Mixoma auricular izquierdo gigante que causa obstrucción valvular mitral, hipertensión pulmonar y tromboembolia pulmonar / Giant atrial myxoma causing mitral valve obstruction, pulmonary hypertension and pulmonary thromboembolism

Resumen: Los tumores cardíacos pueden ser primarios o, más frecuentemente secundarios o metastásicos. Entre los tumores primarios es más frecuente el mixoma, cuya ubicación más común es en la aurícula izquierda. Las manifestaciones clínicas son diversas, producidas principalmente por obstrucción mecánica, embolizaciones, y manifestaciones constitucionales. Se comunica el caso de un paciente de 32 años, con cuadro clínico de insuficiencia cardíaca, hipertensión pulmonar severa y tromboembolismo pulmonar bilateral. Se hizo el diagnóstico de mixoma auricular izquierdo. Se resecó el tumor y se manejó la hipertensión pulmonar desde el ingreso al hospital con inhibidores de la fosfodiesterasa asociado a anticoagulación. Se discute el tema dando énfasis a aspectos fisiopatológicos involucrados tanto en la hipertensión pulmonar como en la presencia de tromboembolia pulmonar.

Abstract: Cardiac tumors may be primary or, more frequently secondary or associated to metastasis. Atril myxoma es the most frequent primary tumor, usually located in the left atrium. Clinical manifestations include those due to mitral valve occlusión, emboli and general non spedific symptoms and signs. Herein we report the clinical case of a 32 year old patient with severe pulmonary hypertension and bilateral pulmonary embolism. The tumor was extirpated, and he received phosphoro-diesterase inhiborts and anticoagulants. Subsequent clinical course was satisfactory. A brief discussion of this condicion is included.

Coexistent mitral stenosis and left circumflex coronary artery to left atrial fistula in a patient with severe pulmonary hypertension

Abstract Coronary artery to left atrial fistula is rare in patients with mitral stenosis. We report an interesting case of a patient with concomitant mitral valve stenosis and coronary fistulae, originating from the left circumflex artery and drained into the left atrium with two terminal orifices.

Accuracy of echocardiography and chest tomography for pulmonary hypertension screening in patients awaiting lung transplantation / Acurácia do ecocardiograma e da tomografia de tórax para rastreamento de hipertensão pulmonar em pacientes aguardando transplante de pulmão

ABSTRACT Objective To examine the accuracy of a pulmonary hypertension screening strategy based on a combination of echocardiographic data and tomographic measurements (pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio) in patients with chronic lung disease referred for lung transplantation. Methods A retrospective observational study with patients with pulmonary emphysema or fibrosis referred for transplantation between 2012 and 2016. Pulmonary hypertension was defined as mean pulmonary artery pressure ≥25mmHg, or between 21 and 24mmHg, with pulmonary vascular resistance >3 Wood units on right heart catheterization. Tomographic measurements were made by two independent radiologists. Results This sample comprised 13 patients with emphysema and 19 patients with pulmonary fibrosis. Of these, 18 had pulmonary hypertension. The level of agreement in tomographic measurements made by radiologists was high (intraclass correlation coefficients 0.936 and 0.940, for pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio, respectively). Areas under the ROC curves constructed for pulmonary artery diameter, pulmonary artery diameter to ascending aorta diameter ratio, and pulmonary artery systolic pressure as predictors of pulmonary hypertension were 0.540, 0.629 and 0.783, respectively. The sensitivity, specificity and negative predictive value of pulmonary artery systolic pressure ≥40mmHg were 67%, 79% and 65%, respectively. The combined criterion (pulmonary artery diameter to ascending aorta diameter ratio >1 and/or pulmonary artery systolic pressure ≥40mmHg) achieved sensitivity of 72%, specificity of 79%, and a negative predictive value of 69%. Conclusion Measurements of pulmonary artery and ascending aorta diameter were highly reproducible. The association of pulmonary artery and aortic diameter >1 and/or pulmonary artery systolic pressure ≥40mmHg improved the sensitivity and the negative predictive value for pulmonary hypertension screening. This strategy demands prospective validation to assess safety and cost-effectiveness.

RESUMO Objetivo Avaliar a acurácia de uma estratégia de rastreamento de hipertensão pulmonar baseada na combinação de dados de ecocardiograma com as medidas derivadas da tomografia computadorizada (diâmetro da artéria pulmonar e razão entre diâmetro da artéria pulmonar e diâmetro da aorta ascendente) em pacientes pneumopatas crônicos encaminhados para transplante pulmonar. Métodos Estudo observacional retrospectivo realizado com pacientes com enfisema e fibrose pulmonar avaliados para transplante entre 2012 e 2016. Definiu-se hipertensão pulmonar como pressão arterial pulmonar média ≥25mmHg, ou entre 21 a 24mmHg, com resistência vascular pulmonar >3 unidades Wood no cateterismo direito. As medidas tomográficas foram realizadas por dois radiologistas independentes. Resultados Foram incluídos 13 pacientes com enfisema e 19 com fibrose pulmonar, sendo 18 com hipertensão pulmonar. Houve alta concordância entre os radiologistas em relação às medidas tomográficas (coeficientes de correlação intraclasse para diâmetro da artéria pulmonar de 0,936 e diâmetro da artéria pulmonar/diâmetro da aorta ascendente de 0,940). As áreas abaixo da curva ROC de diâmetro da artéria pulmonar, diâmetro da artéria pulmonar/diâmetro da aorta ascendente, e pressão sistólica da artéria pulmonar como preditores de hipertensão pulmonar foram 0,540, 0,629 e 0,783, respectivamente. A sensibilidade, especificidade e valor preditivo negativo da pressão de sistólica de artéria pulmonar ≥40mmHg foram 67%, 79% e 65%, respectivamente. O critério combinado de diâmetro da artéria pulmonar/diâmetro da aorta ascendente >1 e/ou pressão sistólica da artéria pulmonar ≥40mmHg mostrou sensibilidade de 72%, especificidade de 79%, e valor preditivo negativo de 69%. Conclusão Os diâmetros da artéria pulmonar e da aorta ascendente foram altamente reprodutíveis. A associação entre diâmetro da artéria pulmonar e diâmetro da aorta ascendente >1 e/ou pressão sistólica da artéria pulmonar ≥40mmHg melhorou a sensibilidade e o valor preditivo negativo para rastreamento de hipertensão pulmonar. Essa estratégia requer validação prospectiva para se avaliarem segurança e custo-efetividade.

Achados ao ecocardiograma transtorácico em pacientes hospitalizados com COVID- 19: resultados do registro brasileiro de ecocardiografia durante a pandemia de COVID-19 (ECOVID) / Transthoracic echocardiography findings of hospitalized patients with COVID-19: results from the brazilian echocardiography registry during the COVID-19 Pandemic (ECOVID)

Fundamento: A ecocardiografia transtorácica (ETT) pode desempenhar um papel crucial na avaliação das manifestações cardíacas da COVID-19. Objetivo: Nosso objetivo foi relatar a prevalência das principais anormalidades ecocardiográficas em pacientes hospitalizados com COVID-19. Métodos: Realizou-se estudo observacional multicêntrico prospectivo com pacientes com COVID-19 submetidos a ETT durante a internação. Pacientes com insuficiência cardíaca prévia, doença arterial coronariana ou fibrilação atrial foram classificados como portadores de doença cardiovascular (DCV) prévia. Foram coletados dados clínicos e ecocardiográficos da estrutura e da função cardíaca. Resultados: Avaliamos 310 pacientes com COVID-19, com 62±16 anos de idade, 61% homens, 53% com hipertensão arterial, 33% com diabetes e 23% com DCV prévia. No total, 65% dos pacientes necessitaram de suporte em unidade de terapia intensiva. As alterações ecocardiográficas mais prevalentes foram hipertrofia do ventrículo esquerdo (VE) (29%), hipertensão pulmonar (25%), disfunção sistólida do VE (16,5%), disfunção sistólica do ventrículo direito (VD) (15,9%), disfunção diastólica do VE grau II/III (11%) e alteração da contratilidade regional do VE (11%). Derrame pericárdico foi incomum (7%). Hipertrofia do VE (25 vs. 45%, p=0,001), disfunção sistólica do VE (11 vs. 36%, p<0,001), alterações da contratilidade regional (6 vs. 29%, p<0,001), disfunção diastólica do VE grau II/III (9 vs. 19%, p=0,03) e hipertensão pulmonar (22 vs. 36%, p=0,019) foram menos comuns nos pacientes sem do que com DCV prévia. A disfunção sistólica do VD mostrou-se semelhante em pacientes sem e com DCV prévia (13 vs. 25%, p=0,07). Conclusões: Entre os pacientes hospitalizados com COVID-19, os achados ecocardiográficos anormais foram comuns, porém menos encontrados naqueles sem DCV. A disfunção sistólica do VD pareceu afetar de forma semelhante pacientes com e sem DCV prévia. (AU)

Background: Transthoracic echocardiography (TTE) may play a crucial role in the evaluation of cardiac manifestations of coronavirus disease 2019 (COVID-19). Objective: We aimed to report the prevalence of the main echocardiographic abnormalities of hospitalized COVID-19 patients. Methods: We performed a prospective multicenter observational study in patients with COVID-19 who underwent TTE during hospitalization. Patients with pre-existing heart failure, coronary artery disease, or atrial fibrillation were categorized as having previous cardiovascular disease (CVD). Clinical and echocardiographic data about cardiac structure and function were collected. Results: We evaluated 310 patients with COVID-19 (mean age, 62±16 years; 61% men; 53% with arterial hypertension; 33% with diabetes; and 23% with previous CVD). Overall, 65% of the patients required intensive care unit support. The most prevalent echocardiographic abnormalities were LV hypertrophy (29%), pulmonary hypertension (25%), left ventricular (LV) systolic dysfunction (16.5%), right ventricular (RV) systolic dysfunction (15.9%), grade II/III LV diastolic dysfunction (11%), and LV regional wall motion abnormality (11%). Pericardial effusion was uncommon (7% of cases). LV hypertrophy (25% vs. 45%, p=0.001), LV systolic dysfunction (11% vs. 36%, p<0.001), regional wall motion abnormalities (6% vs. 29%, p<0.001), grade II/III LV diastolic dysfunction (9% vs. 19%, p=0.03), and pulmonary hypertension (22% vs. 36%, p=0.019) were less common in patients without previous CVD. RV systolic dysfunction occurred at similar frequencies in patients with versus without previous CVD (13% vs. 25%, p=0.07). Conclusions: Among patients hospitalized with COVID-19, abnormal echocardiographic findings were common, but less so among those without previous CVD. RV systolic dysfunction appeared to affect similar proportions of patients with versus without previous CVD. (AU)

Análisis de los resultados del programa de ecocardiografía funcional neonatal en un hospital pediátrico de tercer nivel / Analysis of the results of the neonatal functional echocardiography program in a third-level pediatric hospital

Resumen Introducción: Se ha demostrado la utilidad del estudio ecocardiográfico en las enfermedades que afectan a los neonatos en estado crítico, por lo que se debe capacitar al neonatólogo para la toma y la interpretación de variables hemodinámicas que apoyen la toma de decisiones. El objetivo de este estudio fue describir los resultados del programa de ecocardiografía funcional neonatal y comparar las variables clínicas y hemodinámicas entre sobrevivientes y no sobrevivientes. Métodos: Durante un periodo de 2 años se realizó un estudio observacional, transversal y comparativo en neonatos que fueron evaluados con ecocardiografía funcional neonatal por alteraciones hemodinámicas del conducto arterioso persistente, hipertensión pulmonar aguda y crónica, estado de choque y búsqueda de trombos y vegetaciones. Se evaluaron parámetros de funcionalidad de los ventrículos derecho e izquierdo, presión pulmonar y subrogados de sobrecarga pulmonar. Se realizó un análisis comparativo (U de Mann Whitney y Χ2). De acuerdo con los resultados, se emitieron recomendaciones para el inicio, el ajuste o el retiro de fármacos vasoactivos. Resultados: Se realizaron 269 estudios en 119 neonatos (64.7% prematuros, mortalidad del 15%) atendidos por hipertensión pulmonar aguda (38%), conducto arterioso persistente (27%), choque (19%), hipertensión pulmonar crónica (14%) o búsqueda de trombos (2%). Se recomendó cambiar el manejo en el 45% de los estudios. El grupo de no sobrevivientes presentó diferencias significativas en los parámetros de función sistólica del ventrículo derecho y aumento de su poscarga. Conclusiones: La causa principal de la evaluación con ecocardiografía funcional neonatal fue la hipertensión pulmonar. De forma global, se recomendó un ajuste farmacológico en el 45% de los casos.

Abstract Background: Echocardiography is useful in the group of comorbidities of critically ill newborns. The targeted neonatal echocardiography program trains neonatologists for acquiring and interpreting hemodynamic variables to support decision making. This study aimed to describe the results of the functional echocardiography program (fNE) and compare clinical and hemodynamic variables between survivors and non-survivors. Methods: Observational, cross-sectional, and comparative study of neonates that received a fNE evaluation for hemodynamic disturbances related to patent ductus arteriosus (PDA), acute and chronic pulmonary hypertension (aPH, cPH), state of shock and thrombus/vegetations surveillance for two years. Functional parameters of the right and left ventricle, pulmonary pressure, and surrogates of pulmonary over circulation were assessed. Comparative analysis with U Mann Whitney test and Χ2 was performed. Based on the results, recommendations to start, adjust, or withdraw vasoactive medications were issued. Results: Of 269 studies on 119 neonates (65% premature, 15% mortality), the reasons for consultation were aPH (38%), PDA (27%), shock (19%), cPH (14%), and thrombus surveillance (2%). A change in management was recommended on 45% of studies. Non-survivors presented significant differences in the right ventricular (RV) systolic performance and an increased right ventricular afterload. Conclusions: The main indication for fNE was pulmonary hypertension. A pharmacological adjustment was recommended on 45% of the cases.

Uso de treprostinil sistémico en una paciente con hipertensión pulmonar grave, sin respuesta a la máxima terapia disponible / Use of treprostinil in a patient with a severe pulmonary arterial hypertension no responding to the maximal therapy available

En los pacientes con Hipertensión Arterial Pulmonar (HAP) de alto riesgo, en clase funcional (CF)IV, la terapia específica debe ser combinada y debe incluir una prostaciclina (PGI2) de uso sistémico en espera de trasplante bipulmonar (TBP). En el sistema público la única PGI2 disponible para asociar a Sildenafil y algún inhibidor de endotelina (Ambrisentan o Bosentan) es Iloprost nebulizado, que si bien es efectiva, no logra estabilizar los casos graves con severa disfunción del ventrículo derecho (VD). Se presenta el primer caso en el Instituto del Tórax, centro de referencia nacional de HAP, del uso de treprostinil en una paciente de 24 años con HAP grave e indicación de TBP. Treprostinil es un análogo sintético de PGI2 de uso subcutáneo en dosis desde 1 a 40 ng/kg/min. La paciente presentaba una situación de extrema gravedad: CF IV, distancia recorrida en el test de caminata de 6 min (DRTC 6 min) < 300 m,derrame pericárdico y severa disfunción del VD con TAPSE (índice de disfunción del VD) de 13 cm/s asociado a ProBNP >2.500 pg/ml. Luego de 6 meses de hospitalización en intermedio, terapia triple (Sildenafil, Ambrisentan e Iloprost nebulizado) asociado a O2,diuréticos y milrinona, logró ser dada de alta a las 3 semanas del inicio de treprostinil, regresando al trabajo a los 2 meses y estabilizando su condición en CF III, con DRTC 6 min > 440 m, mejoría de la función del VD(TAPSE 19). El ProBNP persistió elevado, 1.491 pg/ml, indicando que su enfermedad es grave y progresiva; sin embargo, ha logrado un nivel de estabilidad clínica que le permite una adecuada vida de relación familiar y laboral.

In high risk Pulmonary Arterial Hypertension (PAH) patients with functional class (FC) IV, specific therapy must be combined and must include systemic prostacyclin (PGI2), meanwhile they are enlisted for double lung transplant (DLT). In Chilean Public Health System, nebulized Iloprost is the only PGI2 available to combine with Sildenafil and either Ambrisentan or Bosentan as endothelin receptor antagonist. This association is not enough for severe cases with right ventricular (RV) dysfunction. The first case from the National Institute of Thorax as a referral center is presented now in a 24 years-old lady treated with treprostinil. She has severe PAH with DLT indication. Treprostinil is a PGI2 analog, for subcutaneous use in a dose from 1 to 40 ng/kg/min. She was extremely sick, with FC IV, she walked < 300 m at 6 min walking test (6 MWT), presented pericardial effusion and severe RV dysfunction, with TAPSE (echocardiography index for RV dysfunction)=13 cm/s, ProBNP > 2,500 pg/ml. Six months after being at intensive care unit with triple therapy (Sildenafil, ambrisentan and nebulized Iloprost) plus oxygen, diuretics and milrinone, she was finally discharged after receiving a 3 weeks treprostinil course. She came back to work two months later and her condition was more stable: FC III, she walked > 440 m at 6MWT, with a significant improvement in RV function with TAPSE = 19. Although ProBNP decreased to 1,491pg/ml, it was still high, pointing out the progressive nature of her disease. However, she met a better clinical condition which allows her to reach a much better quality of life from a personal, familial and social point of view.

Pulmonary Hypertension in General Cardiology Practice / Hipertensão Pulmonar na Prática do Cardiologista

Abstract The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of PH. When direct involvement of pulmonary circulation is suspected, invasive hemodynamic monitoring is recommended to establish the diagnosis. This assessent provides, in addition to the diagnostic confirmation, the correct identification of the vascular territory predominantly involved (arterial pulmonary or postcapillary). Treatment with specific medication for PH (phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues) has been proven effective in patients with pulmonary arterial hypertension, but its use in patients with PH due to left heart disease can even be damaging. In this review, we discuss the diagnosis criteria, how etiological investigation should be carried out, the clinical classification and, finally, the therapeutic recommendations for PH.

Resumo O achado de hipertensão pulmonar (HP) em avaliação ecocardiográfica é frequente e preocupante. No entanto, o ecocardiograma é apenas um exame sugestivo e não diagnóstico de HP. Quando se suspeita de acometimento direto da circulação pulmonar, está indicada medida hemodinâmica invasiva para estabelecer o diagnóstico. Essa avaliação permite, além da confirmação diagnóstica, a correta identificação do território vascular predominantemente acometido (arterial pulmonar ou pós-capilar). O tratamento com as medicações específicas de HP (inibidores da fosfodiestarese 5, antagonistas do receptor de endotelina, análogos da prostaciclina e estimulador da guanilil ciclase solúvel) é comprovadamente eficaz para pacientes com hipertensão arterial pulmonar, mas seu uso em pacientes com HP decorrente de doença cardíaca de câmaras esquerdas pode até mesmo ser prejudicial. Discutiremos nesta revisão o critério diagnóstico, a maneira de proceder a investigação etiológica, a classificação clínica e, finalmente, as recomendações terapêuticas na HP.

Pulmonary hypertension could be a risk for deep vein thrombosis in lower extremities after joint replacement surgery

SUMMARY A background of Pulmonary Hypertension (PH) indicates a progressive elevation of pulmonary vascular resistance, leading to overfilling, elevation of venous pressure, congestion in various organs, and edema in the venous system. This study aimed to investigate whether PH is a risk factor for deep vein thrombosis (DVT) of the lower extremities after hip and knee replacement surgery. METHODS A total of 238 patients who received joint replacement of lower extremities in our department of orthopedics from January 2009 to January 2012 were examined by echocardiography and Color Doppler flow imaging (CDFI) of the lower extremities. Based on pulmonary artery pressure (PAP), the patients were divided into a normal PAP group (n=214) and PH group (n=24). All the patients were re-examined by CDFI during post-operative care. RESULTS Among the 238 patients, 18 had DVT in the lower extremities after the operation. DVT total incidence rate was 7.56% (18/238). In the PH group, 11 patients had DVT (45.83%, 11/24), but in the normal PAP group, only 7 had DVT (3.27%, 7/214). The incidence of DVT was significantly lower in the normal PAP group than in the PH group (P<0.01). In addition, there was a positive correlation between PAP and the incidence of DVT. CONCLUSION PH could be a high-risk factor for the occurrence of DVT in patient's lower extremities after joint replacement surgeries.

RESUMO OBJETIVO A hipertensão pulmonar (HP) indica elevação progressiva da resistência vascular pulmonar, levando ao excesso de enchimento, elevação da pressão venosa, congestão em vários órgãos e edema no sistema venoso. Este estudo teve como objetivo investigar se a HP é um fator de risco para trombose venosa profunda (TVP) das extremidades inferiores após cirurgia de prótese de quadril e joelho. MÉTODOS Um total de 238 pacientes que receberam a substituição da articulação das extremidades inferiores em nosso departamento de ortopedia de janeiro de 2009 a junho de 2012 foi examinado por ecocardiograma e fluxo de imagem Doppler colorido (CDFI) dos membros inferiores. De acordo com a pressão arterial pulmonar (PAP), os pacientes foram divididos em grupo PAP normal (n=214) e grupo PH (n=24). Todos os pacientes foram reexaminados por CDFI durante os cuidados pós-operatórios. RESULTADOS Entre os 238 pacientes, 18 pacientes tiveram TVP nas extremidades inferiores após a operação. A taxa de incidência total de TVP foi de 7,56% (18/238). No grupo PH, 11 pacientes tiveram TVP (45,83%, 11/24), mas no grupo PAP normal, apenas sete pacientes tiveram TVP (3,27%, 7/214). A incidência de TVP foi significativamente menor no grupo PAP normal do que no grupo PH (P<0,01). Além disso, houve uma correlação positiva entre a PAP e a incidência de TVP. CONCLUSÃO A HP poderia ser um fator de alto risco para a ocorrência de TVP nas extremidades inferiores do paciente após cirurgias de substituição articular.

Angioplastía con balón para el tratamiento de la hipertensión arterial pulmonar por enfermedad tromboembólica crónica. Resultados hemodinámicos y clínicos inmediatos / Balloon pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension

Background: Balloon pulmonary angioplasty (BPA) is a therapeutic alternative for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Aim: To report the initial experience with the "refined BPA technique" with the use of intravascular images. Patients and Methods: Between June 2015 and June 2016 we selected fourteen patients with CTEPH who were considered candidates for BPA. Lesions targeted for treatment were further analyzed using intravascular imaging with optical frequency domain imaging (OFDI). We report the immediate hemodynamic results and four weeks of follow-up of the first eight patients of this series. Results: We performed 16 BPA in eight patients aged 61 ± 14 years (88% women). Mean pulmonary artery pressure (PAPm) was 48.6 ± 5.8 mmHg. Success was achieved in seven patients (88%). A mean of 2.3 segments per patient were intervened in 11 sessions (1.6 sessions/ patient). Only one patient developed lung reperfusion injury. No mortality was associated with the procedure. After the last BPA session, PAPm decreased to 37.4 ± 8.6 mmHg (p=0.02). Pulmonary vascular resistance (RVP) decreased from 858,6 ± 377,0 at baseline to 516,6 ± 323,3 Dynes/sec/cm−5 (p<0.01) and the cardiac index increased from 2.4±0.6 at baseline to 2.8±0.3 L/min/m2 (p=0.01). At 4 weeks after the last BPA, WHO functional class improved from 3.3±0.5 to 2.5±0.5 (p<0,01) and six minutes walking distance from 331±92 to 451±149 m (p=0.01). Conclusions: BPA guided by OFDI for the treatment of inoperable CTEPH patients is a safe alternative with excellent immediate hemodynamic and clinical results.

Angioplastia percutánea con balón en hipertensión pulmonar tromboembólica crónica / Percutaneous balloon angioplasty for treatment of chronic thromboembolic pulmonary hypertension

La hipertensión pulmonar tromboembólica crónica se caracteriza por la presencia de material trombótico organizado dentro de las arterias pulmonares que genera elevación de la resistencia vascular pulmonar, insuficiencia cardíaca derecha y, eventualmente, la muerte. El tratamiento de elección es la tromboendarterectomía pulmonar, que suele ser curativa si la obstrucción es proximal. En algunos casos este tratamiento no es posible y surge como alternativa la angioplastia pulmonar con balón (APB), que está generando creciente interés. Se presentan tres casos de pacientes con hipertensión pulmonar tromboembólica a los que por diferentes circunstancias no pudo tratarse con tromboendarterectomía y se realizó APB comprobándose, en los tres casos, mejoría de la clase funcional, prueba de la caminata de seis minutos, además de parámetros hemodinámicos y angiográficos.

Chronic thromboembolic pulmonary hypertension is characterized by the presence of organized thrombotic material in the pulmonary arteries which causes elevation of the pulmonary vascular resistance, right heart failure, and death if not treated. Pulmonary thromboendarterectomy is the treatment of choice and can be curative when the obstruction is proximal. There are cases in which this therapy is not possible, and pulmonary angioplasty is a therapeutic alternative of growing interest. We present our experience with three patients diagnosed with chronic thromboembolic pulmonary hypertension in whom pulmonary endarterectomy was not possible and pulmonary angioplasty was performed. All patients showed improvement of functional class, six-minute walk distance, and hemodynamic as well as angiographic parameters.

Pulmonary hypertension evaluation by Doppler echocardiogram in children and adolescents with mouth breathing syndrome / Avaliação de hipertensão pulmonar pela ecodopplercardiografia em crianças e adolescentes com síndrome do respirador oral

Abstract Introduction: Adenotonsillar hyperplasia (ATH) and allergic rhinitis (AR) are the most common causes of upper airway obstruction in children. Such diseases, by affecting the upper airways, can cause chronic alveolar hypoventilation, pulmonary vasoconstriction and pulmonary hypertension, which in some cases, are irreversible. Objective: This cross-sectional study aimed to evaluate the prevalence of pulmonary hypertension in two groups of mouth-breathing (MB) 2-12 years old children with ATH and isolated allergic rhinitis, through Doppler echocardiography. Methods: 54 patients with ATH and indications for adenoidectomy and/or tonsillectomy and 24 patients with persistent allergic rhinitis were selected and submitted to Doppler echocardiography. The Systolic Pulmonary Artery Pressure (SPAP) was determined by tricuspid regurgitation and the Mean Pulmonary Artery Pressure (MPAP) was calculated from the SPAP. Similar measurements were carried out in 25 nasal breathing (NB) individuals. Results: The mean MPAP and SPAP were higher in the MB than in the NB group (17.62 ± 2.06 [ATH] and 17.45 ± 1.25 [AR] vs. 15.20 ± 2.36 [NB] mmHg, p < 0.005, and 25.61 ± 3.38 [ATH] and 25.33 ± 2.06 [AR] vs. 21.64 ± 3.87 [NB] mmHg, p < 0.005, respectively) and the mean acceleration time of pulmonary flow trace (Act) was higher in the NB than in the MB group (127.24 ± 12.81 [RN] vs. 114.06 ± 10.63 ms [ATH] and 117.96 ± 10.28 [AR] MS [AR]; p < 0.0001). Conclusion: None of the MB children (ATH and AR) met the PH criteria, although individuals with both ATH and isolated AR showed significant evidence of increased pulmonary artery pressure by Doppler echocardiography in relation to NB individuals. No differences were observed between the ATH and AR groups.

Resumo Introdução: A hiperplasia adenotonsilar (HAT) e a rinite alérgica (RA) consistem nas causas mais comuns de obstrução de vias aéreas superiores em crianças. Tais afecções, ao comprometer a via aérea superior, podem ocasionar hipoventilação alveolar crônica, vasoconstrição pulmonar e hipertensão pulmonar, em alguns casos irreversível. Objetivo: Este estudo transversal objetivou avaliar a prevalência de hipertensão arterial pulmonar em dois grupos de crianças respiradoras orais (RO): com HAT e rinite alérgica isolada, de 2 a 12 anos, por meio de exame ecodopplercardiográfico. Método: Foram selecionados e submetidos à ecodopplercardiografia 54 pacientes com HAT com indicação de adenoidectomia e/ou tonsilectomia e 24 pacientes com rinite alérgica persistente. A pressão sistólica da artéria pulmonar (PSAP) foi determinada pela regurgitação tricúspide e a pressão média da artéria pulmonar (PMAP) foi calculada a partir da PSAP. Determinações similares foram feitas em 25 respiradores nasais (RN). Resultados: As médias da PMAP e da PSAP foram maiores nos grupos de RO do que nos RN (17,62 ± 2,06 [HAT] e 17,45 ± 1,25 [RA] vs. 15,20 ± 2,36 [RN] mmHg; p < 0,005; e 25,61 ± 3,38 [HAT] e 25,33 ± 2,06 [RA] vs. 21,64 ± 3,87 [RN] mmHg; p < 0,005; respectivamente) e a média do tempo de aceleração do traçado do fluxo pulmonar (TAc) foi maior nos RN que nos grupos de RO (127,24 ± 12,81 [RN] vs. 114,06 ± 10,63 ms [HAT] e 117,96 ± 10,28 [RA] MS [RA]; p < 0,0001). Conclusão: Nenhuma criança respiradora oral (HAT e RA) preencheu os critérios de HP, embora tanto os portadores de HAT quanto de RA isolada apresentaram evidências significativas de aumento da pressão arterial pulmonar pela ecodopplercardiografia em relação aos respiradores nasais. Não se observou diferença entre os grupos HAT e RA.

Comparison of transthoracic echocardiography and right heart catheterization for assessing pulmonary arterial pressure in patients with congenital or valvular heart defects

Although right heart catheterization [RHC] has acceptable accuracy for the measurement of pulmonary arterial pressure [PAP], significant risks and cost issues are worrisome. Thus, a non-invasive technique such as echocardiography for assessing PAP would clearly be of great clinical value. We aimed to compare estimated systolic PAP [SPAP] by echocardiogram with the actual RHC measurements in the two groups of congenital and valvular heart diseases [CHD and VHD, respectively], in whom pulmonary hypertension [PHT] was clinically suspected. A total of 103 consecutive patients with confirmed CHD or VHD referred to our center between January and December 2009 were studied. Participants underwent transthoracic echocardiogaphy and RHC within 4 hours of each other. The mean SPAP in the CHD group was no different measured by RHC or echo [46.49 +/- 29.04 vs. 46.45 +/- 23 mmHg, p = 0.541]. The mean SPAP in the VHD group measured by RHC was significantly higher than that measured by echo [48.70 +/- 14.50 vs. 44.90 +/- 11.0 mmHg, p = 0.041]. Fifty-one [49.5%] patients were found to have PHT at RHC. Echocardiography correctly identified 48 of these patients [sensitivity=94.1%]. Nineteen of the 52 patients without PHT on RHC were correctly identified by echocardiography [specificity= 36.5%]. The positive and negative predictive values for echocardiography in assessing the presence or absence of PHT were 59.3% and 86.4%, respectively. Integration of hemodynamic data with the eho examination can appropriately provide comprehensive assessment of PHT with high sensitivity in individual patients with congenital or valvular heart defects