A Novel SPEG mutation causing congenital myopathy with fiber size disproportion and dilated cardiomyopathy with heart transplantation.

Congenital myopathies are a heterogeneous group of conditions diagnosed based on the clinical presentation, muscle histopathology and genetic defects. Recessive mutations in the SPEG gene have been described in recent years and are primarily associated with centronuclear myopathy with cardiomyopathy. In this report, we describe two Brazilian siblings, aged 13 and 6 years, with a novel homozygous mutation (c.8872 C>T:p.Arg2958Ter) in the SPEG gene leading to a congenital myopathy. In the older sibling, the muscle biopsy showed fiber size disproportion. The mean diameter of type 2 fibers (119 µm) was significantly higher than type 1 (57 µm) (P < 0,001) with a 72% prevalence of type 1 fibers. The patient also had progressive cardiomyopathy treated with heart transplantation. The present report expands the muscle histopathological findings related to mutations in the SPEG gene, including fiber size disproportion without central nuclei. Additionally, this report describes the first case of heart transplantation in a patient with SPEG mutations.

Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease. / Caracterização Histológica das Lesões da Valva Mitral de Pacientes com Cardiopatia Reumática.

BACKGROUND: The underlying mechanisms by which rheumatic heart disease (RHD) lead to severe valve dysfunction are not completely understood. OBJECTIVE: The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. METHODS: In 40 patients who underwent MV replacement due to RHD, and in 20 controls that underwent heart transplant, histological aspects of the excised MV were analyzed. Clinical and echocardiographic data were also collected. Histological analyses were performed using hematoxylin-eosin staining. Inflammation, fibrosis, neoangiogenesis, calcification and adipose metaplasia were determined. A p value<0.05 was considered to be statistically significant. RESULTS: The mean age of RHD patients was 53±13 years, 36 (90%) were female, whereas the mean age of controls was 50±12 years, similar to the cases, with the majority of males (70%). The rheumatic valve endocardium presented greater thickness than the controls (1.3±0.5 mm versus 0.90±0.4 mm, p=0.003, respectively), and a more intense inflammatory infiltrate in the endocardium (78% versus 36%; p=0.004), with predominance of mononuclear cells. Moderate to marked fibrosis occurred more frequently in rheumatic valves than in control valves (100% vs. 29%; p<0.001). Calcification occurred in 35% of rheumatic valves, especially among stenotic valves, which was associated with the mitral valve area (p=0.003). CONCLUSIONS: Despite intense degree of fibrosis, the inflammatory process remains active in the rheumatic mitral valve, even at late disease with valve dysfunction. Calcification predominated in stenotic valves and in patients with right ventricular dysfunction.

FUNDAMENTOS: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. OBJETIVO: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. MÉTODOS: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p<0,05 foram considerados estatisticamente significativos. RESULTADOS: A idade média dos pacientes com DCR foi de 53±13 anos, sendo 36 (90%) do sexo feminino, enquanto a idade média dos controles foi de 50±12 anos, semelhante aos casos, sendo a maioria do sexo masculino (70%). O endocárdio valvar reumático apresentou espessura maior que os controles (1,3±0,5 mm versus 0,90±0,4 mm, p=0,003, respectivamente), e infiltrado inflamatório mais intenso no endocárdio (78% versus 36%; p=0,004), com predominância de células mononucleares. Ocorreu fibrose moderada a acentuada mais frequentemente em válvulas reumáticas do que em válvulas controle (100% vs. 29%; p<0,001). Ocorreu calcificação em 35% das valvas reumáticas, principalmente entre as valvas estenóticas, associada à área valvar mitral (p=0,003). CONCLUSÕES: Apesar do intenso grau de fibrose, o processo inflamatório permanece ativo na valva mitral reumática, mesmo em doença tardia com disfunção valvar. A calcificação predominou em valvas estenóticas e em pacientes com disfunção ventricular direita.

Caracterização Histológica das Lesões da Valva Mitral de Pacientes com Cardiopatia Reumática / Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease

Resumo Fundamentos: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. Objetivo: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. Métodos: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p<0,05 foram considerados estatisticamente significativos. Resultados: A idade média dos pacientes com DCR foi de 53±13 anos, sendo 36 (90%) do sexo feminino, enquanto a idade média dos controles foi de 50±12 anos, semelhante aos casos, sendo a maioria do sexo masculino (70%). O endocárdio valvar reumático apresentou espessura maior que os controles (1,3±0,5 mm versus 0,90±0,4 mm, p=0,003, respectivamente), e infiltrado inflamatório mais intenso no endocárdio (78% versus 36%; p=0,004), com predominância de células mononucleares. Ocorreu fibrose moderada a acentuada mais frequentemente em válvulas reumáticas do que em válvulas controle (100% vs. 29%; p<0,001). Ocorreu calcificação em 35% das valvas reumáticas, principalmente entre as valvas estenóticas, associada à área valvar mitral (p=0,003). Conclusões: Apesar do intenso grau de fibrose, o processo inflamatório permanece ativo na valva mitral reumática, mesmo em doença tardia com disfunção valvar. A calcificação predominou em valvas estenóticas e em pacientes com disfunção ventricular direita.

Abstract Background: The underlying mechanisms by which rheumatic heart disease (RHD) lead to severe valve dysfunction are not completely understood. Objective: The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. Methods: In 40 patients who underwent MV replacement due to RHD, and in 20 controls that underwent heart transplant, histological aspects of the excised MV were analyzed. Clinical and echocardiographic data were also collected. Histological analyses were performed using hematoxylin-eosin staining. Inflammation, fibrosis, neoangiogenesis, calcification and adipose metaplasia were determined. A p value<0.05 was considered to be statistically significant. Results: The mean age of RHD patients was 53±13 years, 36 (90%) were female, whereas the mean age of controls was 50±12 years, similar to the cases, with the majority of males (70%). The rheumatic valve endocardium presented greater thickness than the controls (1.3±0.5 mm versus 0.90±0.4 mm, p=0.003, respectively), and a more intense inflammatory infiltrate in the endocardium (78% versus 36%; p=0.004), with predominance of mononuclear cells. Moderate to marked fibrosis occurred more frequently in rheumatic valves than in control valves (100% vs. 29%; p<0.001). Calcification occurred in 35% of rheumatic valves, especially among stenotic valves, which was associated with the mitral valve area (p=0.003). Conclusions: Despite intense degree of fibrosis, the inflammatory process remains active in the rheumatic mitral valve, even at late disease with valve dysfunction. Calcification predominated in stenotic valves and in patients with right ventricular dysfunction.

Proinflammatory Matrix Metalloproteinase-1 Associates With Mitral Valve Leaflet Disruption Following Percutaneous Mitral Valvuloplasty.

Mitral regurgitation (MR) is a major complication of the percutaneous mitral valvuloplasty (PMV). Despite high technical expertise and cumulative experience with the procedure, the incidence rate of severe MR has not decreased. Although some of MR can be anticipated by echocardiographic analysis; leaflet tearing, which leads to the most dreaded type of MR, remains unpredictable. Irregular valvular collagen remodeling is likely to compromise tissue architecture and increase the tearing risk during PMV balloon inflation. In this study, we evaluated histological and molecular characteristics of excised mitral valves from patients with rheumatic mitral stenosis (MS) who underwent emergency surgery after PMV due to severe MR caused by leaflet tear. Those findings were compared with patients who underwent elective mitral valve replacement surgery owing to severe MS, in whom PMV was not indicated. In vitro assay using peripheral blood mononuclear cells was performed to better understand the impact of the cellular and molecular alterations identified in leaflet tear mitral valve specimens. Our analysis showed that focal infiltration of inflammatory cells contributes to accumulation of MMP-1 and IFN-γ in valve leaflets. Moreover, we showed that IFN-γ increase the expression of MMP-1 in CD14+ cells (monocytes) in vitro. Thus, inflammatory cells contribute to unevenly remodel collagen resulting in variable thickening causing abnormalities in leaflet architecture making them more susceptible to laceration.

Heart Transplantation for Chagas Cardiomyopathy

Abstract Heart transplantation (HT) is an established treatment for patients with advanced heart failure (HF). Chagas disease (CD), caused by the Trypanosoma cruzi (T.cruzi) is an important cause of HF in Latin America. Considering CD is a chronic infectious disease, the use of immunosuppressive therapy after HT can reactivate T. cruzi infection and compromise outcomes. Early diagnosis and treatment of this complication is extremely important, which requires knowledge, experience, and a high degree of suspicion by transplant physicians. Furthermore, with the international immigration of people, CD is no longer exclusive to Latin America, since a large number of immigrants with T. cruzi infection are living in non-endemic countries. This phenomenon represents not only a new global epidemiological problem, but also a challenge for transplant teams. This review aims to discuss the peculiarities of HT in the context of CD, with a focus on reactivation of the infection, clinical manifestations, etiological treatment of T. cruzi and differential diagnosis with allograft rejection, among HT recipients.

Mitral Regurgitation After Percutaneous Mitral Valvuloplasty: Insights Into Mechanisms and Impact on Clinical Outcomes.

OBJECTIVES: The aim of this study was to assess the incidence, mechanisms, and outcomes of mitral regurgitation (MR) after percutaneous mitral valvuloplasty (PMV). BACKGROUND: Significant MR continues to be a major complication of PMV, with a wide range in clinical presentation and prognosis. METHODS: Consecutive patients with mitral stenosis undergoing PMV were prospectively enrolled. MR severity was evaluated by using quantitative echocardiographic criteria, and its mechanism was characterized by 3-dimensional transesophageal echocardiography, divided broadly into 4 categories based on the features contributing to the valve damage. B-type natriuretic peptide levels were obtained before and 24 h after the procedure. Endpoints estimated cardiovascular death or mitral valve (MV) replacement due to predominant MR. RESULTS: A total of 344 patients, ages 45.1 ± 12.1 years, of whom 293 (85%) were women, were enrolled. Significant MR after PMV was found in 64 patients (18.6%). The most frequent mechanism of MR was commissural, which occurred in 22 (34.4%) patients, followed by commissural with posterior leaflet in 16 (25.0%), leaflets at central scallop or subvalvular damage in 15 (23.4%), and central MR in 11 (17.2%). During the mean follow-up period of 3 years (range 1 day to 10.6 years), 60 patients reached the endpoint. The event-free survival rates were similar among patients with mild or commissural MR, whereas patients with damaged central leaflet scallop or subvalvular apparatus had the worst outcome, with an event-free survival rate at 1 year of only 7%. Long-term outcome was predicted by net atrioventricular compliance (Cn) at baseline and post-procedural variables, including valve area, mean gradient, and magnitude of decrease in B-type natriuretic peptide levels, adjusted for the mechanism of MR. CONCLUSIONS: Significant MR following PMV is a frequent event, mainly related to commissural splitting, with favorable clinical outcome. Parameters that express the relief of valve obstruction and the mechanism by which MR develops were predictors of long-term outcomes.

Outcomes of infective endocarditis in the current era: Early predictors of a poor prognosis.

BACKGROUND: The early identification of patients at risk of complications of infective endocarditis (IE) using parameters obtained as part of routine practice is essential for guiding clinical decision-making. This study aimed to identify a parameter at hospital admission that predicts the outcome, adding value to other well-known factors of a poor prognosis in IE. METHODS: Two hundred and three patients with IE were included in this study. Clinical evaluation, echocardiography, blood cultures, and routine laboratory tests were performed at hospital admission. The endpoint was in-hospital mortality. RESULTS: The mean age of the patients was 48.2±16.6 years; 62% were male and 38% had rheumatic heart disease. During treatment, cardiac surgery was performed in 111 patients (55%), and the overall in-hospital mortality rate was 32%. In the multivariable analysis, the independent predictors of death were age (odds ratio (OR) 1.07, 95% confidence interval (CI) 1.02-1.13), C-reactive protein (CRP) at hospital admission (OR 1.12, 95% CI 1.04-1.21), length of the vegetation at diagnosis (OR 1.15, 95% CI 1.03-1.28), development of heart failure (OR 6.43, 95% CI 2.14-19.33), and embolic events during antimicrobial therapy (OR 12.14, 95% CI 2.11-71.89). CONCLUSIONS: An elevated CRP level at hospital admission and vegetation length at diagnosis were strong predictors of in-hospital mortality in IE, independent of other prognostic parameters, specifically taking into account patient characteristics and complications during therapy.

Associated Clinical and Laboratory Markers of Donor on Allograft Function After Heart Transplant.

INTRODUCTION: Primary graft dysfunction is a major cause of mortality after heart transplantation. OBJECTIVE: To evaluate correlations between donor-related clinical/biochemical markers and the occurrence of primary graft dysfunction/clinical outcomes of recipients within 30 days of transplant. METHODS: The prospective study involved 43 donor/recipient pairs. Data collected from donors included demographic and echocardiographic information, noradrenaline administration rates and concentrations of soluble tumor necrosis factor receptors (sTNFR1 and sTNFR2), interleukins (IL-6 and IL-10), monocyte chemoattractant protein-1, C-reactive protein and cardiac troponin I. Data collected from recipients included operating, cardiopulmonary bypass, intensive care unit and hospitalization times, inotrope administration and left/right ventricular function through echocardiography. RESULTS: Recipients who developed moderate/severe left ventricular dysfunction had received organs from significantly older donors (P =0.020). Recipients from donors who required moderate/high doses of noradrenaline (>0.23 µg/kg/min) around harvesting time exhibited lower post-transplant ventricular ejection fractions (P =0.002) and required longer CPB times (P =0.039). Significantly higher concentrations of sTNFR1 (P =0.014) and sTNFR2 (P =0.030) in donors were associated with reduced intensive care unit times (≤5 days) in recipients, while higher donor IL-6 (P =0.029) and IL-10 (P =0.037) levels were correlated with reduced hospitalization times (≤25 days) in recipients. Recipients who required moderate/high levels of noradrenaline for weaning off cardiopulmonary bypass were associated with lower donor concentrations of sTNFR2 (P =0.028) and IL-6 (P =0.001). CONCLUSION: High levels of sTNFR1, sTNFR2, IL-6 and IL-10 in donors were associated with enhanced evolution in recipients. Allografts from older donors, or from those treated with noradrenaline doses >0.23 µg/kg/min, were more frequently affected by primary graft dysfunction within 30 days of surgery.

Associated clinical and laboratory markers of donor on allograft function after heart transplant

Abstract Introduction: Primary graft dysfunction is a major cause of mortality after heart transplantation. Objective: To evaluate correlations between donor-related clinical/biochemical markers and the occurrence of primary graft dysfunction/clinical outcomes of recipients within 30 days of transplant. Methods: The prospective study involved 43 donor/recipient pairs. Data collected from donors included demographic and echocardiographic information, noradrenaline administration rates and concentrations of soluble tumor necrosis factor receptors (sTNFR1 and sTNFR2), interleukins (IL-6 and IL-10), monocyte chemoattractant protein-1, C-reactive protein and cardiac troponin I. Data collected from recipients included operating, cardiopulmonary bypass, intensive care unit and hospitalization times, inotrope administration and left/right ventricular function through echocardiography. Results: Recipients who developed moderate/severe left ventricular dysfunction had received organs from significantly older donors (P =0.020). Recipients from donors who required moderate/high doses of noradrenaline (>0.23 µg/kg/min) around harvesting time exhibited lower post-transplant ventricular ejection fractions (P =0.002) and required longer CPB times (P =0.039). Significantly higher concentrations of sTNFR1 (P =0.014) and sTNFR2 (P =0.030) in donors were associated with reduced intensive care unit times (≤5 days) in recipients, while higher donor IL-6 (P =0.029) and IL-10 (P =0.037) levels were correlated with reduced hospitalization times (≤25 days) in recipients. Recipients who required moderate/high levels of noradrenaline for weaning off cardiopulmonary bypass were associated with lower donor concentrations of sTNFR2 (P =0.028) and IL-6 (P =0.001). Conclusion: High levels of sTNFR1, sTNFR2, IL-6 and IL-10 in donors were associated with enhanced evolution in recipients. Allografts from older donors, or from those treated with noradrenaline doses >0.23 µg/kg/min, were more frequently affected by primary graft dysfunction within 30 days of surgery.

Determinants of prolonged length of hospital stay after cardiac surgery: impact of rheumatic heart disease / Determinantes de internação hospitalar prolongada após cirurgia cardíaca: impacto de cardiopatia reumática

BACKGROUND: Rheumatic heart disease remains a major health problem in developing countries. Several factors contribute to valve-related morbidity after cardiac surgery, but the role of rheumatic etiology of valve disease is not well defined. This study was designed to determine the additional value of rheumatic valve disease in predicting morbidity after cardiac surgery in the current era of heart valve disease treatment. METHOD: This study prospectively included 164 patients for cardiac surgery from June 2010 to June 2011. The outcome was prolonged length of stay, defined as a length of stay greater than or equal to the 75th percentile for length of stay for each operation, including the day of discharge. RESULTS: Rheumatic heart disease was present in 32 patients (20%) and all rheumatic patients underwent valve replacement. Rheumatic heart disease patients were younger with less comorbidities compared to non-rheumatic patients, with most (63%) having had previous surgery. Forty-one patients were classified as having a prolonged hospital length of stay; 11 (34%) patients with rheumatic and 30 (23%) non-rheumatic fever. Rheumatic heart disease was not associated with prolonged hospital stay in the univariate analysis; however, after adjustment for other factors including infectious endocarditis, surgery duration, mechanical ventilation time, EuroSCORE, and postoperative pneumonia, it was found to be a predictor of prolonged hospitalization. CONCLUSION: This study demonstrates that rheumatic heart disease was an important factor associated with prolonged hospital, after adjustment for well-known risk factors of morbidity after cardiac surgery. Rheumatic fever is still prevalent among the patients who underwent to cardiac surgery in the current age, contributing to increase the postoperative morbidity.

OBJETIVO: A doença cardíaca reumática continua a ser um problema grave de saúde nos países em desenvolvimento. Vários fatores contribuem para a morbidade relacionada com a cirurgia valvar cardíaca, mas o papel da etiologia reumática das valvopatias não está bem definido. Este estudo foi desenhado para determinar participação adicional de valvopatias reumáticas na previsão de morbidade após cirurgia cardíaca na era atual de tratamento da doença. MÉTODOS: Este estudo incluiu prospectivamente 164 pacientes submetidos a cirurgia cardíaca, entre junho de 2010 a junho de 2011. O resultado medido foi a duração da estadia prolongada, definido como tempo de permanência maior ou igual ao percentil 75 para a duração da estada para cada operação, incluindo a dia da alta. RESULTADOS: A cardiopatia reumática esteve presente em 32 pacientes (20%) e em todos os pacientes submetidos à substituição da válvula. Pacientes com doenças cardíacas reumáticas eram mais jovens e com menos comorbidades comparados com pacientes não-reumáticos; a maioria deles (63%) tinha tido cirurgia prévia. Quarenta e um pacientes foram classificados como tendo um tempo de permanência hospitalar prolongado; 11 (34%) pacientes com doenças reumáticas e 30 (23%) com doenças não-reumáticas. A doença reumática não se apresentou associada com período de internação prolongado, na análise univariada; No entanto, após o ajuste para outros fatores, incluindo endocardite infecciosa, duração da cirurgia, tempo de ventilação mecânica, EuroSCORE, e pneumonia no pós-operatório, a doença reumática revelou-se um preditor de hospitalização prolongada. CONCLUSÕES: Este estudo demonstra que a doença cardíaca reumática é um importante fator associado com internação prolongada, após o ajuste para fatores de risco bem conhecidos de morbidade após cirurgia cardíaca. A febre reumática ainda é prevalente entre os pacientes que se submeteram à cirurgia cardíaca na época atual, contribuindo para aumentar a morbidade pós-operatória.

Indicators of surgical treatment of patent ductus arteriosus in preterm neonates in the first week of life / Indicadores para o tratamento cirúrgico na persistência do ducto arterial em neonatos prematuros na primeira semana de vida

OBJECTIVE: To identify clinical and echocardiographic indicators of the necessity for early surgical closure of patent ductus arteriosus in preterm neonates. METHODS: The prospective study was conducted at the Neonatal Unit of Hospital Municipal Odilon Behrens between 2006 and 2010. The study population comprised 115 preterm neonates diagnosed with patent ductus arteriosus in the first week after birth, of whom 55 (group S) were submitted to clinical and or surgical closure and 60 (group NS) received non-surgical treatment. The parameters analyzed were birth weight, diameter of the ductus arteriosus (DAD), left atrial-to-aortic root diameter ratio (LA:Ao), the quotient of DAD² and birth weight (mm²/kg), and ductal shunting. RESULTS: The study population comprised 58 males and 57 females. The average birth weight of group S (924 ± 224.3 g) was significantly (P=0.049) lower than that of group NS (1012.3 ± 242.8 g). The probability of the preterm neonates being submitted to surgical closure was 62.1% (P=0.006) when the DAD2/birth weight index was > 5 mm²/kg, 72.2% (P=0.001) when the LA:Ao ratio was > 1.5, and 61.2% when ductal shunting was high (P=0.025). CONCLUSION: The parameters DAD²/birth weight index > 5 mm²/kg, LA:Ao ratio > 1.5 and high ductal shunting were statistically significant indicators (P<0.05) of the need for surgical closure of patent ductus arteriosus in low birth weight preterm neonates. Moreover, when an LA:Ao ratio > 1.5 was associated with the occurrence of shock, the probability of surgical closure increased to 78.4%.

OBJETIVO: Identificar parâmetros clínicos e ecocardiográficos para a indicação do tratamento cirúrgico precoce da persistência do ducto arterial. MÉTODOS: Esse estudo prospectivo foi conduzido na Unidade Neonatal do Hospital Municipal Odilon Behrens entre 2006 e 2010. A população estudada compreendeu 115 neonatos prematuros diagnosticados com persistência do ducto arterial na primeira semana após o nascimento, dos quais 55 (grupo S) foram submetidos ao tratamento clínico e ou cirúrgico e 60 (grupo NS) ao tratamento clínico. Os parâmetros analisados foram peso ao nascer, diâmetro do ducto arterial (DAD), relação diâmetro do átrio esquerdo pelo diãmetro da aorta (AE/Ao), índice DAD2/peso ao nascer e fluxo no ducto. RESULTADOS: O estudo abrangeu 58 pacientes do sexo masculino e 57 do feminino. O peso médio ao nascer do grupo S (924 ± 224,3 g) foi significativamente (P=0,049) menor do que do grupo NS (1012,3 ± 242,8 g). A probabilidade dos neonatos prematuros serem submetidos à cirurgia foi 62.1% (P=0,006) quando o índice DAD2/peso ao nascer era > 5 mm2/kg, 72,2% (P=0,001) quando a razão LA:Ao era > 1,5 e 61,2% (P=0,025) quando o fluxo no ducto era alto. CONCLUSÃO: Os parâmetros DAD²/peso ao nascer > 5 mm²/kg, razão LA:Ao > 1,5 e alto fluxo no ducto foram preditores estatisticamente significativos (P<0,05) da necessidade de fechamento cirúrgico do persistência do ducto arterial em neonatos prematuros com baixo peso ao nascer. Adicionalmente, quando a razão LA:Ao > 1,5 estava associada ao choque, a probabilidade de tratamento cirúrgico aumentou para 78,4%.

Indicators of surgical treatment of patent ductus arteriosus in preterm neonates in the first week of life.

OBJECTIVE: To identify clinical and echocardiographic indicators of the necessity for early surgical closure of patent ductus arteriosus in preterm neonates. METHODS: The prospective study was conducted at the Neonatal Unit of Hospital Municipal Odilon Behrens between 2006 and 2010. The study population comprised 115 preterm neonates diagnosed with patent ductus arteriosus in the first week after birth, of whom 55 (group S) were submitted to clinical and or surgical closure and 60 (group NS) received non-surgical treatment. The parameters analyzed were birth weight, diameter of the ductus arteriosus (DAD), left atrial-to-aortic root diameter ratio (LA:Ao), the quotient of DAD² and birth weight (mm²/kg), and ductal shunting. RESULTS: The study population comprised 58 males and 57 females. The average birth weight of group S (924 ± 224.3 g) was significantly (P=0.049) lower than that of group NS (1012.3 ± 242.8 g). The probability of the preterm neonates being submitted to surgical closure was 62.1% (P=0.006) when the DAD2/birth weight index was > 5 mm²/kg, 72.2% (P=0.001) when the LA:Ao ratio was > 1.5, and 61.2% when ductal shunting was high (P=0.025). CONCLUSION: The parameters DAD²/birth weight index > 5 mm²/kg, LA:Ao ratio > 1.5 and high ductal shunting were statistically significant indicators (P<0.05) of the need for surgical closure of patent ductus arteriosus in low birth weight preterm neonates. Moreover, when an LA:Ao ratio > 1.5 was associated with the occurrence of shock, the probability of surgical closure increased to 78.4%.

Terminal ischemic cardiomyopathy associated with complication of stenting in the treatment of acute myocardial infarction.

A serious complication such as dissection of the left main coronary artery, with significant reduction in coronary blood flow by the true light, requires quick action. Therefore, the immediate choice of stent with appropriate length and size to treat the complication is necessary.

A large pericardial cyst presenting with compression of the right-side cardiac chambers.

Pericardial cysts are rare, usually congenital. Cysts frequently occur in the right cardiophrenic angle and their diagnosis is usually suspected after an abnormal chest X ray. The present case report shows a case of pericardial cyst with atypical radiographic aspect in an athletic patient who presented clinical with symptoms of right ventricular failure. The diagnosis was suggested by echocardiogram and subsequently was confirmed by pathologic examination.

Pulmonary pressure checked by echocardiography in chagasic patients on the heart transplant waiting list.

INTRODUCTION: The patients suffering heart failure develop an increase in pulmonary pressure because of a retrograde mechanism. The pulmonary hypertension is a prognostic marker. OBJECTIVE: The aim of this study is to correlate pulmonary hypertension measured by echocardiogram versus catheterization in pre-heart transplant patients on waiting list. METHODS: Data from 90 patients of the Clinical Hospital UFMG were collected between 2004 and 2009. All the patients took an echo and catheterization as an integral part of pre-heart transplant. Mean age was 45.5 years old, 68 (75.6%) male. Fourty-two (46.7%) were Chagas' disease patients, 32 (35.6%) presented idiopathic dilated cardiomyopathy, 10 (11.1%) had ischemic cardyomiopathy. RESULTS: The mean eco-PASP was 45 ± 12mmHg). The mean cat-PASP was 47 ±14mmHg. The eco-PASP-Chagas was 41.7 ±12,5 mmHg and non-Chagas 47.6 ±12.8 mmHg P=0.04. The cat-PASP-Chagas was 46 ±12.1 mmHg and non-Chagas 48.7 ±12.8 mmHg P=0.43. Eight patients had cat-PASP>60. The correlation between eco-PASP and cat-PASP in Chagas' patients was r=0.45; P=0.008 and in the non-Chagas was r=0.66; P<0.001. The eco-PASP-Chagas>32,5mmHg has a sensitivity of 79% and specificity of 75% to diagnose PH, with an area under the curve of 0.819. The eco-PASP-non-Chagas>35.5 mmHg has a sensitivity of 82% and a specificity of 70% to diagnose PH, with an area under the curve of 0.776. CONCLUSIONS: There is a good correlation between eco-PASP and cat-PASP (r=0.54) in pre-heart transplant patients. The eco-PASP was lower in the Chagas' group. The echocardiogram is an important method to diagnosis and control pulmonary pressure in pre-heart transplant, specifically in Chagas' patients. The catheterization is still important to evaluate pulmonary reactivity during vasodilation test.

Grande cisto pericárdico manifestando-se com compressão das câmaras cardíacas direitas / A large pericardial cyst presenting with compression of the right-side cardiac chambers

Cistos pericárdicos são raros, comumente congênitos, com localização mais frequente no ângulo cardiofrênico direito. O diagnóstico é suspeitado pela radiografia de tórax anormal. O presente relato descreve um caso de cisto pericárdico com aspecto radiográfico atípico, em um paciente atleta, manifestando-se clinicamente com sintomas de insuficiência ventricular direita. O diagnóstico foi sugerido pelo ecocardiograma transesofágico e confirmado pelo estudo anatomopatológico.

Pericardial cysts are rare, usually congenital. Cysts frequently occur in the right cardiophrenic angle and their diagnosis is usually suspected after an abnormal chest X ray. The present case report shows a case of pericardial cyst with atypical radiographic aspect in an athletic patient who presented clinical with symptoms of right ventricular failure. The diagnosis was suggested by echocardiogram and subsequently was confirmed by pathologic examination.

Large atrial myxoma causing mitral obstruction and severe pulmonary hypertension.

Myxoma is the most common type of cardiac tumor, accounting for 30-50% of all primary cardiac tumors. Clinically, patients usually present with at least one of the classic triad of obstructive cardiac, embolic, and constitutional signs. The case is reported of a large atrial myxoma in the left atrium which caused mitral valve obstruction, leading to severe pulmonary hypertension and right heart failure, mimicking rheumatic mitral stenosis. Postoperatively, a fall occurred in the patient's pulmonary artery pressure and she showed clinical improvement.

Pressão pulmonar aferida pela ecocardiografia em pacientes chagásicos indicados para transplante cardíaco / Pulmonary pressure by echocardiophy in chagasic patients on heart transplant waiting list

INTRODUÇÃO: O paciente com insuficiência cardíaca desenvolve aumento da pressão pulmonar por mecanismo retrógrado e a hipertensão arterial pulmonar (HP) é um marcador de mau prognóstico. OBJETIVO: Correlacionar pressão pulmonar ao ecogardiograma (eco) e ao cateterismo, em pacientes em lista de espera para transplante cardíaco (TC), especialmente nos chagásicos. MÉTODOS: Avaliamos 90 pacientes no HC-UFMG entre 2004 e 2009. Todos realizaram cateterismo e eco no pré-transplante. A idade média foi de 45,5 anos, sendo 68(75,6%) homens, 42(46,7%) chagásicos, 32(35,6%) portadores de miocardiopatia dilatada e 10(11,1%) isquêmicos. RESULTADOS: A eco-PSAP (pressão sistólica arterial pulmonar) média foi de 45 ± 12mmHg. A cat-PSAP média foi de 47 ± 14mmHg. A eco-PSAP-chagásicos foi 41,7 ±12,5 mmHg e não-chagásicos, 47,6 ±12,8 mmHg P=0,04. A cat-PSAP-chagásicos foi de 46 ±12,1 mmHg e não-chagásicos 48,7±12,8mmHg; P=0,43. Oito pacientes apresentavam cat-PSAP>60. A correlação entre a eco-PSAP e o cat-PSAP nos chagásicos foi r=0,45, P=0,008 e nos não-chagásicos de r=0,66, P<0,001. A eco-PSAP-chagásico >32,5mmHg tem uma sensibilidade de 79% e especificidade de 75% para diagnosticar HP, com área sob a curva ROC de 0,819. A eco-PSAP-não chagásico>35,5 mmHg tem sensibilidade de 82% e especificidade de 70% para HP, com área sob a curva ROC de 0,776. CONCLUSÕES: Há boa correlação entre a eco-PSAP e a cat-PSAP (r=0,54) entre os pacientes em fila de espera. A eco-PSAP foi menor no grupo dos chagásicos. O ecocardiograma é um método útil para diagnosticar e monitorar a pressão pulmonar previamente ao TC, especialmente em pacientes chagásicos. Entretanto, não é possível prescindirmos do cateterismo para avaliar a reatividade pulmonar com o teste com vasodilatador e indicar com segurança o TC mesmo nos pacientes chagásicos.

INTRODUCTION: The patients suffering heart failure develop an increase in pulmonary pressure because of a retrograde mechanism. The pulmonary hypertension is a prognostic marker. OBJECTIVE: The aim of this study is to correlate pulmonary hypertension measured by echocardiogram versus catheterization in pre-heart transplant patients on waiting list. METHODS: Data from 90 patients of the Clinical Hospital UFMG were collected between 2004 and 2009. All the patients took an echo and catheterization as an integral part of pre-heart transplant. Mean age was 45.5 years old, 68 (75.6%) male. Fourty-two (46.7%) were Chagas' disease patients, 32 (35.6%) presented idiopathic dilated cardiomyopathy, 10 (11.1%) had ischemic cardyomiopathy. RESULTS: The mean eco-PASP was 45 ± 12mmHg). The mean cat-PASP was 47 ±14mmHg. The eco-PASP-Chagas was 41.7 ±12,5 mmHg and non-Chagas 47.6 ±12.8 mmHg P=0.04. The cat-PASP-Chagas was 46 ±12.1 mmHg and non-Chagas 48.7 ±12.8 mmHg P=0.43. Eight patients had cat-PASP>60. The correlation between eco-PASP and cat-PASP in Chagas' patients was r=0.45; P=0.008 and in the non-Chagas was r=0.66; P<0.001. The eco-PASP-Chagas>32,5mmHg has a sensitivity of 79% and specificity of 75% to diagnose PH, with an area under the curve of 0.819. The eco-PASP-non-Chagas>35.5 mmHg has a sensitivity of 82% and a specificity of 70% to diagnose PH, with an area under the curve of 0.776. CONCLUSIONS: There is a good correlation between eco-PASP and cat-PASP (r=0.54) in pre-heart transplant patients. The eco-PASP was lower in the Chagas' group. The echocardiogram is an important method to diagnosis and control pulmonary pressure in pre-heart transplant, specifically in Chagas' patients. The catheterization is still important to evaluate pulmonary reactivity during vasodilation test.