Acometimento da função ventricular direita em pacientes com esclerose sistêmica e doença pulmonar intersticial: avaliação pelo doppler tecidual / Impairment of right ventricular function in patients with systemic sclerosis and interstitial lung disease: tissue doppler evaluation

Introdução: A esclerose sistêmica (ES) é uma doença autoimune do tecido conjuntivo que cursa com fibrose e disfunção microvascular. O envolvimento dos órgãos viscerais, incluindo os pulmões e o coração, é a principal causa de óbito na ES. Nesse contexto, analisamos a relação entre os parâmetros ventriculares direitos (VD) pela ecocardiografia com Doppler tecidual e o acometimento pulmonar em pacientes com ES. Métodos: Os pacientes que preencheram os Critérios de Classificação da ES de 2013 foram submetidos à ecocardiografia com Doppler tecidual para avaliação da função sistólica (fração de ejeção) ventricular esquerda (VE), enquanto a função sistólica do VD foi avaliada por meio da fração de variação de área do VD (fractional area change ­ FAC), velocidade (sistólica) do Doppler tecidual, índice de desempenho miocárdico (IDM) e excursão sistólica do plano anular tricúspide (TAPSE). A pressão sistólica pulmonar foi estimada por insuficiência tricúspide. A tomografia computadorizada de alta resolução (TCAR) de tórax avaliou a presença de fibrose pulmonar. De acordo com os resultados da TCAR, os pacientes foram divididos em 2 subgrupos: Grupo I, incluindo pacientes com fibrose pulmonar (n=26), e Grupo II sem fibrose (n=17). Resultados: Entre os 43 pacientes com ES, a maioria era do sexo feminino (86%) com idade de 51±12 anos. Todos os pacientes apresentavam função ventricular sistólica normal, avaliada pela FEVE>55% e FAC VD>35%. Não houve diferença significativa em termos de idade ou duração da doença para os grupos. Exceto pela diminuição das velocidades do Doppler tecidual em pacientes com fibrose pulmonar, todos os índices de desempenho do VD foram semelhantes. Conclusão: Em pacientes com ES e fibrose pulmonar, o Doppler tecidual identifica acometimento miocárdico longitudinal precoce do VD, apesar do desempenho sistólico radial preservado do VD.(AU)

Introduction: Systemic sclerosis (SSc) is an autoimmune tissue connective disease that courses with fibrosis and microvascular dysfunction. Involvement of the visceral organs, including the lungs and heart, is the main cause of death among patients with SSc. In this context, here we analyzed the relationship between right ventricle (RV) parameters assessed by tissue Doppler echocardiography and lung involvement in patients with SSc. Methods: Patients fulfilling the 2013 SSc Classification Criteria underwent tissue Doppler echocardiography for the assessment of left ventricular (LV) systolic function (ejection fraction) and RV fractional area change (FAC), tissue Doppler s' (systolic) velocity, myocardial performance index, and tricuspid annular plane systolic excursion for the assessment of RV systolic function. Pulmonary systolic pressure was estimated using tricuspid regurgitation. Chest high-resolution computed tomography was used to evaluate the presence of pulmonary fibrosis. The patients were divided into two subgroups accordingly: Group I, patients with pulmonary fibrosis (n=26); and Group II, those without fibrosis (n=17). Results: Among the 43 patients with SSc, most were female (86%), and the mean age was 51 ± 12 years. All patients had normal systolic ventricular function as evidenced by an LV ejection fraction > 55% and an RV FAC > 35%. No significant intergroup difference was noted in age or disease duration. Except for a decreased tissue Doppler s' velocity in patients with lung fibrosis, all indexes of RV performance were similar. Conclusion: In patients with SSc and pulmonary fibrosis, tissue Doppler identified early RV longitudinal myocardial involvement despite preserved RV radial systolic performance.(AU)

Pulsatile varicose veins: an uncommon presentation of a common condition / Varizes pulsáteis: uma apresentação incomum de uma condição comum

Abstract Varicose veins of the lower limbs are common. However, pulsatile varicose veins are unusual. They could be an indicator of a sinister underlying pathology, such as severe cardiac dysfunction. It is easy to miss these rare cases during clinical workup, which can result in futile treatment with potentially dangerous consequences. In this report, we describe 2 cases of pulsatile varicose veins that highlight different etiologies and management strategies for this condition.

Resumo Varizes dos membros inferiores são comuns. Entretanto, varizes pulsáteis são raras, podendo ser indicadoras de uma patologia subjacente sombria como disfunção cardíaca grave. É fácil deixar passar esses casos raros durante exames clínicos, o que pode resultar em tratamento fútil com consequências potencialmente perigosas. Neste relato, descrevemos dois casos de varizes pulsáteis que evidenciam as diferentes etiologias e estratégias de manejo para essa condição.

Varizes pulsáteis simulando aneurisma de artéria femoral: relato de caso / Pulsatile varicose veins simulating femoral artery aneurysm: case report

Resumo A regurgitação tricúspide severa é citada como fator associado ao desenvolvimento ou à recidiva de varizes em membros inferiores as quais podem, retrogradamente, apresentar um fluxo pulsátil. O diagnóstico etiológico diferencial desse achado ultrassonográfico deve ser feito com a pesquisa de fístulas arteriovenosas, tendo em vista as diferentes formas de tratamento. Dada a complexidade do estado geral dos pacientes com regurgitação tricúspide, a escolha do tratamento das varizes pulsáteis deve ser individualizada, fruto de uma avaliação multidisciplinar. Fazem parte do arsenal terapêutico todas as técnicas habitualmente empregadas no tratamento de varizes, bem como a associações entre elas a serem escolhidas levando-se em conta a gravidade das manifestações clínicas e o risco cardiovascular envolvido. Relatamos um caso de varizes pulsáteis secundárias a regurgitação tricúspide, diagnosticado durante a suspeita primária de aneurisma de artéria femoral em paciente de 73 anos, com a classificação clínica da doença venosa (CEAP) 4a, oligossintomática, tratada com medidas posturais e elastocompressão.

Abstract Severe tricuspid regurgitation is mentioned as a factor associated with development or recurrence of varicose veins in the lower limbs and may present with retrograde pulsatile flow. Differential etiological diagnosis of this ultrasound finding must include investigation of arteriovenous fistulas, since the treatment methods are different. Given the complexity of the general condition of patients with tricuspid regurgitation, treatment for pulsatile varices should be chosen on a case-by-case basis after multidisciplinary evaluation. All of the techniques commonly used to treat varicose veins are part of the therapeutic arsenal, as well as combinations of them, taking into account the severity of clinical manifestations and the cardiovascular risk involved. We report a case of pulsatile varices secondary to tricuspid regurgitation diagnosed when investigating a primary suspicion of femoral artery aneurysm in a 73-year-old patient, CEAP 4a, oligosymptomatic, who was treated with postural measures and elastic compression.

Desplazamiento del anillo tricuspídeo en niños normales determinado mediante extrapolación de programa ecocardiográfico / Tricuspid annular displacement in normal children measured by an extrapolated echocardiographic program

Introducción: la excursión sistólica del plano lateral del anillo tricuspídeo (TAPSE) es un parámetro útil en la evaluación de la función del ventrículo derecho en pacientes pediátricos. Objetivos: mostrar los valores normales del TAPSE en niños cubanos según grupos etarios, y describir su relación con la edad, gasto del ventrículo izquierdo, tiempo de aceleración pulmonar y la fracción de eyección del ventrículo izquierdo. Métodos: se realizó un estudio prospectivo en el que se incluyeron 102 niños normales, a cuya medición del TAPSE se les realizó adaptando el programa para la mensuración de la distancia entre el punto E y el septum interventricular. Resultados: el TAPSE medio fue de 19,4 mm (DS±6) con valores medios en la primera semana de 9,5 mm hasta 21,2 a los 5 años y 24,1 en niños mayores. Se encontró correlación positiva significativa entre el TAPSE y la edad (r= 0,679) descrita por la ecuación TAPSE= 13,2787 + 5,2354 log (X). Se mostraron los valores del TAPSE en 5 grupos de edades. Se encontró también una correlación significativa entre el TAPSE, el tiempo de aceleración pulmonar y el gasto sistólico del ventrículo izquierdo. Conclusiones: existen 5 grupos etarios bien definidos, los mayores cambios del TAPSE se presentan antes de los 5 años de edad, y se encontró una relación logarítmica entre el TAPSE, la edad y el tiempo de aceleración pulmonar. Se recomienda el programa utilizado como alternativa en la medición del TAPSE

Introduction: the tricuspid annular plane systolic excursion (TAPSE) is a useful parameter to evaluate the right ventricular function in pediatric patients. Objectives: to show the normal values of TAPSE in Cuban children by age groups, and to describe their relationship with the age, the left ventricular output, the pulmonary acceleration time and the ejection fraction of the left ventricle. Methods: a prospective study included 102 normal children to whom TAPSE was measured by adapting the program for distance mensuration between point E and the interventricular septum. Results: average TAPSE was 19.4 mm (DS±6) with mean values equal to 9.5 mm in the first week up to 21.2 mm at 5 years and 24.1 in older children. There was significant positive correlation between TAPSE figures and age (r= 0.679) described in equation TAPSE= 13.2787 + 5.2354 log (X). The TAPSE values were presented in five age groups. It was also found that there was significant correlation among TAPSE, pulmonary acceleration time and systolic output of the left ventricle. Conclusions: there exist five well-defined age groups, the major changes occur before 5 years of age and log relation was found among TAPSE, age and pulmonary acceleration time. The used program is recommended as an alternative to measure TAPSE

Pericardite constritiva em paciente com passado de múltiplas trocas de valva mitral, complicada com insuficiência tricúspide grave e hemorragia cerebral por angiopatia amilóide / Constrictive pericarditis in the patient with a history of numerous mitral valve replacements, complicated by serious tricuspid regurgitation and cerebral hemorrhage for amyloid angiopathy

Paciente do sexo feminino, portadora de cardiopatia reumática, com passado de cirurgias de troca valvar mitral que desenvolveu insuficiência tricúspide grave e pericardite constritive, fez hemorragia cerebral grave por angiopatia amilóide, tendo evoluído para óbito

Left main coronary artery compression by dilated main pulmonary artery in endocardial cushion defect.

A 56-year-old man was evaluated for exertional dyspnoea. Chest X-ray showed mild cardiomegaly and a dilated main pulmonary artery. On echocardiogram he was found to have ostium primum atrial septal defect with moderate tricuspid insufficiency. Cardiac catheterization revealed an oximetry step-up of 14% at low right atrium with angiogram demonstrating a cleft in the mitral valve, an elongated left ventricular outflow tract and ventricular septal defect closed by a septal aneurysm. Coronary angiogram revealed ostial compression of the left main coronary artery with the rest of the coronary artery anatomy being normal.

Cardiac abnormalities in birth asphyxia.

Cardiac abnormalities in birth asphyxia were first recognised in the 1970s. These include (i) transient tricuspid regurgitation which is the commonest cause of a systolic murmur in a newborn and tends to disappear without any treatment unless it is associated with transient myocardial ischemia or primary pulmonary hypertension of the newborn (ii) transient mitral regurgitation which is much less common and is often a part of transient myocardial ischemia, at times with reduced left ventricular function and, therefore, requires treatment in the form of inotropic and ventilatory support (iii) transient myocardial ischemia (TMI) of the newborn. This should be suspected in any baby with asphyxia, respiratory distress and poor pulses, especially if a murmur is audible. It is of five types (A to E) according to Rowe's classification. Type B is the most severe with respiratory distress, congestive heart failure and shock. Echocardiography helps to rule out critical left ventricular obstructive lesions like hypoplastic left heart syndrome or critical aortic stenosis. ECG is very important for diagnosis of TMI, and may show changes ranging from T wave inversion in one lead to a classical segmental infarction pattern with abnormal q waves. CPK-MB may rise and echocardiogram shows impaired left ventricular function, mitral and/or tricuspid regurgitation, and at times, wall motion abnormalities of left ventricle. Ejection fraction is often depressed and is a useful marker of severity and prognosis. Treatment includes fluid restriction, inotropic support, diuretics and ventilatory resistance if required (v) persistent pulmonary hypertension of the newborn (PPHN). Persistent hypoxia sometimes results in persistence of constricted fetal pulmonary vascular bed causing pulmonary arterial hypertension with consequent right to left shunt across patent ductus arteriosus and foramen ovale. This causes respiratory tension and right ventricular failure with systolic murmur of tricuspid, and at times, mitral regurgitation. Treatment consists of oxygen and general care for mild cases, ventilatory support, ECMO and nitric oxide for severe cases. Cardiac abnormalities in asphyxiated neonates are often underdiagnosed and require a high index of suspicion. ECG and Echo help in early recognition and hence better management of these cases.

Cardiac abnormalities in birth asphyxia.

Cardiac abnormalities in birth asphyxia were first recognised in 1970s. These include (i) transient tricuspid regurgitation which is the commonest cause of a systolic murmur in a newborn and tends to disappear without any treatment unless it is associated with transient myocardial ischemia or primary pulmonary hypertension of the new born (ii) transient mitral regurgitation which is much less common and is often a part of transient myocardial ischemia, at times with reduced left ventricular function and therefore, requires treatment in the form of inotropic and ventilatory support, (iii) transient myocardial ischemia (TMI) of the newborn. This should be suspected in any baby with asphyxia, respiratory distress and poor pulses especially if a murmur is audible. It is of five types (A to E) according to Rowe's classification. Type B is the most severe with respiratory distress, congestive heart failure and shock. Echocardiography helps to rule out critical left ventricular obstructive lesions like hypoplastic left heart syndrome or critical aortic stenosis. ECG is very important for diagnosis of TMI, and may show changes ranging from T wave inversion in one lead to a classical segmental infarction pattern with abnormal q waves. CPK-MB may rise and echocardiogram shows impaired left ventricular function, mitral and/or tricuspid regurgitation, and at times, wall motion abnormalities of left ventricle. Ejection fraction is often depressed and is a useful marker of severity and prognosis. Treatment includes fluid restriction, inotropic support, diuretics and ventilatory resistance if required, (v) persistent pulmonary hypertension of the new born (PPHN). Persistent hypoxia sometimes results in persistence of constricted fetal pulmonary vascular bed causing pulmonary arterial hypertension with consequent right to left shunt across patent ductus arteriosus and foramen ovale. This causes respiratory distress and cyanosis (sometimes differential). Clinical examination also reveals evidence of pulmonary arterial hypertension and right ventricular failure with systolic murmur of tricuspid and, at times, mitral regurgitation. Treatment consists of oxygen and general care for mild cases, ventilatory support, ECMO and nitric oxide for severe cases. Cardiac abnormalities in asphyxiated neonates are often underdiagnosed and require a high index of suspicion. ECG and Echo help in early recognition and hence better management of these cases.

Beneficial effect of balloon mitral valvotomy in reducing severity of associated tricuspid regurgitation.

This study was done to find out whether successful balloon mitral valvotomy (BMV) reduces the severity of associated functional tricuspid regurgitation (TR), and if so, which variables predict this reduction. Of the 177 consecutive patients who underwent BMV, 53 were found to have functional TR. 2D echocardiography (Echo) with color Doppler was done before and 24-48 hours after BMV. Using the apical four-chamber view, the severity of TR was assessed by comparing the ratio of maximal tricuspid regurgitant jet area (TRA) to right atrial area (RAA). There was a significant reduction in TRA:RAA, after BMV (0.26 to 0.12; p < 0.05). Stepwise multiple regression analysis showed that the predictors of TR reduction were: age less than 24 years (r = 0.56, p < 0.004), cardiothoracic ratio measured on chest X-ray > 60% (r = 0.43, p < 0.002) and pre-BMV pulmonary artery systolic pressure (PASP) > 50 mm Hg (r = 0.51, p < 0.001).

Double orifice mitral valve: report of 4 cases.

Double Orifice Mitral Valve (DOMV) is a rare congenital abnormality. Reports of 4 patients with DOMV are presented. As associated anomalies, one had ostium primum atrial septal defect (ASD). Second one had Ebstein's anomaly. Third one had grade IV tricuspid regurgitation, thickened tricuspid valve and right atrial mass. Fourth one had sinus venosus ASD. Echocardiography is the gold standard investigation. Surgical treatment depends upon the mitral valve function and associated anomalies.