Correlação entre medidas ecocardiográficas e hemodinâmicas na avaliação do átrio direito e ventrículo direito em pacientes com hipertensão pulmonar / Correlation between echocardiographic and hemodynamic measurements in right atrium and right ventricle assessments of patients with pulmonary hypertension

Introdução: Hipertensão Pulmonar (HP), uma condição clínica grave, pode levar à disfunção sistólica do ventrículo direto (DSVD), com implicações prognósticas. Pacientes com suspeita de HP devem ser submetidos ao ecocardiograma transtorácico (ECOTT) para diagnóstico e avaliação, colocando-o como o principal exame de triagem e acompanhamento. Objetivo: Verificar a associação e a concordância das medidas referentes à pressão média no átrio direito (AD) e à disfunção sistólica do ventrículo direto (DSVD) ao (ECOTT) e ao cateterismo de câmaras direitas (CCD) em pacientes com (HP). Métodos: Foram incluídos indivíduos com diagnóstico de (HP). Todos os pacientes foram submetidos ao ECOTT e CCD. Avaliou-se pelo ECOTT: área do átrio direito (AAD), pressão média do átrio direito (AD) através por meio do diâmetro e da colapsabilidade da veia cava inferior (PMADECOTT ), strain AD (SAD), TAPSE (excursão sistólica do plano anular tricúspide), MAF (mudança da área fracional), SPLVD (strain da parede livre do VD) e onda s´ tricuspídea. Pelo CCD avaliaram-se pressão média do (PMADCCD ) e índice cardíaco (IC). Resultados: Dos 16 pacientes, 13 eram do sexo feminino. A idade média foi de 44,4 anos (±14,9). Constataram-se associação entre pressão média do átrio direito PMADCCD com área do átrio direito, PMADECOTT pressão média do átrio direito e SAD strain do átrio direito (r=0,845, r=0,621 e r=-0,523, respectivamente; p< 0,05). Verificou-se associação entre as categorias de risco de mortalidade, mensuradas pelas medidas AAD da área do átrio direito e pressão média do átrio direito PMADCCD (X2=10,42; p=0,003), com concordância moderada (k=0,44; p=0,012). DSVD A disfunção sistólica do ventrículo direto estava presente em dez pacientes. Houve associação entre disfunção sistólica do ventrículo direto DSVD (presente ou ausente) e índice cardíaco IC (r=0,522; p=0,04), com concordância moderada (k=0,43; p=0,037). Conclusão: As medidas do ecocardiograma transtorácico (ECOTT) e cateterismo de câmara direita (CCD) demostraram associação na avaliação da pressão média do átrio direito com melhor associação entre área do átrio direito AAD e pressão média do átrio direito (PMADCCD) . Houve associação com concordância moderada quanto à disfunção sistólica do ventrículo direto (DSVD) entre métodos. (AU)

Introduction: Pulmonary hypertension (PH), a serious clinical condition, can lead to right ventricular systolic dysfunction (RVSD) with prognostic implications. Patients with suspected PH should undergo transthoracic echocardiography (TTE) for diagnosis and evaluation as the main screening and follow-up exam. Objective: To verify the associations of and agreement between measurements of mean pressure in the right atrium (RA) and RVSD with TTE Method: Individuals diagnosed with PH were included. All patients underwent TTE and RCC. The following were evaluated by TTE: right atrial area (RAA), mean right atrial pressure through the diameter and collapsibility of the inferior vena cava (RMAPTTE), RA strain (RAS), tricuspid annular plane systolic excursion, fractional area change, RV free wall strain, and tricuspid s' wave. Mean RA pressure (RMAPRCC) and cardiac index (CI) were evaluated through the RCC. Results: Of the 16 patients, 13 were female. The mean patient age was 44.4 (±14.9) years. An association was found between RMAPRCC and AAD, RMAPTTE, and RAS (r=0.845, r=0.621, and r=-0.523, respectively; p<0.05). There was an association between the mortality risk categories measured by the RAA and RMAPRCC measures (X2=10.42; p=0.003), with moderate agreement (k=0.44; p=0.012). RVSDJ was present in 10 patients. There was an association between RVSD (present or absent) and CI (r=0.522; p=0.04) with moderate agreement (k=0.43; p=0.037). Conclusion: The TTE and RCC measurements showed an association in the assessment of mean right atrial pressure, especially between RAA and RMAPRCC. An association with RVSD and moderate agreement between methods were also noted. (AU)

Uso de sildenafil para la hipertensión pulmonar en neonatos / Use of sildenafil for pulmonary hypertension in neonates

Resumen La hipertensión arterial pulmonar es una enfermedad multifactorial que incrementa la mortalidad en el neonato como consecuencia de falla cardiaca. Los vasodilatadores pulmonares son la piedra angular del tratamiento, de los cuales el sildenafil es el fármaco más empleado. A continuación, se resumen los resultados de una revisión sistemática Cochrane en la que se evaluaron la eficacia y la seguridad del sildenafil para el tratamiento de la hipertensión arterial pulmonar en neonatos.

Abstract Pulmonary arterial hypertension is a multifactorial nosological entity that increases neonatal mortality as a result of heart failure. Pulmonary vasodilators are the cornerstone of treatment, of which sildenafil is the most commonly used drug. Therefore, the results of a recently updated Cochrane systematic review are summarized, in which the efficacy and safety of sildenafil for the treatment of pulmonary hypertension in neonates was evaluated.

Avaliação ecocardiográfica da hipertensão pulmonar em crianças / Echocardiographic evaluation of pulmonary hypertension in children

A doença pulmonar hipertensiva pode ser definida como um conjunto de alterações fisiopatológicas pulmonares que resultam em uma patologia grave, progressiva e com alta morbimortalidade. O ecocardiograma transtorácico é um método de imagem de fácil acesso e essencial para avaliação desta doença, principalmente na faixa pediátrica, na qual há limitações para realização frequente e de rotina do cateterismo direito. Nesta revisão, abordaremos as principais técnicas ecocardiográficas para o diagnóstico e a avaliação hemodinâmica da hipertensão pulmonar na população pediátrica. O diagnóstico precoce e o adequado estadiamento no acompanhamento das intervenções clínicas são fundamentais para escolha assertiva da abordagem terapêutica e, consequentemente, melhora do desfecho clínico

Sobrevida a mediano plazo en los pacientes con hipertensión arterial pulmonar en la era de terapias vasodilatadoras específicas del territorio vascular pulmonar / Survival of patients with pulmonary arterial hypertension after the advent of specific pulmonary vasodilator therapies

Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disease. Long-term survival remains poor despite of advances in specific vasodilator therapy. Aim: To describe the survival rate in a cohort of PAH patients in two referral centers in Chile. Patients and Methods: One hundred fifteen patients aged 43 ± 15.6 years (85% females) with PAH qualified for this study. Their median pulmonary artery pressure was 55.4 ± 14 mmHg and their six minutes walking capacity was 368 ± 119 m. They were followed for 58 ± 0.4 months and their actual survival rates were compared with the estimated survival using the equation proposed by the French registry of PAH. Results: One, two and three year survival rates were 97, 94 and 89%, respectively. The observed survival rates were greater than the estimated survival. Conclusions: The improvement in survival rates observed in this cohort of patients is similar to what has been described in literature.

Impacto da Hipertensão Pulmonar na Mortalidade Cirúrgica e na Sobrevida em Três Anos Pós-Cirurgia de Troca Valvar Aórtica / Impact of Pulmonary Hypertension on Surgical Mortality and 3-Year Survival After Aortic Valve Replacement

A hipertensão pulmonar (HP) grave contribui para significante morbidade e mortalidade, e podeaumentar o número de complicações e morte na cirurgia cardíaca. Objetivo: Avaliar o impacto da HP na mortalidade cirúrgica e na sobrevida em três anos, após cirurgia de troca valvar aórtica.Métodos: Estudo de coorte retrospectiva realizado com pacientes submetidos à cirurgia de troca valvar aórtica, demaio de 2011 a dezembro de 2012, no Instituto Nacional de Cardiologia, RJ. A população estudada foi estratificadaem dois grupos, de acordo com a pressão sistólica da artéria pulmonar (PSAP) em: <45 mmHg e ≥45 mmHg. Foi utilizado o teste do qui-quadrado para a comparação entre os grupos; a mortalidade e cirúrgica foi estudada por regressão logística e a sobrevida pela curva de Kaplan-Meier e teste de log-rank; e a razão de risco pelo método de Cox. Resultados: Estudados 182 pacientes, 57,0% do sexo masculino, idade 61,0±13,0 anos, com hipertensão arterial sistêmica (72,0%), diabetes mellitus (22,0%), classe funcional pela New York Heart Association III/IV (61,1%), PSAP≥45mmHg (14,3%). A mortalidade geral foi 12,0%. A sobrevida foi maior no grupo com PSAP <45mmHg – 89,0%em um ano e 86,0% em três anos, comparado com o grupo com PSAP ≥45mmHg – 68,8% em um ano e 58,1%(35,1% a 75,4%) em três anos (p=0,0004). Conclusão: Pacientes com PSAP ≥45mmHg apresentaram maior mortalidade cirúrgica e pior sobrevida em três anos após cirurgia de troca valvar aórtica...

Background: Severe pulmonary hypertension (PH) contributes to significant morbidity and mortality and may increase the number of complications and death in heart surgery.Objective: To evaluate the impact of PH on surgical mortality and three-year survival after aortic valve replacement surgery. Methods: Retrospective cohort study of patients undergoing aortic valve replacement surgery from May 2011 to December 2012 at Instituto Nacional de Cardiologia, RJ. The study population was stratified into two groups according to the systolic pulmonary artery pressure (SPAP): <45mmHg and ≥45mmHg. The chi-square test was used for comparison between groups; surgical mortality was studied by logistic regression and survival by Kaplan-Meier method and log-rank test; the hazard ratio was examined using the Cox method. Results: The study included 182 patients, 57.0% were males aged 61.0±13.0 years, with systemic arterial hypertension (72.0%), diabetes mellitus (22.0%), functional class by the New York Heart Association III/IV (61.1%), SPAP ≥45mmHg (14.3%). Overallmortality rate was 12.0%. Survival rate was higher in the group with SPAP <45mmHg — 89.0% at one year and 86.0% at three year compared with the group with SPAP ≥45mmHg — 68.8% at one year and 58.1% (35.1% to 75.4%) at three years (p=0.0004). Conclusion: Patients with SPAP ≥45mmHg had higher surgical mortality and worse survival at three years after aortic valvereplacement surgery...

Histidine-tryptophan-ketoglutarate solution decreases mortality and morbidity in high-risk patients with severe pulmonary arterial hypertension associated with complex congenital heart disease: an 11-year experience from a single institution

Cardioplegic reperfusion during a long term ischemic period interrupts cardiac surgery and also increases cellular edema due to repeated solution administration. We reviewed the clinical experiences on myocardial protection of a single perfusion with histidine-tryptophan-ketoglutarate (HTK) for high-risk patients with severe pulmonary arterial hypertension associated with complex congenital heart disease. This retrospective study included 101 high-risk patients undergoing arterial switch operation between March 2001 and July 2012. We divided the cohort into two groups: HTK group, myocardial protection was carried out with one single perfusion with HTK solution; and St group, myocardial protection with conventional St. Thomas' crystalloid cardioplegic solution. The duration of cardiopulmonary bypass did not differ between the two groups. The mortality, morbidity, ICU stay, post-operative hospitalization time, and number of transfusions in HTK group were lower than those in St group (P<0.05). Univariate and multivariate analysis showed that HTK is a statistically significant independent predictor of decreased early mortality and morbidity (P<0.05). In conclusion, HTK solution seems to be an effective and safe alternative to St. Thomas' solution for cardioplegic reperfusion in high-risk patients with complex congenital heart disease.

Pulmonary hypertension due to acute respiratory distress syndrome

Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS), to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46%) who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%). The most common cause of ARDS was pneumonia (56.3%). The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

Effect of pulmonary hypertension on outcome of pulmonary tuberculosis

Background: This study performed at the National Research Institute of Tuberculosis and Lung Disease, Tehran, Iran, aimed to evaluate the effect of concomitant pulmonary hypertension on the outcome of pulmonary tuberculosis. Methods: New cases of pulmonary tuberculosis were recruited for the study. Pulmonary hypertension was defined as systolic pulmonary arterial pressure ≥35 mm Hg estimated by transthoracic Doppler echocardiography. We assessed the relationship between pulmonary hypertension and mortality during the six-month treatment of tuberculosis. Results: Of 777 new cases of pulmonary tuberculosis, 74 (9.5%) had systolic pulmonary arterial pressure ≥35 mm Hg. Ten of them (13.5%) died during treatment compared to 5% of cases with pulmonary arterial pressure less than 35 mm Hg (p = 0.007). Logistic regression analysis showed that pulmonary hypertension and drug abuse remained independently associated with mortality (OR = 3.1; 95% CI: 1.44–6.75 and OR = 4.4; 95% CI: 2.35–8.17, respectively). Conclusion: A significant association was found between mortality and presence of pulmonary hypertension and drug abuse among new cases of pulmonary tuberculosis. .

Hipertensión pulmonar asociada a HIV / HIV-associated pulmonary hypertension

La hipertensión pulmonar (HP) es una patología grave, que si bien es infrecuente, en los pacientes HIV+ se presenta hasta 12 veces más que en la población general; su diagnóstico precoz, en pacientes asintomáticos o con síntomas leves brinda la posibilidad de un tratamiento específico que mejora sustancialmente el pronóstico en estos pacientes. Si bien la fisiopatología de la HP asociada a HIV permanece desconocida y se considera de origen ultifactorial, existe evidencia de que distintas proteínas virales juegan un importante rol en su génesis. En todo paciente HIV+ con disnea a mínimos esfuerzos se debe descartar junto con otras etiologías la HP. el método de screening inicial es el ecodoppler cardíaco y la confirmación se realiza mediante cateterización cardiaca derecha. Es imprescindible tanto el tratamiento específico como la instauración precoz del TARV al diagnóstico, como así manejarse en conjunto con equipos con experiencia en esta patología...

Pulmonary hypertension (PH) is a serious condition, although it is uncommon in HIV + patients presented up to 12 times more than in the general population; its early diagnosis in asymptomatic or mildly symptomatic offers the possibility of a treatment specific substantially improve prognosis in these patients. Although the pathophysiology of pulmonary hypetension associated with HIV remains unknown and is considered multifactorial, there is evidence that different viral proteins play an important role in its genesis. In any patient with dyspnea on minimal exertion should be discarded along with other etiologies of PH. The initial screening method is the heart doppler and confirmation is donde by right heart catheterization. It is essential to both the specific treatment as early institution of ART at diagnosis; as well it handled in conjuntion with experienced teams in this pathology...

Plasma von Willebrand factor as a predictor of survival in pulmonary arterial hypertension associated with congenital heart disease

Biomarkers have been identified for pulmonary arterial hypertension, but are less well defined for specific etiologies such as congenital heart disease-associated pulmonary arterial hypertension (CHDPAH). We measured plasma levels of eight microvascular dysfunction markers in CHDPAH, and tested for associations with survival. A cohort of 46 inoperable CHDPAH patients (age 15.0 to 60.2 years, median 33.5 years, female:male 29:17) was prospectively followed for 0.7 to 4.0 years (median 3.6 years). Plasma levels of von Willebrand factor antigen (VWF:Ag), tissue plasminogen activator (t-PA) and its inhibitor (PAI-1), P-selectin, reactive C-protein, tumor necrosis factor alpha, and interleukin-6 and -10 were measured at baseline, and at 30, 90, and 180 days in all subjects. Levels of six of the eight proteins were significantly increased in patients versus controls (13 to 106 percent increase, P < 0.003). Interleukin-10 level was 2.06 times normal (P = 0.0003; Th2 cytokine response). Increased levels of four proteins (t-PA, PAI-1, P-selectin, and interleukin-6) correlated with disease severity indices (P < 0.05). Seven patients died during follow-up. An average VWF:Ag (mean of four determinations) above the level corresponding to the 95th percentile of controls (139 U/dL) was independently associated with a high risk of death (hazard ratio = 6.56, 95 percentCI = 1.46 to 29.4, P = 0.014). Thus, in CHDPAH, microvascular dysfunction appears to involve Th2 inflammatory response. Of the biomarkers studied, plasma vWF:Ag was independently associated with survival.

Cambios en el pronóstico a largo plazo de la hipertensión arterial pulmonar / Changes long term prognosis of 17 patients with pulmonary artery hypertension

Background: Pulmonary artery hypertension (PAH) is a progressive disease with high mortality. Major advances had been made in the treatment of this condition during the last decade. Aim: To characterize the clinical evolution and mortality of a cohort of Chilean patients. Material and Methods: Seventeen patients with PAH diagnosed in the last 10 years in two Chilean hospitals were enrolled. Measurements at diagnosis included hemodynamic variables and 6-minute walk test. The patients were followed clinically for 3 years and the observed mortality was compared with that predicted by the prognostic equation proposed by the historic registry of the National Institutes of Health (NIH). Results: The mean age of patients was 45 years and 80 percent had an idiopathic PAH. The mean median pulmonary artery pressure was 57 ± 15 mmHg, the cardiac index was 2.4 ± 0.7 l/min/m² and the right atrial pressure was 12 ± 8 mmHg. The 6-minute walk distance was 348 ± 98 m. All patients received anticoagulants. Eighty two percent received ambrisentan, 12 percent received bosentan, 29 percent received iloprost and 24 percent sildenafil. At the end of follow-up only 3 patients had died, with an observed survival rate of88, 82 and 82 percent at 1, 2 and 3 years, respectively. In contrast, the survival calculated according to the predictive formula of the NIH was 67, 56 and 45 percent, respectively. Among surviving patients, an improvement in exercise capacity was observed after one year (p < 0.05). Conclusions: The observed survival rate was significantly better than that estimated according to historical data. Furthermore, therapy was associated with an improvement in functional capacity after one year. This prognostic improvement is consistent with data of other contemporary registries published after the NIH Registry.

Sarcoidosis: Presentación clínica y pronóstico / Sarcoidosis: Clinical presentation and prognosis

Se analizaron las características clínicas de 26 pacientes con cuadro clínico compatible y diagnóstico histopatológico de sarcoidosis, y se identificaron variables asociadas a mortalidad en el seguimiento. Se examinaron datos clínicos y de diversos estudios complementarios. El seguimiento se realizó mediante consulta médica y encuesta telefónica. La edad media fue 42.6 ± 12.7 años. El 53.8% eran mujeres. El 88.4% presentó afección pulmonar y el 30.7% tuvo manifestaciones extratorácicas. Predominó el estadio radiológico II (34.7%). La alteración espirométrica más frecuente fue la capacidad de difusión de monóxido de carbono (DLCO) < 80% del predicho (56.5%). Se constató hipertensión pulmonar en el 34.7%. Recibieron tratamiento corticoideo el 69.2% de los pacientes. El seguimiento se realizó en el 96.1% de los casos durante 98 ± 73 meses (rango: 3 a 228). Se constató una mortalidad del 23% (n = 6). Los factores asociados en forma significativa a mayor mortalidad fueron: gasometría arterial con menor presión parcial de oxígeno (41.5 mm Hg vs. 73.3 mm Hg; p = 0.041); mayor presión parcial de dióxido de carbono (59.5 mm Hg vs. 39.6 mm Hg; p = 0.0008); presencia de hipertensión pulmonar (83.3% vs. 16.6%; p = 0.001) y mayor presión pulmonar wedge (12.5 mm Hg vs. 9.5 mm Hg; p = 0.041). Hubo una tendencia a mayor mortalidad en pacientes con estadio radiológico III/IV (66% vs. 27%; p = 0.082) y menor DLCO (33.5% vs. 51.4%; p = 0.087). Las características clínicas y evolutivas encontradas en nuestra serie difirieron en algunos aspectos con las publicadas en la literatura internacional. Los factores asociados a mortalidad estuvieron relacionados a la gravedad de la enfermedad.

We analyzed clinical characteristics of 26 patients with suggesting clinical picture and histopatological diagnosis of sarcoidosis. We identified mortality-related variables in the follow-up. We examined clinical data and several complementary tests. Follow-up was performed by clinical consultation and telephonic interview. The patients mean age was 42.6 ± 12.7 years old, and 53.8% were female. Pulmonary affection was present in 88.4% of patients and extrapulmonary manifestation were seen in 30.7%. Radiological stage II was the most frequent (34.7%). The predominant spirometric abnormality was a low carbon monoxide diffusing capacity (DLCO) in 56.5% of cases. Pulmonary hypertension was found in 34.7% of cases. Steroid therapy was performed in 69.2%. The follow-up was completed in 96.1% of patients with a mean of 98 ± 73 months (range 3 to 228). The mortality rate was 23% (n = 6). The factors significantly associated with mortality were: blood arterial gases with lower partial oxygen pressure (41.5 mm Hg vs. 73.3 mm Hg; p = 0.041); higher partial carbon dioxide pressure (59.5 mm Hg vs. 39.6 mm Hg; p = 0.0008); presence of pulmonary hypertension (83.3% vs. 16.6%; p = 0.001) and higher pulmonary capillary wedge pressure (12.5 mm Hg vs. 9.5 mm Hg; p = 0.041). There was a tendency to higher mortality in patients with radiological stage III/IV (66% vs. 27%; p = 0.082) and lower DLCO (33.5% vs. 51.4%; p = 0.087). Clinical characteristics and long-term prognosis in our serie differed from others publications in international literature. Mortality-related factors were associated with severity of disease.

Validação de um protocolo para o teste de caminhada de seis minutos em esteira para avaliação de pacientes com hipertensão arterial pulmonar / Validation of a treadmill six-minute walk test protocol for the evaluation of patients with pulmonary arterial hypertension

OBJETIVO: Elaborar e validar um protocolo para teste de caminhada de seis minutos em esteira (TC6est) para a avaliação de pacientes com hipertensão arterial pulmonar (HAP). MÉTODOS: A população do estudo foi composta por 73 pacientes com HAP diagnosticados através de cateterismo cardíaco direito, com ou sem inalação de NO. Todos os pacientes realizaram um TC6 em solo e três TC6est baseados em um protocolo de incremento de velocidade pré-determinado e intercalados por um período de repouso. Os pacientes que haviam realizado o teste hemodinâmico com inalação de NO realizaram o terceiro TC6est com a inalação da mesma dose de NO utilizada durante o cateterismo. RESULTADOS: Os resultados mostraram uma correlação da distância caminhada no TC6est com os dados hemodinâmicos, assim como com a classe funcional e com a distância caminhada no solo. Além disso, a distância percorrida no TC6est apresentou uma correlação significativa com a sobrevida, confirmando, portanto, sua correlação com a gravidade da doença. A inalação de NO durante o TC6est levou a variações compatíveis com as variações hemodinâmicas frente à mesma dose de NO, sugerindo que o protocolo em questão pode refletir o efeito de intervenções terapêuticas. CONCLUSÕES: Concluímos que a distância percorrida no TC6est é um marcador funcional e prognóstico na avaliação de rotina de pacientes com HAP.

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.

Embolismo del líquido amniótico vs hipertensión pulmonar primaria: presentación de un caso / Amniotic fluid embolism vs primary pulmonary hypertension: a case report

El embolismo del líquido amniótico continúa apareciendo con una mortalidad elevada. A pesar de que algunas pacientes sobreviven, las probabilidades de morir son mayores que las de salvarse, según las estadísticas. Tiene una incidencia de 1 x 30 000 partos y la tasa de mortalidad es mayor del 80 porciento. El cuadro clínico es variable según la cantidad de líquido amniótico y la brusquedad con que pase al torrente sanguíneo materno. El diagnóstico se establece por la presencia de células escamosas fetales y mucinas en las arterias pulmonares maternas. Este hallazgo no es sensible, ni específico para el diagnóstico. El presente caso trata de una gestante de 38,4 semanas, multípara con antecedentes de linfocitosis heredo familiar, histiocitosis eritrofagocitaria, esplecnectomía y anemia con feto en pelviana que se inicia en trabajo de parto, comienza con cuadro de pielo erección, cefalea, cianosis, escalofríos, hipotensión y pulso débil. Se opera de urgencia. Se extrae recién nacido vivo, la paciente continúa con igual cuadro clínico, más sangramiento vaginal y por punturas, coagulograma y plaquetas alterados. Se interpreta como un embolismo del líquido amniótico y coagulación intravascular diseminada (CID). Fallece la paciente, anatomía patológica informa como causa bßsica: hipertensión pulmonar primaria y embarazo más CID. Este diagnóstico generó una intensa polémica que inspiró la realización de este artículo.

The amniotic fluid embolism still shows an elevated mortality. In spite of the fact that some patients survive, the probabilities of dying are higher than those of surviving, according to the statistics. It has an incidence of 1 x 30 000 deliveries and the mortality rate is over 80 percent. The clinical picture varies according to amount of amniotic fluid and to the suddenness with which it goes to the maternal bloodstream. The diagnosis is established by the presence of fetal squamous cells and mucins in the maternal pulmonary arteries. This finding is neither sensitive nor specific for the diagnosis. The case of a multiparous pregnant of 38.4 weeks with previous history of hereditary familiar lymphocytosis, erythrophagocytic histiocytosis, splenectomy and anemia with pelvic presentation was reported. Labor was initiated and a clinical picture of piloerection, headache, cyanosis, chills, hypotension and weak pulse started. Emergent surgery was performed, the live newborn was extracted, and the patient continued with the same clinical picture, more bleeding through the vagina and punctures, and altered coagulogram and platelets. It was interpreted as an embolism of the amniotic fluid and disseminated intravascular coagulation (DIC). The patient died and the pathological anatomy department informed as a basic cause primary pulmonary hypertension and pregnancy plus CID. This diagnosis generated an intense polemic that led us to make this review.

Traque-oclusão intra-útero: técnica cirúrgica assistida à corticoterapia promove aumento nos receptores de VEGF (Vascular Endothelial Growth Factor) em pulmões de fetos de ratas / In utero tracheal occlusion: surgical approach associated to steroid treatment increase VEGF (Vascular Endothelial Growth Factor) receptors in lungs of fetal rats

A hérnia diafragmática congênita (HDC) causa hipoplasia e hipertensãopulmonar e em geral leva a alta morbidade e mortalidade neonatal. Traqueo-oclusão fetal (TO) e corticoterapia pré-natal são alternativas para acelerar o crescimento pulmonar fetal e diminuir a hipoplasia na HDC. A produção de VEGF (Vascular Endothelial Growth Factor) está relacionada com a maturidade pulmonar e sofre alterações na HDC ainda não elucidadas.Materiais e métodos: Seis grupos de 12 fetos de ratos Spreague-Dawley foram comparados: TO, Sham, Controle, TO+Dex, Sham+Dex e Controle+Dex. No dia 18,5º foi realizada TO come sem corticoterapia utilizando dexametasona. No 21,5º dia gestacional os pesos corporal e...

Congenital diaphragmatic hernia (CDH) presents with hypoplastic lungs and usually leads to pulmonary hypertension and high neonatal mortality. Fetal tracheal occlusion (TO) and prenatal corticotherapy are alternatives to accelerate fetal pulmonary growth and decrease hypoplasia in CDH. VEGF (Vascular Endothelial Growth Factor)production and surfactant production by type II pneumocytes are related with pulmonary maturity and are altered in CDH, but little has been described about VEGF receptors. Our objective wasto quantify the receptors of VEGF (VEGFR) and type II pneumocytes, verifying the effects of TO and corticotherapy on normal lungs of fetal rats...

Hipertensão pulmonar na doença tromboembólica crônica / Pulmonary hypertension in chronic thromboembolic disease

Hipertensão pulmonar crônica tromboembólica é observada em até 3,8 por cento dos pacientes que sobreviveram a embolia pulmonar aguda. O desenvolvimento da técnica da tromboendarterectomia possibilitou a melhora significativa da sobrevida dos pacientes portadores de hipertensão pulmonar tromboembólica, com resolução do quadro hipertensivo na maioria dos pacientes operados. Nos últimos anos, tornou-se possível a definição de critérios mais claros para a indicação cirúrgica, assim como de marcadores de prognóstico, o que determinou significativa diminuição do risco cirúrgico associado ao procedimento. Atualmente tratamento médico com drogas está sendo feito em pacientes em que a tromboendarterectomia não apresentou bons resultados ou foi contra-indicada.

Hipertensão arterial pulmonar nas doenças do tecido conjuntivo / Pulmonary arterial hypertension in connective tissue diseases

A hipertensão arterial pulmonar é uma causa importante de morbidade e mortalidade em pacientes com doenças difusas do tecido conjuntivo. É uma manifestação que ocorre em cerca de 10 por cento a 15 por cento dos pacientes com esclerose sistemica, isolada ou associada à doença intersticial pulmonar, e tem prognóstico ruim. Também pode acometer indivíduos com lúpus eritomatoso sistêmico e doença mista do tecido conjuntivo, e mais raramente artrite reumatóide e miopatias inflamatórias. Na esclerose sistêmica, pela pesquisa obrigatória de hipertensão arterial pulmonar no diagnóstico e seguimneto dos pacientes, é frequente o disgnóstico de formas precoces da doença.

Primary pulmonary hypertension and pregnancy

Primary pulmonary hypertension (PPH) is a disorder intrinsic to the pulmonary vascular bed characterized by sustained elevation in pulmonary artery pressure and pulmonary vascular resistance with normal pulmonary artery wedge pressure, in the absence of a known cause. Cardiovascular disorders are the most common cause of morbidity and mortality in pregnant women. Risk is so great with some cardiovascular abnor-malities that recommendation of avoidance or interruption of pregnancy is supportable, one of these conditions is pulmonary hypertension. In this article two patients with primary pulmonary hypertension who sustained two pregnancies each are described. Both patients had uneventful pregnancies and deliveries, with all neonates surviving. When evaluating primary pulmonary hypertension and pregnancy, early studies reported a maternal mortality rate as high as 50%. More recent studies report a maternal mortality of 30%. In this article, cases of primary pulmonary hypertension undergoing pregnancy from 1978 to 2005 were reviewed, revealing a 22% maternal mortality for the total number of pregnancies. Despite advances in treatment, primary pulmonary hypertension continues to carry considerable maternal morbidity and mortality, and prevention of pregnancy is still the main recommendation. Early diagnosis and treatment is critical and a multi-disciplinary approach is required when dealing with a patient with PPH who desires to continue pregnancy.