Hipertensión pulmonar en cardiopatías congénitas del adulto / Pulmonary hypertension in adult congenital heart disease / Hipertensão pulmonar na cardiopatia congênita do adulto

Introducción: la hipertensión pulmonar asociada a cardiopatías congénitas del adulto implica un amplio espectro de situaciones clínicas complejas de difícil manejo para el cardiólogo clínico. Revisión: se repasarán los principales aspectos fisiopatológicos relacionados con el desarrollo de hipertensión pulmonar en los diferentes grupos de cardiopatías congénitas, así como los criterios para establecer el diagnóstico. Se discutirá el tratamiento guiado por perfil de riesgo y los grupos farmacológicos disponibles en nuestro medio. Por último, se discutirán grupos especiales, como el síndrome de Eisenmenger y la embarazada con cardiopatía e hipertensión pulmonar. Conclusión: es importante tener presente de todas formas que la hipertensión pulmonar en este escenario puede tener un mal pronóstico (como en el síndrome de Eisenmenger), por lo cual se deben realizar los mayores esfuerzos para hacer un diagnóstico precoz y tratamiento oportuno de estos pacientes.

Introduction: pulmonary hypertension associated with congenital heart disease in adults involves a wide spectrum of complex clinical situations that are difficult to manage for the clinical cardiologist. Review: the main pathophysiological aspects related to the development of pulmonary hypertension in the different groups of congenital heart disease will be reviewed, as well as the criteria to establish the diagnosis. Treatment guided by risk profile and the pharmacological groups available in our setting will be discussed. Finally, special groups such as Eisenmenger syndrome and pregnant women with heart disease and pulmonary hypertension will be discussed. Conclusion: it is important to keep in mind, however, that pulmonary hypertension in this scenario could have a poor prognosis (for example in Eisenmenger syndrome), which is why every effort should be made to make an early diagnosis and timely treatment of these patients.

Introdução: a hipertensão pulmonar associada à cardiopatia congênita em adultos envolve um amplo espectro de situações clínicas complexas e de difícil manejo para o cardiologista clínico. Revisão: serão revistos os principais aspectos fisiopatológicos relacionados ao desenvolvimento da hipertensão pulmonar nos diferentes grupos de cardiopatias congênitas, bem como os critérios para estabelecer o diagnóstico. Serão discutidos o tratamento guiado pelo perfil de risco e os grupos farmacológicos disponíveis em nosso meio. Por fim, serão discutidos grupos especiais como síndrome de Eisenmenger e gestantes com cardiopatia e hipertensão pulmonar. Conclusão: de qualquer forma, é importante ter em mente que a hipertensão pulmonar nesse cenário pode ter um prognóstico ruim (como na síndrome de Eisenmenger), razão pela qual todo esforço deve ser feito para o diagnóstico precoce e tratamento oportuno desses pacientes.

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)

Tempo de aceleração na via de saída do ventrículo direito: correlação com a função diastólica do ventrículo esquerdo, sexo e idade / Right ventricular outflow tract acceleration time: correlation with left ventricular diastolic function, sex, and age

Fundamento: O tempo de aceleração na artéria pulmonar (TAP) pode ser utilizado para avaliação da hipertensão pulmonar na análise da função diastólica do ventrículo esquerdo. Objetivo: Avaliar se existe correlação entre o valor do tempo de aceleração na artéria pulmonar e parâmetros da função diastólica do ventrículo esquerdo em indivíduos com função sistólica do ventrículo esquerdo preservada e de acordo com sexo, idade e fatores de risco cardiovasculares. Métodos: Estudo observacional, transversal. Foram selecionados 119 pacientes (59 mulheres; 49,6%). Os indivíduos foram submetidos ao ecocardiograma transtorácico incluindo os valores de tempo de aceleração na artéria pulmonar; ondas E e A e relação E/A ao Doppler espectral do influxo mitral; ondas e' septal, e' lateral e relação E/e' ao Doppler tecidual do anel mitral; pressão sistólica na artéria pulmonar e volume atrial esquerdo. Resultados: No sexo feminino, foi encontrada correlação positiva (coeficiente de correlação de Spearman) entre o valor do tempo de aceleração na artéria pulmonar e e' lateral (coeficiente de correlação de Spearman de 0,47; p=0,002), relação E/A (coeficiente de correlação de Spearman de 0,32; p=0,04) e e' septal (coeficiente de correlação de Spearman de 0,36; p=0,023) e uma correlação negativa entre o valor do tempo de aceleração na artéria pulmonar e pressão sistólica na artéria pulmonar (coeficiente de correlação de Spearman de -0,43; p=0,034). No sexo masculino, não foi encontrada correlação significativa. Foram encontrados menores valores de tempo de aceleração na artéria pulmonar em mulheres com hipertensão arterial sistêmica quando comparadas a mulheres sem hipertensão arterial sistêmica (0,13±0,03 segundos versus 0,16±0,03 segundos; p = 0,015). Conclusão: O presente estudo mostrou correlação significativa dos valores do TAP com alguns parâmetros da função diastólica do ventrículo esquerdo apenas no sexo feminino, sendo que mulheres hipertensas apresentaram menores valores de TAP. (AU)

Background: Pulmonary artery acceleration time (PAAT) can be used as a parameter in the evaluation of pulmonary hypertension and aids left ventricular diastolic function (LVDF) analyses. Objective: To assess whether there is a correlation between PAAT and LVDF parameters in individuals with a preserved left ventricular systolic function and by sex, age, and cardiovascular risk factors. Method: Observational cross-sectional study. One hundred nineteen patients were selected (59 women [49.6%]). The subjects underwent transthoracic echocardiography including measurements of PAAT, E and A waves and E/A ratio, e' septal and e' lateral waves and E/e' ratio, pulmonary artery systolic pressure (PASP), and left atrial volume. Results: In female patients, a positive correlation (Spearman's correlation coefficient ­ Spearman correlation coefficient [SCC]) was found between the PAAT value and the lateral e' (SCC, 0.47; p = 0.002), with the E/A ratio (SCC, 0.32; p = 0.04), and with septal e' (SCC, 0.36; p = 0.023), and a negative correlation between PAAT and PASP (SCC, -0.43; p = 0.034). In men, no correlation was found between PAAT. and any parameters. Lower PAAT values were found in women with systemic arterial hypertension (hypertension) than in women without hypertension (0.13 ± 0.03 s versus 0.16 ± 0.03 s; p = 0.015). Conclusion: The present study showed a significant correlation between PAAT and some LVDF parameters in female patients only. Hypertension was correlated with lower PAAT values in women. (AU)

Hipertensión pulmonar. Definiendo la estructura y la función departamental a partir del riesgo / Pulmonary hypertension. Defining the structure and the departmental function based on risk

Se presenta un modelo departamental para la atención integral de pacientes con hipertensión pulmonar. Se resume el conocimiento actual de la hipertensión pulmonar, su mortalidad, pronóstico y la estratificación de su severidad que justifican la propuesta de la estructura y la función departamental.

A departmental model for the comprehensive care of patients with pulmonary hypertension is presented. The current knowledge of pulmonary hypertension, its mortality, prognosis and the stratification of its severity that justify the proposal of the departmental structure and function are summarized.

Tratamiento con sildenafilo en disfunción ventricular izquierda e hipertensión pulmonar asociada / Treatment with sildenafil in left ventricular dysfunction and associated pulmonary hypertension

RESUMEN Introducción: la hipertensión pulmonar es un hallazgo frecuente en la insuficiencia cardíaca. El uso del sildenafilo en estos casos es una práctica habitual, pero aún controversial por lo limitado de los estudios realizados. Objetivo: comparar las variables ecocardiográficas de hemodinamia pulmonar, en pacientes con disfunción sistólica ventricular izquierda e hipertensión pulmonar secundaria severa, antes y después del uso del sildenafilo. Materiales y métodos: se realizó un estudio de cohorte, donde se incluyeron 19 pacientes; se realizó un seguimiento de dos años. Se analizaron variables clínicas, de laboratorio y ecocardiográficas. Se evaluaron las principales variables de hemodinamia pulmonar antes del uso del sildenafilo y a las doce semanas de su indicación. Se realizó una curva de supervivencia al concluir el seguimiento. El nivel de significación estadístico empleado fue de p < 0,05. Resultados: la edad promedio fue de 56,16 ± 15,77 años y predominó el sexo masculino, con un 73,7 %. La supervivencia al término del seguimiento fue de 78,9 %. Las principales variables ecocardiográficas de hemodinamia pulmonar mostraron una reducción significativa a las doce semanas del tratamiento con sildenafilo. La supervivencia de los pacientes con una reducción del 25 % de las presiones pulmonares en el ecocardiograma realizado a las doce semanas del tratamiento, fue mayor al terminar el estudio (100 % vs 33 %, log-rank test p = 0,001). Conclusiones: posterior al uso del sildenafilo se encontró una reducción significativa de las variables de hemodinamia pulmonar en el ecocardiograma evolutivo. La sobrevida fue mayor en los pacientes que presentaron dicha reducción (AU).

ABSTRACT Introduction: pulmonary hypertension is a common finding in heart failure. The use of sildenafil in these cases is a common practice, but still controversial due to the limited number of studies carried out. Objective: to compare echocardiographic variables of pulmonary hemodynamics, in patients with left ventricular systolic dysfunction and severe secondary pulmonary hypertension, before and after the use of sildenafil. Materials and methods: a cohort study was led, including 19 patients; a two-year follow-up was carried out. Clinical, laboratory and echocardiographic variables were analyzed. The main pulmonary hemodynamics variables were evaluated before the use of sildenafil and 12 weeks after its indication. A survival curve was performed at the end of the follow-up. The statistical significance level used was p < 0.05. Results: the average age was 56.16 ± 15.77 years, and male sex predominated with 73.3 %. Survival at the end of the follow up was 78.9 %. The main echocardiographic variables of pulmonary hemodinamics showed a significant reduction at 12 weeks of treatment with sildenafil. The survival of patients with a 25 % reduction in pulmonary pressures in the echocardiogram performed at 12 weeks of treatment was greater at the end of the study (100 % vs 33 %, log-rank test p = 0.001). Conclusions: after using sildenafil, a significant reduction of pulmonary hemodynamics variables was found in the evolutionary echocardiogram. Survival was higher in patients who had this reduction (AU).

Manejo clínico de la hipertensión arterial pulmonar. Relevancia del ventrículo derecho / Relevance of right ventricle in the clinical management of pulmonary arterial hypertension

Resumen La hipertensión arterial pulmonar (HAP) requiere procesos estructurados de diagnóstico y estratificación de riesgo, siendo la función del ventrículo derecho (VD) un marcador pronóstico central. Los principales objetivos terapéuticos en la HAP son mejorar y/o intentar revertir la disfunción del VD y mantener condición de bajo riesgo. Actualmente existen múltiples fármacos con diferentes mecanismos de acción cuya combinación en doble o triple terapia ha mostrado mejores resultados que la monoterapia. Evidencia actual demuestra la importancia de incorporar tempranamente prostanoides parenterales al esquema, mejorando la funcionalidad del VD y la supervivencia. En esta revisión se refleja el papel de la función del VD en el diagnós tico, pronóstico y seguimiento de la HAP. Se recomienda la evaluación sistemática y estandarizada del VD, así como el inicio temprano de tratamiento combinado en riesgo intermedio-alto para obtener las metas de alcanzar y mantener un riesgo bajo y/o evitar la progresión de la HAP.

Abstract Pulmonary arterial hypertension (PAH) requires structured processes of diagnosis and risk stratifica tion, being the function of the right ventricle (RV) a hallmark prognosis determinant. The main therapeutic goals in PAH are to improve and try to revert RV dysfunction and maintaining a low risk. Currently, there are multiple treatments with different mechanisms of action, the combination of which in double or triple therapy has shown improved results compared to monotherapy. Recent clinical evidence shows the importance of early incorpora tion of parenteral prostanoids to the scheme, improving RV function and survival. In this review, we discuss the role of the RV function in the diagnosis, prognosis, and follow-up of PAH. We recommend the systematic and standardised evaluation of the RV as well as the early initiation of combined treatment in cases of intermediate-high risk to try to reach and keep the patient with PAH at a low risk and / or avoid the progression of PAH.

Collaborative registry of pulmonary hypertension in Argentina (RECOPILAR). Final analysis / Registro colaborativo de hipertensión pulmonar en Argentina (RECOPILAR): análisis final

Abstract The epidemiology of pulmonary hypertension (PH), especially pulmonary arterial hypertension (PAH), has not been evaluated in our country, therefore there is no reference parameter to establishing the representativeness of this information in the national order. This registry represents the first collaborative effort to provide a knowledge base of this disease, including 5 scientific societies that represent different specialties (pediatrics, rheumatology, pulmonology and cardiology) with data from 23 Argentine provinces. These efforts involved five societies of various adult (cardiology, rheumatology, and pulmonology) and pediatric (cardiology) specialties. Subjects were grouped (1-5) in accord with the 2013 Nice classification. A total of 627 patients (mean age, 50.8±18 years; women, 69.2%) were recruited. Incident cases accounted for 53%. Functional class III-IV accounted for 69% at time of diagnosis and 33.4% at time of inclusion. Distributions in groups 1-5 were 63.6%, 15.9%, 8.3%, 9.7%, and 2.4%, respectively. Treatment consisted of diuretics (51.2%), mineralocorticoid receptor antagonists (44.7%), digoxin (16.6%), anticoagulants (39.2%), renin-angiotensin antagonists (15.5%), beta blockers (15.6%), and calcium channel blockers (8%). Rates of specific therapies usage in PAH vs. non-PAH group were 80.5% vs. 40.8% (phosphodiesterase-5 inhibitors: 71% vs. 38.6%; endothelin receptor antagonists: 54.4% vs. 14.5%; prostanoids: 14.3 vs. 3.1%; all p < 0.001). Three-year survival in PAH and non-PAH differed significantly (82.8% vs. 73.3%; p = 0.001). In the Argentine RECOPILAR registry, the clinic-epidemiologic profile was that of advanced-stage disease. Diagnostic workups and therapeutics interventions, including use of specific therapy for PAH, were consistent with current recommendations. Despite delays in diagnosis, survival was aligned with other contemporary registries.

Resumen La epidemiología de la hipertensión pulmonar (HP), especialmente la arterial (HAP), no ha sido evaluada en nuestro país, por lo cual no existe un parámetro de referencia para establecer la representatividad de esta información en el orden nacional. El presente registro representa el primer esfuerzo colaborativo para una base de conocimiento de esta enfermedad, incluyendo 5 sociedades científicas que representan a distintas especiali dades médicas (pediatría, reumatología, neumonología y cardiología) con datos de 23 provincias argentinas. Los sujetos se agruparon (1-5) de acuerdo con la clasificación de Niza de 2013. El seguimiento se completó en 583 pacientes (93%) un año después del final de la inscripción. Se incluyeron 627 pacientes (edad media, 50.8 ± 18 años; mujeres, 69.2%). Los casos incidentes representaron el 53%. La clase funcional III-IV representaba 69% en el momento del diagnóstico y 33.4% en el momento de la inclusión. Las manifestaciones clínicas fueron disnea (81.8%), fatiga (54.1%), síncope (10.8%), dolor torácico (14.7%), palpitaciones (20.9%) e insuficiencia cardíaca (20.4%). Las tasas de uso de terapias específicas en la hipertensión arterial pulmonar (HAP) frente al grupo sin HAP fueron del 80.5% frente al 40.8%. La supervivencia a tres años en los subconjuntos de HAP y no HAP difirió significativamente (82.8% vs. 73.3%; p = 0.001). En el registro RECOPILAR argentino, que aborda principalmente la HAP, el perfil clínico-epidemiológico fue el d e una enfermedad en estadios avanzados. El diag nóstico y las intervenciones terapéuticas, incluido el uso de terapia específica para la HAP, fueron consistentes con las recomendaciones actuales.

Enfermedad de whipple e hipertensión pulmonar reversible / Whipple's disease and reversible pulmonary hypertension

Resumen La enfermedad de Whipple es una enfermedad multisistémica crónica, causada por la bacteria Tropherima whipplei. Se han descripto aproximadamente 1200 casos en la literatura. La incidencia mundial se estima en 9.8 casos por millón de personas. Los datos provenientes de Sudamérica y Europa muestran que afecta a varones de mediana edad. Se cree que los factores inmunológicos del huésped son los que influyen en el curso de la infección y no el genotipo del agente. Dado que las características clínicas no suelen ser específicas y el espectro de manifestaciones en órganos individuales puede ser subestimado, el diagnóstico sigue siendo muy difícil. Presentamos un caso confirmado por histopatología con compromiso multisistémico. Consideramos importante su difusión dados los escasos casos documentados en Sudamérica y la relevancia de tener presente la sospecha diagnóstica para el abordaje terapéutico precoz que mejora el pronóstico de esta rara enfermedad.

Abstract Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The worldwide incidence is estimated at 9.8 cases per million people. Data from South America and Europe show that it affects middle-aged males. It is believed that host immunological factors rather than agent genotypic traits influence the course of the infection. Since the clinical characteristics are usually nonspecific and the wide spectrum of manifestations in individual organs may be underestimated, the diagnosis remains challenging. We present a case with multisystem compromise confirmed by histopathology. We consider its publication important given the few cases documented in South America and the relevance of bearing in mind the importance of an early diagnosis for a prompt treatment that improves the prognosis of this rare disease.

Protocolo Clínico e Diretrizes Trapêuticas (PCDT) de hipertensão pulmonar tromboembólica no Estado de Goiás / Clinical Protocol and Treatment Guidelines (PCDT) for thromboembolic pulmonary hypertension in the state of Goiás

Este Protocolo Estadual e o PCDT do Ministério da Saúde, devem ser usados como referência diagnóstica e terapêutica pelos profissionais de saúde, em Goiás, para o atendimento de pessoas com hipertensão pulmonar tromboembólica crônica. A hipertensão pulmonar tromboembólica crônica (HPTEC) está classificada dentro do Grupo IV da Classificação de Hipertensão Pulmonar. Trata-se de uma forma de hioertensão pulmonar causada pela obstrução da vasculatura pulmonar por material tromboembólico fibrótico organizado que leva ao aumento da ressitência vascular pulmonar (RVP), aumento progressivo da pressão na artéria pulmonar e insuficiência cardíaca direita

State Protocol and the Ministry of Health's PCDT should be used as a diagnostic and therapeutic reference by health professionals in Goiás to care for people with chronic thromboembolic pulmonary hypertension. Chronic thromboembolic pulmonary hypertension (CTEPH) is classified within Group IV of the Pulmonary Hypertension Classification. This is a form of pulmonary hypertension caused by obstruction of the pulmonary vasculature by organized fibrotic thromboembolic material that leads to increased pulmonary vascular resistance (PVR), progressive increase in pressure in the pulmonary artery and right heart failure

Hipertensión pulmonar crónica tromboembólica tromboendarterectomía pulmonar / Chronic thromboembolic pulmonary hypertension

A nivel mundial, la tercera causa de muerte por causa cardiovascular es el Tromboembolismo Pulmonar (TEP), después del Infarto agudo de Miocardio y el Accidente cerebrovascular, con una incidencia anual estimada de 40 casos por cada 100.000 habitantes. Se comunica el caso clínico de un paciente de 44 años con diagnóstico de TEP recurrente con Hipertensión pulmonar tromboembólica crónica (CTPH) que fue sometido a tromboendarterectomía pulmonar bilateral (PTE) bajo paro circulatorio con hipotermia profunda. Se informa de los resultados de los medios de diagnóstico y del tratamiento quirúrgico mediante la tromboendarterectomía. Se discute el tema en el contexto de la experiencia internacional y nacional.

Lung cavities in chronic thromboembolic pulmonary hypertension

OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.

Causes de l'hypertension pulmonaire au Sud-Kivu, cas de l'Hôpital Provincial Général de Référence de Bukavu

Contexte et objectif. L'hypertension pulmonaire (HTP) entraîne des symptômes qui altèrent la qualité de vie des patients. En plus, le diagnostic tardif et le traitement inefficace de l'HTP réduisent considérablement la durée de vie des malades. Peu d'études sur cette maladie ont été publié en Afrique subsaharienne et pratiquement aucune en république démocratique du Congo. Le présent travail a pour objectif de déterminer la prévalence et les étiologies de l'HTP à l'hôpital provincial Général de Référence de Bukavu (HPGRB) dans le Sud-Kivu, une province de l'Est de la République Démocratique du Congo (RD Congo). Méthodes. La présente étude mono centrique s'est déroulée dans le Département de Médecine interne de l'HPGRB. C'est une étude de descriptive rétrospective qui s'est réalisée entre le 1er octobre 2014 et le 1er octobre 2019. La population de la présente étude était constituée de tout patient reçus dans le département de Médecine interne et chez qui il a été diagnostiqué une hypertension pulmonaire. Il s'agissait d'un échantillonnage exhaustif. Résultats. La prévalence de l'hypertension pulmonaire était de 3,7 %. Le sexe féminin est majoritaire avec un sex ratio de 1,49 : 1. Dans notre étude, la majorité des patients (61,3 %) avait une hypertension pulmonaire due à des cardiopathies gauches. L'hypertension pulmonaire des maladies respiratoires concernait un quart des patients (25,5 %). L'hypertension pulmonaire post embolique chronique était retrouvée chez 10,2 % des patients. Conclusion. L'hypertension pulmonaire est une pathologie assez fréquente au Sud-Kivu. Son diagnostic est possible à Bukavu et sa prise en charge demande un suivi spécialisé

Hipertensión pulmonar en niños y adolescentes: un desafío / Pulmonary hypertension in children and adolescents: a challenge

Pulmonary Hypertension is not a disease but a hemodynamic condition, caused by various etiologies, which are different in children and adults not only in terms of the age of onset but also as for incidence and prognosis. In the pediatric population a multifactorial etiology is typical. As this condition is rare and caused by different etiologies both a high clinical suspicion and a complete diagnostic algorithm are necessary for proper diagnosis and adequate staging to choose the best treatment option. In this manuscript the definition, classification and recommendations of treatment of PH in children will be presented, taking into account the updates based on results of studies and opinions of the experts that are exposed in the World Symposium of Pulmonary Hypertension (WSPH) that takes every 5 years.

La Hipertensión Pulmonar (HP) no es una enfermedad, sino una condición hemodinámica. Se produce por variadas etiologías, que difieren en los niños respecto de los adultos no solo en la edad de presentación, sino también en su incidencia y pronóstico, siendo una característica en la edad pediátrica la etiología multifactorial. Debido a lo poco frecuente de esta afección y a la multiplicidad de etiologías, su diagnóstico requiere de alta sospecha clínica, un completo algoritmo diagnóstico para llegar a la causa y una adecuada categorización del paciente según su riesgo para ofrecerle el tratamiento oportuno. En este trabajo se expondrán la definición, clasificación y recomendaciones de tratamiento de la HP en niños, teniendo en cuenta las actualizaciones basadas en resultados de estudios y opiniones de los expertos que se exponen en el Simposio Mundial de Hipertensión Pulmonar (WSPH) que se lleva acabo cada 5 años.

Análogos de prostaglandinas I2 (epoprostenol y treprostinil) para el tratamiento de la hipertensión pulmonar refractaria secundaria a displasia broncopulmonar en un lactante / Prostaglandin 12 analogs (epoprostenol and treprostinil) as a treatment for refractory bronchopulmonary dysplasia-induced pulmonary hypertension in an infant

La hipertensión pulmonar es una complicación frecuente de la displasia broncopulmonar. A pesar de su alta incidencia, existen pocos tratamientos disponibles. El epoprostenol y el treprostinil son análogos de las prostaglandinas I2, que activan la adenilato ciclasa e incrementan el adenosín monofosfato cíclico en las células de la musculatura lisa de la arteria pulmonar y pueden resultar eficaces en el tratamiento de estos pacientes. Se presenta el caso de un prematuro de extremado bajo peso con hipertensión pulmonar secundaria a displasia broncopulmonar grave, no respondedora a óxido nítrico inhalado y sildenafilo, que fue tratado con análogos de prostaglandinas I2. En nuestro paciente, este tratamiento evidenció mejoría clínica y ecocardiográfica significativa tras varias semanas de tratamiento.

Pulmonary hypertension is a common complication of bronchopulmonary dysplasia, with a high mortality rate. Despite the high incidence of pulmonary hypertension, there are few available treatments. Epoprostenol and treprostinil are prostaglandin I2 analogs that activate adenylate cyclase and increase cyclic adenosine monophosphate in the pulmonary arterial smooth muscle cells. Therefore, they may be an effective treatment for these patients. We report the use of prostaglandin I2 analogs in an extremely low birth weight preterm baby with severe bronchopulmonary dysplasia associated with pulmonary hypertension non-responding to inhaled nitric oxide and sildenafil. In our patient this treatment resulted in remarkable clinical and echocardiographic improvement, evident after a few weeks of treatment.

Valoración de ecuaciones derivadas del flujo pulmonar y regurgitación tricuspídea: utilidad en el test de vasoreactividad pulmonar / Valuation of equations derived from Pulmonary Flow and Tricuspid Regurgitation: usefulness in Pulmonary Vasoreactivity Testing

RESUMEN: Antecedentes: La presión media arterial pulmonar (PMAP) es una variable hemodinámica indispensable para el diagnóstico, clasificación y pronóstico de la Hipertensión Pulmonar (HP). Su cuantificación se realiza en forma invasiva por cateterismo cardíaco derecho (CCD) y no invasivamente por ecocardiografía Doppler. Masuyama propuso su medición mediante el gradiente transvalvular pulmonar diastólico derivado de la velocidad máxima inicial de la regurgitación pulmonar (∆RPi2) correspondiendo cercanamente a la medición invasiva. Objetivos: Revalidar 3 métodos ecocardiográficos que estiman la PMAP y valorar la utilidad del método de Chemla en el Test de Reactividad Vascular Pulmonar (TRVP). Métodos: Estudio prospectivo, observacional, doble ciego divido en dos etapas. A) o I) 30 pacientes se realizó ecocardiografía Doppler diagnóstica en nuestro centro. Se midieron regurgitación tricuspídea (RT) y tiempo de aceleración pulmonar (TAP) para derivar las siguientes ecuaciones: 1) 0.61xPSAP+1.95 (Chemla) 2) Gradiente presión media RT (∆PmRT) +PAD (presión-aurícula derecha) (Aduen) y 3) 79-0.45xTAP o 90-0.60xTAP, según sea el valor del TAP. B) o II) 10 pacientes enrolados para realizar el TRVP comparando la medición ecocardiográfica (Chemla) con CCD. Resultados: En la primera parte del estudio se encontró alta correlación entre las 3 ecuaciones: ChemlaAduen, R2=0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0,91. En la segunda parte comparando la PMAP-Chemla y Cateterismo derecho (CD) obtuvimos alta correlación: en tiempo 0, 30 min y recuperación:(R2=0.87, 0.99, 0.98, respectivamente). Ambas partes del estudio mostraron límites de concordancia satisfactoria con valor medio de la diferencia entre los métodos cercano a 1 en el t30 y tR del TRVP. Conclusión: los métodos dependientes de la medición de la RT son efectivos y confiables para estimar la PMAP. El método de Chemla es útil en el TRVP.

ABSTRACTS: Background: Mean Pulmonary arterial pressure (PMAP)is an indispensable hemodynamic variable for the diagnosis, classification and prognosis of Pulmonary Hypertension (PH). Its quantification is performed invasively by cardiac catheterization and non-invasively by Doppler echocardiography. Masuyama proposed its measurement by the transvalvular diastolic pulmonary gradient derived from the initial maximum velocity of pulmonary regurgitation(ΔPRi2) corresponding closely to the invasive measurement. Objectives: to compare 3 known echocardiographic methods to estimate MPAP and demonstrate the usefulness of the Chemla method in the Pulmonary Vascular Reactivity Test (PVRT). Methods: prospective, observational, double-blind study divided into two stages. A) 30 patients underwent diagnostic Doppler echocardiography. Tricuspid regurgitation (TR) and pulmonary acceleration time (PAT) were measured to derive the equations: 1) 0.61xSPAP + 1.95 (Chemla) 2) Gradient mean pressure TR (ΔPmTR) + RAP (right atrium pressure) (Aduen).3) 79-0.45xPAT o 90-0.60xPAT depending on the value of PAT. B) 10 patients enrolled to PVRT comparing the echocardiographic measurement (Chemla) with right catheterization. Results: in the first part of the study a high correlation between the 3 equations was found : ChemlaAduen, R2 = 0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0.91. In the second part comparing the MPAP-Chemla and RHC we obtained a high correlation in time 0, 30 min and recovery: (R2=0.87,0,99,0.98, respectively). Both parts of the study showed satisfactory limits concordance with mean value of the difference between the methods close to 1 in the t30 and tR of the TRVP. Conclusion: the methods dependent on the measurement of the TR are effective and reliable for estimating MPAP. The Chemla method is useful in the PVRT.

Hipertensión arterial pulmonar en pediatría / Pulmonary arterial hypertensión in children

La hipertensión arterial pulmonar (HAP) en pediatría comparte características comunes de la enfermedad en adultos, pero está asociada con varios trastornos y desafíos adicionales que requieren enfoques diferentes. En este artículo se analizan los avances recientes, los desafíos actuales y los distintos enfoques para el cuidado de niños con HAP. Se actualizan la definición, epidemiología, clasificación, diagnóstico y tratamiento. Se plantea el uso del cateterismo cardíaco como diagnóstico y las definiciones hemodinámicas de HAP, incluido el test de vasorreactividad. Se proporcionan actualizaciones sobre los enfoques pediátricos específicos del manejo médico e intervencionista de la HAP (incluyendo la derivación de Potts). Aunque la falta de datos de ensayos clínicos para el uso de la terapia dirigida a la HAP, los datos emergentes están mejorando la identificación de objetivos adecuados para la terapia orientada a objetivos en niños.(AU)

Pulmonary arterial hypertension (PAH) in children shares the typical features of the disease in adults, but is associated with different disorders and additional challenges that require different approaches. This article discusses recent developments, current challenges, and different approaches to PAH care in children. Definition, epidemiology, classification, diagnosis, and treatment are updated. The use of cardiac catheterization as a diagnostic tool and hemodynamic definitions of PAH are proposed, including the vasoreactivity test. Updates are provided on specific pediatric approaches to the medical and interventional management of PAH (including Potts shunt). In spite of the lack of clinical trial data for the use of PAH-targeted therapy, emerging data are improving the identification of appropriate targets for therapy in children (AU)

Dor torácica na sala de emergência: quem fica e quem pode ser liberado? / Chest pain in the emergency room: who is left and who can be released?

A dor torácica aguda é um sintoma muito frequente nas unidades de emergência, constituindo-se em um possível sinal de alerta para as doenças com risco iminente de morte. Como a maioria desses pacientes é internada para avaliação de uma possível síndrome coronariana aguda, isso gera um custo hospitalar muito alto por paciente. Por conta dessa possibilidade diagnóstica, muitos emergencistas internam a maioria dos pacientes. Por outro lado, a liberação inapropriada daqueles com infarto agudo do miocárdio representa um risco para o médico e, especialmente, para o paciente. Outro ponto importante é a demora para o atendimento, em que há a influência de fatores relacionados ao paciente, assim como, pontos negativos na logística de atendimento dos serviços de emergência em nosso país. Para excelência no atendimento, é importante uma anamnese detalhada adicionada ao exame físico, a qual permite a elaboração das hipóteses diagnósticas. E para auxiliar os médicos na escolha da hipótese diagnóstica e na tomada rápida de decisão, escores de risco são disponibilizados, os quais, facilmente, identificam a probabilidade de eventos adversos. A conduta imediata de casos com risco de morte imediata tem como principal objetivo reduzir a morbidade e a mortalidade, aumentando, consequentemente, a segurança do profissional da emergência. Sugestões de fluxogramas e algoritmos para o atendimento desses pacientes na sala de emergência definem, de forma objetiva, quem fica e quem pode ser liberado

Acute chest pain is a frequent symptom in emergency units, being a possible war-ning sign of diseases with an imminent risk of death. Since most of these patients are hospitalized to evaluate possible acute coronary syndrome, this generates a very high hospital cost per patient. Because of this diagnostic possibility, emergency professionals admit most patients. In contrast, the inappropriate release of those with acute myocardial infarction poses a risk to the physician and, especially, the patient. Another important point is the delay in care, where there is an influence of patient-related factors, as well as negative points in the logistics of care in the emergency services in our country. For excellence in care, a detailed anamnesis added to the examination is important, allowing the elaboration of diagnostic hypotheses. Moreover, to assist physicians in selecting the diagnostic hypothesis and making fast decisions, there are risk scores that easily identify the likelihood of adverse events. The immediate management of cases with an imminent risk of death is the main objective to reduce morbidity and mortality and, consequently, increase the safety of emergency professionals. Flowcharts and algorithm suggestions targeting patients in the emergency room objectively define who stays and who can be released

Correlação entre graus de hipertensão pulmonar e achados em exames complementares / Correlation between degrees of pulmonary hypertension and findings in complementary tests

Fundamento: A hipertensão pulmonar é potencialmente fatal, com grande impacto na qualidade de vida do paciente. O tratamento pode alterar sua progressão, mas o aumento na sobrevida é relacionado ao diagnóstico e à terapêutica precoces. Objetivo: Analisar a relação entre alterações em exames usados de rotina (ecocardiografia, eletrocardiografia e tomografia computadorizada) e o aumento da pressão sistólica da artéria pulmonar. Métodos: Avaliamos fichas de pacientes portadores de hipertensão pulmonar, separando-os em dois grupos de acordo com a pressão sistólica da artéria pulmonar estimada à ecocardiografia. O Grupo 1 consistiu de pacientes com pressão sistólica da artéria pulmonar entre 35 e 65 mmHg, e o Grupo 2, de pacientes com pressão sistólica da artéria pulmonar > 65 mHg. Analisamos a prevalência de alterações sugestivas de hipertensão pulmonar em exames de ecocardiografia, eletrocardiografia e tomografia computadorizada. Foram realizadas análises descritiva dos dados e comparativa entre os grupos.Resultados: Foram analisados 101 pacientes (43 do Grupo 1 e 58 do Grupo 2). A maioria foram mulheres (82). Valores maiores de pressão sistólica da artéria pulmonar foram correlacionados com dilatação de câmaras direitas à ecocardiografia (p < 0,001). À eletrocardiografia, sinais de hipertrofia direita e presença de padrão strain ventricular foram mais prevalentes no Grupo 2 (p < 0,05). À tomografia computadorizada, observaram-se maiores diâmetros de tronco da artéria pulmonar em pacientes do Grupo 2 (p < 0,05). Houve correlação entre progressão clínica da doença pelo escore e aumento da pressão sistólica da artéria pulmonar (p < 0,05).Conclusão: É possível, em um primeiro momento, fazer uso do exame clínico e da eletrocardiografia para uma estimativa da gravidade do quadro de hipertensão pulmonar. Os achados, então, devem ser confirmados com análise secundária por tomografia computadorizada e ecocardiografia

Introduction: Pulmonary hypertension is potentially fatal and courses with important day-to-day limitation. While the treatment is capable of slowing the disease's progression, increase in life expectancy is directly linked to early diagnosis and treatment. Objectives:To analyze the relation between alterations detectable on routine exams performed on pulmonary hypertension patients (echocardiography, electrocardiography and computerized tomography) and increases on pulmonary artery systolic pressure. Methods: We analyzed the recordings of patients presented with pulmonary hypertension, and separated them in two groups based on their echocardiography-estimated pulmonary artery systolic pressure. Group 1 was composed of patients with pulmonary artery systolic pressure between 35 mmHg and 65 mmHg, and Group 2 of patients with pulmonary artery systolic pressure > 65 mmHg. We analyzed the prevalence of alterations suggestive of pulmonary hypertension on echocardiography, electrocardiography and computerized tomography. A descriptive analysis of the findings was conducted, followed by comparative analysis between the groups. Results: We analyzed 101 patients (43 from Group 1 and 58 from Group 2). Most were women (82). Higher pulmonary artery systolic pressure values were correlated with right-heart dilation on echocardiography (p < 0.001). Electrocardiography findings revealed that right-heart hypertrophy and ventricular strain pattern were more common on Group 2 (p < 0.05). Computerized tomography analysis showed Group 2 patients had superior pulmonary artery diameters (p < 0.05). There was correlation between high pulmonary artery systolic pressure and clinical progression of the disease measured via the NYHA score (p < 0.05). Conclusion: It is possible to make use of clinical examination and a simple electrocardiography to stratify the severity of a pulmonary artery patient. Computerized tomography and echocardiography should be used to confirm these findings

Hipertensión pulmonar asociada al virus de la inmunodeficiencia humana en pediatría: tratamiento con sildenafil. A propósito de un caso / Pulmonary hypertension associated with the human immunodeficiency virus in children: treatment with sildenafil. A case report

La hipertensión pulmonar asociada a la infección por virus de inmunodeficiencia humana es una enfermedad sumamente infrecuente en pediatría, por lo que requiere alta sospecha clínica para llegar a su diagnóstico. Su aparición es de pronóstico desfavorable, pero el diagnóstico precoz y el tratamiento específico pueden mejorar su evolución. Se presenta el caso clínico de un paciente de 15 años con diagnóstico de infección por virus de inmunodeficiencia humana de transmisión vertical, sin tratamiento antirretroviral, con tos y disnea de esfuerzo progresiva asociadas a signos de falla cardíaca derecha en el cual se diagnosticó hipertensión pulmonar grave. Luego de descartarse otras causas, se asumió la hipertensión pulmonar asociada a la infección por virus de inmunodeficiencia humana. Se realizó el tratamiento con sildenafil y presentó buena respuesta.

Pulmonary hypertension associated with human immunodeficiency virus infection is an extremely rare disease in pediatrics; it requires a high clinical suspicion to reach a diagnosis. Its appearance poses an unfavorable prognostic, but early diagnosis and specific treatment can improve outcomes. We report the clinical case of a fifteen-year-old patient diagnosed with human immunodeficiency virus infection of vertical transmission, without antiretroviral treatment, with cough and progressive exertional dyspnea, associated with signs of right heart failure in which severe pulmonary hypertension was diagnosed. After discarding other causes, it was assumed pulmonary hypertension associated with human immunodeficiency virus infection. Treatment was performed with sildenafil with good response.

Hipertensión portopulmonar: Revisión actualizada / Portopulmonary hypertension: Updated review

Resumen: La hipertensión portopulmonar (HPP) es una entidad poco frecuente a nivel mundial, aunque se desconocen los datos epidemiológicos en México. Sin embargo, las enfermedades crónicas del hígado son muy prevalentes en mexicanos. La HPP es el 4.◦ subtipo en frecuencia del grupo de la hipertensión arterial pulmonar. Su diagnóstico está dentro de 2 escenarios: los pacientes con sospecha de hipertensión pulmonar y los candidatos a trasplante hepático ortotópico (THO). Tanto el ecocardiograma como el cateterismo cardiaco derecho son determinantes para el diagnóstico en ambos escenarios. La HPP es un reto para el THO, pues aumenta la mortalidad perioperatoria de manera importante. El uso de terapia específica es la piedra angular de este padecimiento, como una medida para poder mejorar el desenlace de los que llegan a ser candidatos a un THO con HPP moderada a grave. Es importante reconocer que la HPP puede llegar a ser una contraindicación para el THO. Hasta el momento el papel del trasplante combinado pulmón-hígado o corazón-pulmón-hígado como una medida de curación de la enfermedad vascular pulmonar en pacientes con HPP es incierto. © 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Publicado por Masson Doyma Me´xico S.A. Este es un art´ıculo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Abstract: Portopulmonary hypertension (PPH) is a rare condition worldwide, although epidemiological data are unknown in Mexico. However, chronic liver diseases are very prevalent in Mexico. PPH is the 4th subtype in frequency in the group of pulmonary arterial hypertension. Its diagnosis is made within 2 scenarios: patients with suspected pulmonary hypertension and candidates for orthotopic liver transplantation (OLT). Both echocardiogram and a right cardiac catheterisation are crucial for diagnosis in both cases. PPH is a challenge for OLT, since it can significantly increase perioperative mortality. The use of specific therapy is the cornerstone of this disease, as a measure to improve the outcome of those who become candidates for OLT with moderate to severe PPH. It is important to recognise that PPH can be a contraindication to OLT. The role of lung-liver transplantation or heart-lung-liver transplantation as a measure to heal pulmonary vascular disease in patients with PPH is still uncertain.© 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Published by Masson Doyma Me´xico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).