Introdução: A linfangioleiomiomatose (LAM) está associada a HP e está incluída no grupo 5 da classificação atual (mecanismos multifatoriais desconhecidos). No entanto, os dados referentes à ocorrência de HP na LAM são escassos. Os objetivos do estudo foram avaliar a prevalência e as características da HP em pacientes com LAM em diferentes estágios de evolução, além de comparar as características clínicas, funcionais, do teste de caminhada de 6 minutos (TC6M) e da qualidade de vida das pacientes com e sem HP. Metodologia: Cento e cinco pacientes com LAM foram submetidos a ecocardiograma, prova de função pulmonar (PFP) e TC6M. Pacientes com suspeita de HP no ecocardiograma, definida pela presença de pressão arterial pulmonar sistólica estimada (PsAP) acima de 35 mmHg, ou PFP mostrando DLco abaixo de 40% do valor previsto, foram submetidos a cateterismo cardíaco direito para confirmar o diagnóstico de HP. Resultados: Oito pacientes (7,6%) tinham HP confirmada no cateterismo cardíaco direito, seis pacientes (5,7%) tinham padrão pré-capilar e dois pacientes (1,9%) tinham padrão pós-capilar. Apenas um paciente (1%) apresentou pressão média de artéria pulmonar (PAPm) acima de 35 mmHg. Os pacientes com HP apresentaram menor VEF1 e DLco em PFP e maior dessaturação de oxigênio e intensidade de dispneia durante o TC6M comparado com aqueles sem PH. Em 63% dos pacientes com HP confirmada, o cateterismo cardíaco direito foi realizado devido ao resultado do DLco. Conclusões: A prevalência de HP é baixa em pacientes com LAM. A hipertensão pulmonar é de pouca gravidade e significativamente associada ao envolvimento parenquimatoso pulmonar. A capacidade de difusão de monóxido de carbono foi bastante útil na identificação de HP em pacientes com LAM / Introduction: Lymphangioleiomyomatosis (LAM) is associated with pulmonary hypertension (PH) and is included in group 5 of the current classification (unknown multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The objectives of the study were to evaluate the prevalence and characteristics of PH in patients with LAM at different stages of evolution, as well as to compare the clinical and functional characteristics of the 6-minute walk test (6MWT) and the quality of life of patients with and without PH. Methodology: One hundred and five patients with LAM underwent echocardiogram, pulmonary function test (PFT) and 6MWT. Patients with suspected PH on the echocardiogram, defined as the presence of estimated systolic pulmonary arterial pressure (PsAP) above 35 mmHg, or PFT showing carbon monoxide diffusion (DLco) below 40% of the predicted value, were submitted to right cardiac catheterization to confirm the diagnosis of PH. Results: Eight patients (7.6%) had PH confirmed in right cardiac catheterization, six patients (5.7%) had a pre-capillary pattern and two patients (1.9%) had a post capillary pattern. Only one patient (1%) presented mean pulmonary artery pressure (PAPm) above 35 mmHg. Patients with PH had lower FEV1 and DLco in PFP and greater oxygen desaturation and dyspnea intensity during the 6MWT compared to those without PH. In 63% of patients with confirmed PH, right heart catheterization was performed because of the DLco result. Conclusions: The prevalence of PH is low in patients with LAM. Pulmonary hypertension is commonly mild and is significantly associated with pulmonary parenchymal involvement. The measure DLco has improved the identification of PH in patients with LAM

Introdução: A esquistossomose (Sch) é uma das doenças infecciosas crônicas mais prevalentes do mundo. Entretanto, dados a respeito de uma de suas complicações, a hipertensão arterial pulmonar (HAP), são escassos. O objetivo deste estudo é avaliar a história natural de pacientes com HAP-Sch comparados a pacientes com HAP idiopática (HAPI). Métodos: Análise retrospectiva dos prontuários de todos os pacientes consecutivamente diagnosticados como HAP-Sch e HAPI no Instituto do Coração, São Paulo, Brasil, entre 2004 e 2008. Nenhum dos pacientes com Sch-PAH recebeu tratamento específico para HAP enquanto todos os pacientes com HAPI receberam. Resultados: Pacientes com HAP-Sch (n=54) apresentavam hipertensão pulmonar menos grave ao diagnóstico, com menores níveis de resistência vascular pulmonar (11.3 ± 11.3 vs 16.7 ± 10.6 UI; p=0.002) e pressão média de artéria pulmonar (56.7 ± 18.7 vs. 64.6 ± 17.4 mmHg; p=0.01) e ainda maior débito cardíaco (4.62 ± 1.5 vs. 3.87 ± 1.5 L/min; p=0.009) quando comparados com os pacientes com HAPI (n=95). Nenhum dos pacientes HAP-Sch apresentou resposta positiva ao teste agudo com vasodilatador enquanto 16.2% dos pacientes com HAPI apresentaram (p=0.015). As taxas de sobrevida em 1, 2 e 3 anos foram 95.1%, 95.1%, 85.9% e 95%, 86% e 82%, para HAP-Sch e HAPI, respectivamente (p=0.49). Ambos os grupos tinham uma maior taxa de sobrevida quando comparadas àquela estimada pela equação do NIH para os pacientes com HAPI sem tratamento específico para HAP (71%, 61% e 52%, respectivamente). Conclusão: HAP-Sch tem um curso clínico mais benigno do que HAPI apesar da falta de vasorreatividade aguda na avaliação hemodinâmica inicial / Background: Schistossomiasis (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. The objective of this study was to evaluate the natural history of Sch-PAH patients as compared to idiopathic PAH (IPAH). 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. Findings: Sch-PH patients (n=54) had less severe pulmonary hypertension as evidenced by lower levels of pulmonary vascular resistance (11.3 ± 11.3 vs 16.7 ± 10.6 IU; p=0.002) and mean pulmonary artery pressure (56.7 ± 18.7 vs. 64.6 ± 17.4 mmHg; p=0.01) and higher cardiac output (4.62 ± 1.5 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%, 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 untreated IPAH survival as estimated by the NIH equation (71%, 61% and 52%, respectively). Conclusion: Sch-PAH has a more benign clinical course than IPAH despite a lack of demonstrable acute vasoreactivity at hemodynamic evaluation

La fraction d'éjection du ventricule droit (FEVD) est un marqueur de survie en cas d'hypertension pulmonaire (PH), mais sa mesure est compliquée et fastidieuse. Le TAPSE (Tricuspid Annular Plane Systolic Excursion) est un bon indice de la FEVD mais il ne mesure que la composante longitudinale de la contraction ventriculaire droite. La fraction de variation surfacique du ventricule droit RVFAC (Right Ventricular Fractional Area Change) semble être un meilleur indice de FEVD car il prend en compte le sens longitudinal et transversal des éléments de la contraction du ventricule droit. Le but de notre étude était d'évaluer la performance RVFAC fonction de la sévérité hémodynamique chez les deux groupes de patients atteints de PH:l'hypertension artérielle pulmonaire (HAP) et l'hypertension pulmonaire thromboembolique chronique (CTEPH).Methodes: Soixante-deux patients atteints d'HTAP et CTEPH ont bénéficié d’un cathétérisme cardiaque droit et d’une IRM cardiaque dans un délai de 72 heures. Les surfaces ventriculaires droite et gauche a la fin de la diastole (RVEDA, LVEDA), la surface du ventricule droit a la fin da la systole (RVESA) et TAPSE ont été mesurés dans la vue quatre cavités. Le RVFAC (RVFAC=RVEDA-RVESA/RVEDA) et le rapport RVEDA/LVEDA ont été calculés. Le diamètre entre la paroi libre et le septum (DF-S) et le diamètre entre les parois antérieure et postérieures du ventricule gauche (DA-P) ont été mesurés et l'indice d’excentricité LV (IE) a été calculé (= DA-P / DF-S). Le RVEF a été calculée à l'aide de coupes jointives de 6 mm en petit axe du ventricule droit.Résultats: La population avait un âge moyen de 58 ans avec une majorité des femmes, la plupart des patients étaient en classe fonctionnelle III, 23 avaient des HAP) et 39 des CTEPH. La RVEF était faiblement corrélée aux variables hémodynamiques de la post-charge et de la fonction VD. Le RVFAC était plus fortement corrélée à FEVD (R2 = 0,65, p <0,001) que TAPSE (R2 = 0,35, p <0,001). Une FEVD <35% était mieux prédite par un RVFAC bas que par une diminution de TAPSE (TAPSE: AUC 0,73 et RVFAC: AUC 0,93, p = 0,0065). Nous avons divisé la population par la médiane de la résistance artérielle pulmonaire (RAP) et nous avons observé que dans le groupe avec la pire sévérité hémodynamique, cette différence a augmenté: dans le groupe avec PVR < 8,5 UW (RVFAC: R2 = 0,66, p <0,001 et TAPSE: R2 = 0,30, p =0,002) et dans le groupe avec PVR > 8,5 UW (RVFAC: R2 = 0,51, p <0,001 et TAPSE: R2 = 0,14, p = 0,041). Le groupe avec PVR> 8,5 WU avait un rapport RVEDA/LVEDA augmenté et une augmentation de l`indice excentricité. Les relations RVEF-RVFAC n’étaient pas différentes entre les groupes de HAP et CETPH.Conclusion: La fraction de variation surfacique du ventricule droit RVFAC fournit un reflet simple et fiable de la FEVD peut-être parce que contrairement à TAPSE qui ne prend en compte que le raccourcissement longitudinal, RFVAC prend également en compte la composante transversale de la fonction ventriculaire droite. / The right ventricular ejection fraction (RVEF) is a surrogate marker in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The TAPSE (Tricuspid Annular Plane Systolic Excursion) is a good index of RVEF, though it measures only the longitudinal component of right ventricular contraction. The RVFAC (Right Ventricular Fractional Area Change) seems to be a better index of RVEF because it takes into account the longitudinal and the transversal components of right ventricular contraction. The aim of our study was to evaluate the RVFAC performance according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolicpulmonary hypertension (CTEPH).Methos: Sixty-two patients with PAH and CTEPH underwent right heart catheterization and cardiac MR in a 72-hour delay. The right and left ventricle end diastolic areas (RVEDA, LVEDA), the right ventricle end systolic area (RVESA) and TAPSE were measured in the four chamber view. The RVFAC (RVFAC=RVEDA–RVESA/RVEDA) and the RVEDA/LVEDA relationship werecalculated. The diameter between the left ventricle (LV) free wall and the septum (dL-S) and the diameter between the anterior and posterior walls (dAP) were measured and the LV eccentricity index (EI) was calculated (EI=dAP/dL-S). The RVEF was calculated by using 6 mm RV short axis cines.Results: The population had mean age of 58 years with female majority, most of the patients were in functional class III, 23 had pulmonary arterial hypertension (PAH) and 39 had chronic thromboembolic pulmonary hypertension (CTEPH). The RVEF was weakly correlated to the hemodynamic variables of RV afterload and function. The RVFAC was morestrongly correlated to RVEF (R2=0.65, p<0.001) than TAPSE (R2=0.35, p<0.001). RVEF<35% was better predicted by RVFAC than TAPSE (TAPSE: AUC 0.73 and RVFAC: AUC 0.93, p=0.0065). We divided the population by the median of the pulmonary vascular resistance (PVR) and we observed that in the group with worse hemodynamic severity this difference increased: inthe group with PVR<8,5WU (RVFAC: R2=0.66, p<0.001 and TAPSE: R2=0.30, p=0.002) and in the group with PVR>8,5 WU (RVFAC: R2=0.51, p<0.001 and TAPSE: R2=0.14, p=0.041). The group with PVR>8,5WU had an increased RVEDA/LVEDA and an increased EI. There was no differences in the RVEF relationships between the groups of PAH and CETPH.Conclusion: The RVFAC was better correlated to RVEF than TAPSE in the groups with less severe and more severe hemodynamics. In patients with increased hemodynamic severity, with no difference in the performance in theHAP or CTEPH groups. RVFAC was a better index of RVEF possibly because it takes into account the transversal component of right ventricular function.

Objetivamos neste estudo avaliar de maneira quantitativa as alterações do parênquima e da vasculatura pulmonar em exames de angiotomografia computadorizada (angioTC) de pacientes com hipertensão pulmonar (HP), utilizando programa de análise totalmente automática. Foram analisados retrospectivamente os prontuários físicos e eletrônicos e os exames de angioTC de 45 pacientes com HP, em comparação com um grupo controle. Os pacientes com HP também foram divididos de acordo com a classificação de Nice, sendo representados principalmente pelos grupos 1 (hipertensão arterial pulmonar, incluindo a idiopática) e grupo 4 (hipertensão pulmonar secundária ao tromboembolismo crônico). A análise automática mostrou que os pacientes com HP apresentaram valores menores do percentil 10 (p10) da densidade dos pulmões e valores maiores de volume vascular do lobo superior direito (LSD) e de volume vascular relativo entre lobos superiores e inferiores. Neste estudo não encontramos diferenças da análise quantitativa por TC entre os grupos de pacientes com HP. Inferimos que a diferença nos valores de p10 indique a presença das áreas hipoatenuantes de oligoemia nos pacientes com HP, enquanto as diferenças nos volumes vasculares indiquem a redistribuição da trama vascular pulmonar. A análise quantitativa da vasculatura pulmonar em imagens de angioTC pode representar uma ferramenta diagnóstica objetiva na avaliação da HP. / In this study, we aimed to quantitatively analyze the lung parenchyma and pulmonary vasculature in computadorized tomography angiography (angioCT) from patients with pulmonary hypertension (PH), using a complete automated software. We retrospectively analyzed 45 patients\'s exams and clinical records, comparing with a control group. Patients were also divided in groups, according to Nice classification, represented mainly by group 1 (pulmonary arterial hypertension, including idiopathic) and 4 (chronic thromboembolic pulmonary hypertension). In patients with PH, the automated analysis found lower values of the 10th percentile (p10) of pulmonary density and higher values of vessel volumes in the upper right lobe (RUL) and higher relative vessel volumes between the uppers and lowers lobes. In this study, we didn\'t find any difference between the groups of patients with PH in the quantitative analysis of angioCT. We infer that the p10 differences indicate presence of hypoattenuating areas of olygoemia in patients with PH and that the difference in pulmonary vessel volumes indicates pulmonary vasculature redistribution. The analysis of pulmonary vessels in angioCT images can represent an objective diagnostic tool in PH evaluation.

As principais causas de morte perinatal em bezerros estão de alguma forma relacionadas a problemas cardiorrespiratórios. A maior parte de morte de bezerros ocorre justamente no período de até 48h após o nascimento, quando ainda estão sendo feitos os ajustes fisiológicos necessários à vida extrauterina. Com o aumento de animais advindos das técnicas de produção in vitro, também aumentaram os problemas de sobrevivência e sobrevida dos recém nascidos. Este estudo teve como objetivo geral padronizar valores de referência de hemogasometria e espirometria de bezerros a termo, nascidos por parto espontâneo, e prematuros, nascidos por cesariana, da raça Nelore concebidos por inseminação artificial (IA), assim como estabelecer protocolos de cuidados intensivos neonatais aos animais prematuros. Objetivamente, o estudo compreendeu: 1. A avaliação dos valores de pressão invasiva de átrio direito, ventrículo direito, artéria pulmonar e artéria pulmonar ocluída, hemogasometria, espirometria e capnografia de bezerros da raça Nelore, a termo, nascidos por parto espontâneo; 2. A avaliação e padronização da hemogasometria arterial de bezerros raça Nelore, a termo, nascidos de partos espontâneos; 3. A avaliação da pressão invasiva da artéria pulmonar e dos efeitos do sildenafil sobre os valores de pressão de artéria pulmonar em bezerros da raça Nelore, prematuros, nascidos por cesariana; 4. Diferenças hemodinâmicas (utilizando o cateter de Swan-Ganz), na função pulmonar e nas trocas gasosas entre bezerros a termo nascidos por parto espontâneo e prematuros nascidos por cesariana; 5. O estabelecimento de protocolos de cuidados neonatais para bezerros advindos de gestações complicadas com risco de morte neonatal prematura, onde foram realizadas 3 cesarianas em bezerros concebidos por IA e 16 concebidos por transferência nuclear por de células somática (clonagem), sendo estabelecidos protocolos de terapia neonatal com sildenafil, surfactante e oxigenoterapia. Bezerros da raça Nelore a termo nascidos por parto espontâneo estabilizam seus parâmetros de hemogasometria a valores de animais adultos em até 48 horas após o nascimento e têm valores de hemogasometria mais próximos dos valores normais para a espécie, enquanto os bezerros prematuros, nascidos por cesariana, têm valores mais deslocados em relação a normalidade. A padronização da hemogasometria de bezerros da raça Nelore nascidos por eutocia, pode fomentar as pesquisas com animais advindos de técnicas de PIV, assim como aumentar a sobrevida e sobrevivência além de diminuir a mortalidades desses animais. Já o sildenafil não apresenta efeitos adversos significativos quanto a oxigenação dos bezerros em que foi usado, mesmo quando houve alterações não desejadas da PAP. Dessa forma, quando não existe a possibilidade de se aferir a PAP e suspeita-se de hipertensão, o sildenafil é pode ser usado sem maiores consequências para a saúde do paciente. / The main causes of perinatal death in cattle are frequently related to cardiorespiratory distress. A great number of losses are placed during the first 48h after birth, when important physiological adjustments to the extra-uterine life are still in progress. Recent increase of offspring derived from assisted reproductive in vitro biotechnologies resulted in diminished survival rates of the newborn. The present study aimed to obtain standard values of hemodynamic evaluation, blood gas and spirometry of Nelore calves conceived by artificial insemination (AI) born at term, spontaneously, or from premature calves born by cesarean delivery, as well as to establish protocols of neonatal intensive care focusing on premature animals. Objectively, the study comprised: 1. The evaluation of invasive pressure values from right atrium, right ventricle, pulmonary artery and pulmonary wedge pressure, blood gas, spirometry and capnography of Nelore calves born spontaneously; 2. Evaluation and standardization of arterial blood gas of Nelore calves born at term spontaneously; 3. Evaluation of invasive pressure of pulmonary artery (PAP) and effects of sildenafil administration on its values from Nelore premature calves born by cesarean; 4. The comparison of hemodynamic, blood gas and spirometry of calves born at term spontaneously or premature calves born by cesarean delivery, during the first 30 days of life; 5. The establishment of neonatal care protocols focusing on complicated pregnancies at risk of premature neonatal death, when 3 cesarean sections of calves conceived by AI and 16 conceived by nuclear transfer of somatic cells (cloning) were monitored, and therefore protocols involving neonatal therapy with sildenafil, surfactant and oxygen therapy were established. Nelore calves born at term spontaneously have their blood gas stabilized in values similar to adult cattle until 48 hours after birth, and present a blood gas profile more similar to the expected for the species, whereas premature calves born from cesarean delivery present values different from normality. The standardization of blood gas of Nelore calves born at term from eutocic deliveries may promote better perinatal survival rates in offspring derived from in vitro production. The treatment with sildenafil does not present adverse effects related to the oxygen levels of calves even when adverse alterations were observed in PAP. Therefore, whenever hypertension is suspected and measurement of PAP is impaired, the sildenafil treatment may be used without major consequences to the patient health

Introdução: A fração de ejeção do ventrículo direito (FEVD) é um importante fator prognóstico em pacientes com hipertensão pulmonar (HP), porém a sua medida é complicada e demorada devido à complexidade anatômica do ventrículo direito (VD). O TAPSE (Tricuspid Annular Plane Systolic Excursion) é um bom índice da FEVD, mas ele avalia apenas o componente longitudinal da contração ventricular direita. A RVFAC (Right Ventricular Fractional Area Change) parece ser um melhor índice da FEVD por incluir os componentes longitudinal e transversal da contração ventricular direita. O objetivo deste estudo foi avaliar a performance da RVFAC de acordo com a gravidade do acometimento hemodinâmico em dois grupos distintos de pacientes portadores de HP pré-capilar: hipertensão arterial pulmonar (HAP) e tromboembolismo pulmonar crônico hipertensivo (TEPCH). Métodos: 62 pacientes realizaram cateterismo cardíaco direito e ressonância magnética cardíaca em ±72h. As áreas sistóica e diastólica finais do ventrículo direito (ASFVD, ADFVD), a área diastólica final do ventrículo esquerdo (ADFVE) e o TAPSE foram medidos nas imagens de quatro cavidades. A RVFAC (ADFVD-ASFVD/ADFVD) e a relação entre as áreas diastólica finais ventriculares (ADFVD/ADFVE) foram calculadas. Os diâmetros entre as paredes livre e septal (dL-S) e antero-posterior (dA-P) do ventículo esquerdo (VE) foram medidos nas imagens em eixo curto e o índice de excentricidade do VE (IE) foi calculado (=dA-P/dL-S). A FEVD foi calculada a partir de imagens consecutivas de 6mm no eixo curto. . Resultados: A população tinha 58 anos em média, a maioria era do sexo feminino e estava em classe funcional III, 23 tinham HAP e 39 TEPCH. A FEVD apresentou correlações fracas com as medidas hemodinâmicas de sobrecarga e de função do VD. A RVFAC apresentou melhor correlação (R2=0,65, p < 0,001) do que o TAPSE (R2=0,35, p<0,001) com a FEVD e melhor capacidade para estimar FEVD<35% do que o TAPSE (TAPSE: AUC 0,73 e RVFAC: AUC 0,93, p=0,0065). Dividimos a população pela mediana da resistência vascular pulmonar (RVP) e observamos que no grupo com maior gravidade hemodinâmica essa diferença se acentuou: no grupo com RVP<8,5UW (RVFAC: R2=0,66, p<0,001 e TAPSE: R2=0,30, e p=0,002) e no grupo com RVP>8,5UW (RVFAC: R2=0,51, p<0,001 e TAPSE: R2=0,14, e p=0,041). O grupo com RVP>8,5UW apresentou maior ADFVD/ADFVE e maior IE. As correlações da RVFAC e TAPSE com FEVD foram semelhantes entre os grupos HAP e TEPCH. Conclusão: A RVFAC se correlacionou melhor com a FEVD do que o TAPSE tanto no grupo com menor como no grupo com maior gravidade hemodinâmica. No grupo com maior gravidade as correlações da RVFAC com a FEVD foram ainda mais significativas, não havendo diferenças na performance da RVFAC entre os pacientes com HAP e TEPCH. A RVFAC foi um melhor índice da FEVD talvez por incluir o movimento transversal da contração ventricular / Introduction: The right ventricular ejection fraction (RVEF) is a surrogate marker in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The TAPSE (Tricuspid Annular Plane Systolic Excursion) is a good index of RVEF, though it measures only the longitudinal component of right ventricular contraction. The RVFAC (Right Ventricular Fractional Area Change) seems to be a better index of RVEF because it takes into account the longitudinal and the transversal components of right ventricular contraction. The aim of our study was to evaluate the RVFAC performance according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methos: Sixty-two patients with PAH and CTEPH underwent right heart catheterization and cardiac MR in a 72-hour delay. The right and left ventricle end diastolic areas (RVEDA, LVEDA), the right ventricle end systolic area (RVESA) and TAPSE were measured in the four chamber view. The RVFAC (=RVEDARVESA/RVEDA) and the RVEDA/LVEDA relationship were calculated. The diameter between the left ventricle (LV) free wall and the septum (dF-S) and the diameter between the LV anterior and posterior walls (dA-P) were measured and the LV eccentricity index (EI) was calculated (=dA-P/dF-S). The RVEF was calculated by using 6 mm RV short axis cines. Results: The population had mean age of 58 years with female majority, most of the patients were in functional class III, 23 had PAH and 39 CTEPH. The RVEF was weakly correlated to the hemodynamic variables of RV afterload and function. The RVFAC was more strongly correlated to RVEF (R2=0.65, p<0.001) than TAPSE (R2=0.35, p<0.001). RVEF<35% was better predicted by RVFAC than TAPSE (TAPSE: AUC 0.73 and RVFAC: AUC 0.93, p=0.0065). We divided the population by the median of the pulmonary vascular resistance (PVR) and we observed that in the group with worse hemodynamic severity this difference increased: in the group with PVR<8,5WU (RVFAC: R2=0.66, p<0.001 and TAPSE: R2=0.30, p=0.002) and in the group with PVR>8,5 WU (RVFAC: R2=0.51, p<0.001 and TAPSE: R2=0.14, p=0.041). The group with PVR>8,5WU had an increased RVEDA/LVEDA and an increased EI. There was no differences in the RVEF relationships between the groups of PAH and CETPH. Conclusion: The RVFAC was better correlated to RVEF than TAPSE in the groups with less severe and more severe hemodynamics. In patients with increased hemodynamic severity RVFAC perfomed even better, there was no difference in the performance of RVFAC in PAH or CTEPH. RVFAC was a better index of RVEF possibly because it takes into account the transversal component of right ventricular function

INTRODUÇÃO: As Hemoglobinopatias podem ser divididas em Talassemias e Doença Falciforme (DF), mas do ponto de vista clínico, ambas apresentam um quadro de anemia hemolítica crônica, o que acarreta uma série de complicações, entre estas a Hipertensão Pulmonar (HP). Estima-se que cerca de 20-40% da população com DF/talassemia apresente HP, sendo que este diagnóstico está associado a uma elevada morbi-mortalidade. Poucos estudos avaliaram esta prevalência em crianças. O Objetivo deste estudo foi avaliar a prevalência desta complicação na população pediátrica, e associá-la com características clínicas e laboratoriais. MÉTODOS: Estudo de Corte Transversal, com avaliação de 45 pacientes com diagnóstico de DF ou Talassemia maior/ intermédia entre 3-18 anos, atendidos de forma consecutiva no ambulatório de Hemoglobinopatias do HCPA. Os pacientes foram submetidos a um ecocardiograma para estimativa da pressão sistólica da artéria pulmonar, sendo que foi considerado como tendo risco de HP os pacientes com velocidade de regurgitação tricúspide (VRT) ≥ 2,5m/s. Foram obtidos dados clínicos e laboratoriais para avaliação dos parâmetros hemolíticos, função hepática e renal por levantamento de prontuário e comparados os grupos. RESULTADOS: 15% (6/40) dos pacientes apresentaram VRT ≥ 2,5m/s, sugestivo de HP, sendo que destes pacientes todos tinham diagnóstico de Anemia Falciforme (AF). Considerando apenas esta população, a prevalência de HP aumenta para 20% (6/30). A população com VRT ≥ 2,5m/s apresentou média de idade mais elevada, Hb mais baixa, RDW mais alargado, reticulócitos e LDH mais elevado que o grupo com VRT < 2,5m/s. A principal intercorrência clínica nesta população foi a ocorrência de priapismo (p< 0,05). CONCLUSÕES: Os pacientes com Hemoglobinopatias estão em risco aumentado para desenvolvimento de HP desde a infância, principalmente aqueles com AF. Estes pacientes apresentam os parâmetros laboratoriais sugestivos de hemólise alterados, assim como outros sintomas associados ao quadro hemolítico como o priapismo quando comparados com pacientes com VRT normal. Desta forma sugere-se a realização de triagem com ecocardiograma nesta população de forma precoce. / INTRODUCTION: The Hemoglobinopathies can be divided in Thalassemias and Sickle Cell Disease (SCD), but clinically both present with chronic hemolytic anemia, which leads to various complications, one of them being Pulmonary Hypertension (PH). About 20-40% of patients with SCD have PH, and this diagnosis is associated with a high risk of mortality. The objective of this study was to estimate the prevalence of this complication in the pediatric population, and associate clinical and laboratory characteristics. METHODS: A cross sectional descriptive study, with the evaluation of 45 patients with diagnosis of SCD or thalassemia major/intermedia between 3-18 years, which received treatment at the Hemoglobinopathies ambulatory at HCPA. The patients were submitted to an echocardiogram to estimate the pulmonary artery systolic pressure, being considered to have PH patients with a tricuspid regurgitate jet velocity (TRV) ≥ 2.5m/s. Clinical and laboratory data were obtained to evaluate hemolytic parameters, renal and liver function and compared between groups. RESULTS: 15% (6/40) of patients had a TRV ≥ 2.5m/s, suggestive of PH, of which all had Sickle Cell Anemia (SCA). Considering this group of patients alone the prevalence would be of 20% (6/30). Patients with TRV ≥ 2.5m/s had a higher median age, lower hemoglobin count, higher RDW, reticulocyte and DHL then patients with a TRV < 2.5m/s. The major clinical feature was the occurrence of priapism (p<0,05). CONCLUSIONS: Patients with diagnosis of hemoglobinopathies are at higher risk of developing PH since early childhood, especially those with SCA. These patients showed a higher level of hemolytic parameters, as well as symptoms associated with hemolysis, like priapism, when compared with patients with a normal TRV. Therefore, it would be indicated to submit these patients to an echocardiogram routinely in their early years.

As principais causas de morte perinatal em bezerros estão de alguma forma relacionadas a problemas cardiorrespiratórios. A maior parte de morte de bezerros ocorre justamente no período de até 48h após o nascimento, quando ainda estão sendo feitos os ajustes fisiológicos necessários à vida extrauterina. Com o aumento de animais advindos das técnicas de produção in vitro, também aumentaram os problemas de sobrevivência e sobrevida dos recém nascidos. Este estudo teve como objetivo geral padronizar valores de referência de hemogasometria e espirometria de bezerros a termo, nascidos por parto espontâneo, e prematuros, nascidos por cesariana, da raça Nelore concebidos por inseminação artificial (IA), assim como estabelecer protocolos de cuidados intensivos neonatais aos animais prematuros. Objetivamente, o estudo compreendeu: 1. A avaliação dos valores de pressão invasiva de átrio direito, ventrículo direito, artéria pulmonar e artéria pulmonar ocluída, hemogasometria, espirometria e capnografia de bezerros da raça Nelore, a termo, nascidos por parto espontâneo; 2. A avaliação e padronização da hemogasometria arterial de bezerros raça Nelore, a termo, nascidos de partos espontâneos; 3. A avaliação da pressão invasiva da artéria pulmonar e dos efeitos do sildenafil sobre os valores de pressão de artéria pulmonar em bezerros da raça Nelore, prematuros, nascidos por cesariana; 4. Diferenças hemodinâmicas (utilizando o cateter de Swan-Ganz), na função pulmonar e nas trocas gasosas entre bezerros a termo nascidos por parto espontâneo e prematuros nascidos por cesariana; 5. O estabelecimento de protocolos de cuidados neonatais para bezerros advindos de gestações complicadas com risco de morte neonatal prematura, onde foram realizadas 3 cesarianas em bezerros concebidos por IA e 16 concebidos por transferência nuclear por de células somática (clonagem), sendo estabelecidos protocolos de terapia neonatal com sildenafil, surfactante e oxigenoterapia. Bezerros da raça Nelore a termo nascidos por parto espontâneo estabilizam seus parâmetros de hemogasometria a valores de animais adultos em até 48 horas após o nascimento e têm valores de hemogasometria mais próximos dos valores normais para a espécie, enquanto os bezerros prematuros, nascidos por cesariana, têm valores mais deslocados em relação a normalidade. A padronização da hemogasometria de bezerros da raça Nelore nascidos por eutocia, pode fomentar as pesquisas com animais advindos de técnicas de PIV, assim como aumentar a sobrevida e sobrevivência além de diminuir a mortalidades desses animais. Já o sildenafil não apresenta efeitos adversos significativos quanto a oxigenação dos bezerros em que foi usado, mesmo quando houve alterações não desejadas da PAP. Dessa forma, quando não existe a possibilidade de se aferir a PAP e suspeita-se de hipertensão, o sildenafil é pode ser usado sem maiores consequências para a saúde do paciente. / The main causes of perinatal death in cattle are frequently related to cardiorespiratory distress. A great number of losses are placed during the first 48h after birth, when important physiological adjustments to the extra-uterine life are still in progress. Recent increase of offspring derived from assisted reproductive in vitro biotechnologies resulted in diminished survival rates of the newborn. The present study aimed to obtain standard values of hemodynamic evaluation, blood gas and spirometry of Nelore calves conceived by artificial insemination (AI) born at term, spontaneously, or from premature calves born by cesarean delivery, as well as to establish protocols of neonatal intensive care focusing on premature animals. Objectively, the study comprised: 1. The evaluation of invasive pressure values from right atrium, right ventricle, pulmonary artery and pulmonary wedge pressure, blood gas, spirometry and capnography of Nelore calves born spontaneously; 2. Evaluation and standardization of arterial blood gas of Nelore calves born at term spontaneously; 3. Evaluation of invasive pressure of pulmonary artery (PAP) and effects of sildenafil administration on its values from Nelore premature calves born by cesarean; 4. The comparison of hemodynamic, blood gas and spirometry of calves born at term spontaneously or premature calves born by cesarean delivery, during the first 30 days of life; 5. The establishment of neonatal care protocols focusing on complicated pregnancies at risk of premature neonatal death, when 3 cesarean sections of calves conceived by AI and 16 conceived by nuclear transfer of somatic cells (cloning) were monitored, and therefore protocols involving neonatal therapy with sildenafil, surfactant and oxygen therapy were established. Nelore calves born at term spontaneously have their blood gas stabilized in values similar to adult cattle until 48 hours after birth, and present a blood gas profile more similar to the expected for the species, whereas premature calves born from cesarean delivery present values different from normality. The standardization of blood gas of Nelore calves born at term from eutocic deliveries may promote better perinatal survival rates in offspring derived from in vitro production. The treatment with sildenafil does not present adverse effects related to the oxygen levels of calves even when adverse alterations were observed in PAP. Therefore, whenever hypertension is suspected and measurement of PAP is impaired, the sildenafil treatment may be used without major consequences to the patient health

<p>Nitric oxide (NO) is a mediator of vascular smooth muscle tension that metabolises rapidly in blood. NO delivered by inhalation can therefore be used as a selective pulmonary vasodilator to relieve pulmonary hypertension or to improve oxygenation with no systemic effects. In artificial ventilation nitric oxide has been administered in inspiration gas as a continuous gas flow or to form constant inspired concentration. Homogeneous inspired gas mixture has been regarded essential for successful therapy and the therapy has been characterized by the mixture NO concentration. The response in oxygenation on NO therapy has, however, been variable. Administration of NO as a short pulse synchronously with inspiration has been suggested to improve the response. In this study the NO administration was examined theoretically and experimentally with the aim to relieve pulmonary hypertension and improve oxygenation during artificial ventilation. For the experimental study a system for the synchronized administration was developed.</p><p>The effect on oxygenation was studied during equine anaesthesia where hypoxemia develops regularly secondary to left-to-right shunt caused by atelectasis. By administering the NO as a short pulse in early inspiration to well ventilated lung areas the oxygenation could be effectively improved. Delayed administration to low ventilated lung areas was found possible for a negative contribution on oxygenation, which reduces the improvement gained in the well-ventilated lung areas. When NO is delivered into the whole inspiration, the net effect on oxygenation is the sum of these negative and positive contributions, whereas with pulsed delivery to the early inspiration the negative contribution can be avoided. This finding may be the main explanation for the varying response in oxygenation detected in patients as a response to NO inhalation.</p><p>When the NO therapy aimed for the relief of induced pulmonary hypertension in pigs, no difference was observed between NO delivery as a short pulse or given to the whole inspiration. Maximum vasodilatation was observed with 105 nmol/min delivery rate. A larger delivery rate only contributed to an abrupt increase in pulmonary pressure at cessation of the delivery.</p><p>The NO uptake from alveoli to tissue depends on the alveolar NO partial pressure. In a simulation this partial pressure was shown to be independent of the administration mode. Also the relationship between the NO uptake and delivery setting was not explicit. With pulsed delivery, expired NO can be reduced which was confirmed by the experimental results. This is important when the NO therapy is given in rebreathing circuit.</p>

<p>Chronic obstructive pulmonary disease (COPD) is in up to 90 % of all cases caused by</p><p>smoking. COPD often has negative effects on circulation, effects that first and foremost can be</p><p>observed as respiratory insufficiency. Reduced function of the right ventricle of the heart is</p><p>common in patients suffering from chronic obstructive pulmonary disease, especially if they</p><p>also have hypoxemi; insufficient levels of oxygen in blood or tissue. The incidence of this</p><p>cardiac complication reduces the survival time. It is possible in chronic obstructive pulmonary</p><p>disease that the pressure in the pulmonary circulation gradually increases resulting in</p><p>pulmonary hypertension followed by a slow adaptation of the right ventricle by hypertrophy of</p><p>the myocardium.</p><p>To investigate a correlation between COPD and pulmonary hypertension COPD patients</p><p>were subjected to spirometry and ultrasound on heart.</p><p>Of 14 examined patients 5 had developed pulmonary hypertension. A correlation between</p><p>obstruction in the COPD- patients and an increase in left ventricular diameter was found.</p><p>DLCO (diffusion capacity) of the lungs is directly connected to PA (pulmonary arterial</p><p>pressure). The lower DLCO, the higher risk to develop pulmonary hypertension. However, we</p><p>could not find a significant correlation between COPD and pulmonary hypertension in this</p><p>study even if most patients had a decreased DLCO.</p>