa (representada por áreas de opacidade em vidro-fosco na TCAR) na ES de duração relativamente curta.

Hemangioma cavernoso do mediastino / Mediastinal cavernous hemangioma

Os hemangiomas de mediastino säo causa rara de massa mediastinal, compreendendo pouco mais de que 0,5 por cento dos tumores com esta localizaçäo. Säo mais comuns em crianças e adultos jovens, com frequência semelhante entre homens e mulheres, localizando-se, preferencialmente, nos compartimentos anterior e superior do mediastino. Apesar da natureza benígna, representada pelo crescimento lento, por elementos celulares maduros à histopatologia e pela ausência de infiltraçäo de estruturas adjacentes, sua extensäo, por vezes acentuada, bem como a proximidade de estruturas nobres, dificultam a abordagem cirúrgica que é o principal método de tratamento desta entidade. Os autores relatam um caso de uma paciente com esta enfermidade, discutindo suas dificuldades diagnósticas e terapêuticas

Insuficiência cardíaca refratária e sepse em portador de miocardiopatia hipertrófica / Retractory Heart Failure and Sepsis in Hypertrophic Cardiomyopathy

Home de 21 anos, portador de miocardiopatia hipertrófica obstrutiva com gradiente em repouso qeu desenvolve endocardite infecciosa (EI) subaguda e insuficiência mitral por destruiçäo do aparelho valvular. Na evoluçäo ocorre insuficiência cardíaca refratária e sepse. É analisada a associaçäo dessas doenças e as dificuldades no manejo, além dos casos descritos na literatura. É ressaltada a alta mortalidade dessa condiçäo e a indicaçäo cirúrgica precoce, bem como enfatizada a necessidade de profilaxia para EI em pacientes com obstruçäo em repouso