Thromboprophylaxis for Children Post-Fontan Procedure: Insights From the UNIVERSE Study.

Background Patients with single-ventricle physiology who undergo the Fontan procedure are at risk for thrombotic events associated with significant morbidity and mortality. The UNIVERSE Study evaluated the efficacy and safety of a novel liquid rivaroxaban formulation, using a body weight-adjusted dosing regimen, versus acetylsalicylic acid (ASA) in children post-Fontan. Methods and Results The UNIVERSE Study was a randomized, multicenter, 2-part, open-label study of rivaroxaban, in children who had undergone a Fontan procedure, to evaluate its dosing regimen, safety, and efficacy. Part A was the single-arm part of the study that determined the pharmacokinetics/pharmacodynamics and safety of rivaroxaban in 12 participants before proceeding to part B, whereby 100 participants were randomized 2:1 to open-label rivaroxaban versus ASA. The study period was 12 months. A total of 112 participants were enrolled across 35 sites in 10 countries. In part B, for safety outcomes, major bleeding occurred in one participant on rivaroxaban (epistaxis that required transfusion). Clinically relevant nonmajor bleeding occurred in 6% of participants on rivaroxaban versus 9% on ASA. Trivial bleeding occurred in 33% of participants on rivaroxaban versus 35% on ASA. For efficacy outcomes, 1 participant on rivaroxaban in part B had a pulmonary embolism (2% overall event rate); and for ASA, 1 participant had ischemic stroke and 2 had venous thrombosis (9% overall event rate). Conclusions In this study, participants who received rivaroxaban for thromboprophylaxis had a similar safety profile and fewer thrombotic events, albeit not statistically significant, compared with those in the ASA group. Registration URL: https://www.clinicaltrials.gov. Identifier: NCT02846532.

Routine Detection of Atrial Fibrillation/Flutter Predicts a Worse Outcome in a Cohort of Tetralogy of Fallot Patients During 23 Years of Follow-Up.

Atrial flutter/fibrillation (AFL/AF) is a late complication in adults with repaired tetralogy of Fallot (TOF). Its effects on long-term prognosis are not fully understood. We evaluate the impact of AFL/AF in adults with repaired TOF on global mortality and unplanned hospitalizations during follow-up, and the predictors for AFL/AF occurrence. The presence of AFL/FA was analysed in all exams performed during the last 10 years of outpatients follow up in a unicentric cohort of repaired TOF between 1980 and 2003. Two-hundred and six patients were included; at a mean follow-up of 21 ± 8.2 years, there were 5 deaths (19.2%) in the AFL/AF group and 2 (1.1%) in those without arrhythmia (p < 0.001). Patients with AFL/AF where older at the time of the surgical repair (p < 0.001) and had a higher rate of reinterventions (p = 0.003). No differences were observed between the groups regarding the use of a transannular patch, ventriculotomy and previous palliative shunt. QRS duration was longer in patients with AFL/AF (174 ± 33.4) when compared to those without arrhythmia (147 ± 39.6; p < 0.0001). Age at surgery, QRS duration, and tricuspid regurgitation ≥ moderate were independent risk predictors for AFL/AF. In the multivariate analysis, atrial flutter/fibrillation and QRS duration were predictors of death and hospitalization. AFL/AF is associated with an increased risk of death and hospitalization during the follow-up of patients with repaired TOF. Early detection of AFL/AF and their predictors is an essential step in the evaluation of such population.

Entrecruzamento das vias de entrada ventriculres (Criss-Cross Heart) e transposição anatomicamente corrigida das grandes artérias: relato de caso e revisao de literatura / Criss-Cross heart and corrected transposition of great arteries: case report and literature review

Criss-cross heart é uma rara e complexa anomalia congênita que se caracteriza pela rotação da conexão atrioventricular e entrecruzamento dos fluxos de entrada dos ventrículos. É resultado de uma anomalia de rotação embrionária da massa ventricular no seu eixo longo de forma horária ou anti-horária, sem movimentação concomitante dos átrios. O quadro clínico depende das malformações associadas, dimensões da comunicação interventricular, presença ou não de estenose pulmonar, straddling da valva mitral e dimensões das cavidades ventriculares. A presente revisão abordará os critérios ecocardiográficos de criss-cross heart, definindo as questões técnicas do exame, além de relatar um caso de criss-cross com transposição corrigida das grandes artérias.

Transcatheter closure of atrial septal defect with Amplatzer device in children aged less than 10 years old: immediate and late follow-up.

AIMS: To analyze the efficacy and follow-up results of percutaneous closure of Atrial septal defect (ASD) with the Amplatzer septal occluder in children aged <10 years old. METHODS: Between November 1998 and September 2005, 27 patients diagnosed with ASD were treated percutaneously with an Amplatzer septal occluder. The procedure was carried out in the cathlab, under general anesthesia and with both fluoroscopy and transesophageal echocardiography guidance. Basal physical examinations and echocardiograms were performed prior to the procedure and at 30 days, 6, and 12 months of follow-up. Survival free of symptom was estimated by Kaplan-Meier. RESULTS: The mean age, weight, height, body mass index, and corporal surface was: 5.35 +/- 2.11 years, 23.07 +/- 9.43 kg, 110.55 +/- 17.6 cm, 16.77 +/- 2.42 kg/m(2), and 1.24 +/- 2.44 m(2). The prevalence of septal aneurysm was 3.7% and all patients presented single secundum ASD. The mean stretched diameter by fluoroscopy and transesophageal echocardiography were 17.18 +/- 6.75 mm and 16.77 +/- 5.99 mm, and the prostheses sizes were 18.83 +/- 6.98 mm, ranging from 10 to 30 mm. The systolic and diastolic pulmonary pressures were 25.26 +/- 5.97 mm Hg and 13.38 +/- 3.40 mm Hg, respectively. The procedure time was 82.92 +/- 29.14 min and the hospital stay was 2.20 +/- 0.26 days. Clinical and echocardiography follow-ups were performed within 11.59 +/- 4.42 months and all devices were in the correct position with no residual shunt. Right ventricular diameter decreased from 19.38 +/- 5.23mm to 11.38 +/- 11.92 (P 0.001). No major complications or deaths occurred; two patients had a hematoma at the vascular access. CONCLUSION: Secundum atrial septal defect closure can be safely and successfully performed with the Amplatzer septal occluder in children younger than 10 years old.

Increased S100B serum levels in dilated cardiomyopathy patients.

BACKGROUND: The S100B protein is considered a biochemical marker for brain injuries. However, our group demonstrated that the isolated rat heart releases S100B. In this study, we investigated the serum levels of S100B in dilated cardiomyopathy (DCM) patients to evaluate its levels in heart disease. METHODS AND RESULTS: We selected DCM patients, excluding any condition that could influence S100B serum levels. Control individuals were sex and age matched. Both groups were submitted to clinical evaluation and echocardiography. We measured the S100B and NT-proBNP serum levels (expressed as median [interquartile range]). NT-proBNP levels in patients group (1462 pg/mL [426-3591]) were higher than in controls (35 pg/mL [29-55]), P < .001. S100B serum levels were higher in patients group (0.051 microg/L [0.022-0.144]) than in controls (0.017 microg/L [0.003-0.036]), P = .009. Additionally, we found a positive correlation between S100B and NT-proBNP serum levels only in patients group (Spearman's coefficient r = 0.534; P = .013). CONCLUSIONS: Although we cannot rule out the influence of S100B from brain, the positive correlation between S100B and NT-proBNP levels in DCM patients points to the myocardium as the main source for the rise in S100B serum levels.

Effectiveness of the Amplatzer device for transcatheter closure of an ostium secundum atrial septal defect.

OBJECTIVE: To evaluate the effectiveness of the Amplatzer septal occluder in the treatment of ostium secundum atrial septal defects (OS ASDs). METHODS: Retrospective cohort study conducted between November 1998 and September 2005 involving 101 OS ASD transcatheter occlusion procedures in our institution. All procedures were conducted in the hemodynamic laboratory under general anesthesia with transoesophageal echocardiographic monitoring (TEE). Clinical and echocardiography assessments of the patients were conducted at 30 days, six months and on an annual basis. The results are presented as averages, standard deviations and percentages. Event-free survival was estimated using the Kaplan-Meier curve. RESULTS: From the 101 patients, 60 (59.4%) were females. Mean age, weight, height, body mass index and body surface area were, respectively: 24.3 +/- 18.31 years, 51.88 +/- 23.76 kg, 140.59 +/- 39.3 cm, 23.18 +/- 18.9 kg/m(2) and 1.24 +/- 0.21 m(2). The prevalence of interatrial septum aneurysms was 4.95%, and 98 cases had an isolated defect. ASD diameters were 21.47 +/- 6.96 mm using an angiography and 21.22 +/- 7.93 mm using a TEE. The average size of the implanted devices was 23.92 +/- 7.25 mm, ranging from 9 mm to 40 mm. The procedure time was 90.47 +/- 26.67 minutes and the average hospital stay was 2.51 +/- 0.62 days. Clinical and echocardiography follow-up was conducted at 12.81 +/- 8.41 months and all devices were securely anchored without any residual shunts. The procedure success rate was 93% (94/101). In five cases adequate deployment of the device was not possible and 2 patients presented residual ASD. No major complications occurred. CONCLUSION: The Amplatzer septal occluder is an effective OS ASD transcatheter treatment device.

Efetividade da prótese de Amplatzer™ para fechamento percutâneo do defeito do septo interatrial tipo Ostium Secundum / Effectiveness of the Amplatzer ™ device for transcatheter closure of an ostium secundum atrial septal defect

OBJETIVO: Avaliar a efetividade da prótese de Amplatzer® para tratamento de comunicação interatrial tipo ostium secundum (CIA OS). MÉTODOS: Estudo de coorte histórica entre novembro de 1998 e setembro de 2005, em que foram realizados 101 procedimentos para oclusão percutânea de CIA OS em nossa instituição. Os procedimentos foram efetuados no laboratório de hemodinâmica, sob anestesia geral e com monitorização por ecocardiografia transesofágica (ETE). Os pacientes foram acompanhados clinicamente e com ecocardiografia em 30 dias, seis meses e depois anualmente. O resultados são apresentados em média, desvio padrão e porcentual, e a sobrevida livre de eventos foi estimada pela curva de Kaplan-Meier. RESULTADOS: Dos 101 pacientes, 60 (59,4 por cento) eram mulheres. As médias para idade, peso, altura, índice de massa corporal e superfície corporal foram, respectivamente, de 24,3 + 18,31 anos, 51,88 + 23,76 kg, 140,59 + 39,3 cm, 23,18 + 18,9 kg/m², e 1,24 + 0,21 m². A prevalência de aneurisma do septo interatrial foi de 4,95 por cento, e 98 casos eram de defeito único. O diâmetro das CIAs foi de 21,47 + 6,96 mm pela angiografia e de 21,22 + 7,93 mm pela ETE. As próteses implantadas mediam 23,92 + 7,25 mm, variando de 9 mm a 40 mm. O tempo de procedimento foi de 90,47 + 26,67 minutos e a média de internação hospitalar, de 2,51 + 0,62 dias. Os seguimentos clínico e ecocardiográfico ocorreram com 12,81 + 8,41 meses e todas as próteses estavam bem ancoradas e sem shunt residual. O sucesso do procedimento foi de 93 por cento (94/101). Em cinco casos não se conseguiu liberação adequada do dispositivo e dois pacientes apresentaram CIA residual. Não foram registradas complicações maiores. CONCLUSÃO: A prótese de Amplatzer® mostrou-se efetiva para o tratamento percutâneo de CIA OS.

OBJECTIVE: To evaluate the effectiveness of the Amplatzer™ septal occluder in the treatment of ostium secundum atrial septal defects (OS ASDs). METHODS: Retrospective cohort study conducted between November 1998 and September 2005 involving 101 OS ASD transcatheter occlusion procedures in our institution. All procedures were conducted in the hemodynamic laboratory under general anesthesia with transoesophageal echocardiographic monitoring (TEE). Clinical and echocardiography assessments of the patients were conducted at 30 days, six months and on an annual basis. The results are presented as averages, standard deviations and percentages. Event-free survival was estimated using the Kaplan-Meier curve. RESULTS: From the 101 patients, 60 (59.4 percent) were females. Mean age, weight, height, body mass index and body surface area were, respectively: 24.3 ± 18.31 years, 51.88 ± 23.76kg, 140.59 ± 39.3cm, 23.18 ± 18.9kg/m² and 1.24 ± 0.21m². The prevalence of interatrial septum aneurysms was 4.95 percent, and 98 cases had an isolated defect. ASD diameters were 21.47 ± 6.96mm using an angiography and 21.22 ± 7.93 mm using a TEE. The average size of the implanted devices was 23.92 ± 7.25mm, ranging from 9mm to 40mm. The procedure time was 90.47 ± 26.67 minutes and the average hospital stay was 2.51 ± 0.62 days. Clinical and echocardiography follow-up was conducted at 12.81 ± 8.41 months and all devices were securely anchored without any residual shunts. The procedure success rate was 93 percent (94/101). In five cases adequate deployment of the device was not possible and 2 patients presented residual ASD. No major complications occurred. CONCLUSION: The Amplatzer™ septal occluder is an effective OS ASD transcatheter treatment device.

Computer dynamics to evaluate blood flow through the modified Blalock-Taussig shunt

OBJETIVO: Dinâmica computacional foi utilizada para avaliar a influência de fatores geométricos no funcionamento de modelo da cirurgia de Blalock-Taussig modificada (BTm), ou interposiçäo de enxerto de politetrafluoretileno (PTFE) entre as artérias subclávia e pulmonar.

Late clinical outcomes of the fontan operation in patients with tricuspid atresia.

OBJECTIVE: Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. METHODS: A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan operation or one of its variants due to tricuspid atresia. Their mean age at surgery was 5.4+/-3.1 years, and their mean weight was 15.8+/-6.1 kg, the majority of them (63.9%) being males. Four patients underwent the classical Fontan operation, 12 the Kreutzer variant, 6 the Björk variant, 9 total cavopulmonary shunt with a fenestrated tube, and 5 total cavopulmonary shunt with a nonfenestrated tube. RESULTS: The patients were followed-up on an outpatient basis, with a mean long-term survival time of 5.5+/-4.2 years (50 days to 17.8 years) and a late mortality rate of 8%. Arterial saturation increased from 77.2+/-18.8% in the preoperative period to 91+/-6.7% upon the last outpatient visit (p>0.05). At the final check, most (67%) patients were asymptomatic and 87% could tolerate exercise. Ten (40%) patients experienced some kind of complication during the long-term follow-up, such as cardiac arrhythmia, cyanosis, protein-losing enteropathy, neurological events, right heart failure, intolerance to exercise and reoperation. CONCLUSION: The results indicate that, once the immediate postoperative period is over, during which the adaptations to the new circulatory physiology occur, the evolution of patients with tricuspid atresia who underwent the Fontan operation is satisfactory, in spite of a low, yet significant, morbidity.

Desfechos clínicos tardios da cirurgia de Fontan em pacientes com atresia tricúspide / Late clinical outcomes of the Fontan operation in patients with tricuspid atresia

OBJECTIVE: Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. METHODS: A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan operation or one of its variants due to tricuspid atresia. Their mean age at surgery was 5.4±3.1 years, and their mean weight was 15.8±6.1 kg, the majority of them (63.9 percent) being males. Four patients underwent the classical Fontan operation, 12 the Kreutzer variant, 6 the Björk variant, 9 total cavopulmonary shunt with a fenestrated tube, and 5 total cavopulmonary shunt with a nonfenestrated tube. RESULTS: The patients were followed-up on an outpatient basis, with a mean long-term survival time of 5.5±4.2 years (50 days to 17.8 years) and a late mortality rate of 8 percent. Arterial saturation increased from 77.2±18.8 percent in the preoperative period to 91±6.7 percent upon the last outpatient visit (p>0.05). At the final check, most (67 percent) patients were asymptomatic and 87 percent could tolerate exercise. Ten (40 percent) patients experienced some kind of complication during the long-term follow-up, such as cardiac arrhythmia, cyanosis, protein-losing enteropathy, neurological events, right heart failure, intolerance to exercise and reoperation. CONCLUSION: The results indicate that, once the immediate postoperative period is over, during which the adaptations to the new circulatory physiology occur, the evolution of patients with tricuspid atresia who underwent the Fontan operation is satisfactory, in spite of a low, yet significant, morbidity

Corte paracoronal transgástrico transesofágico. Uso e indicaçoes em cardiologia pediátrica / ransesophageal Paracoronal Transgastric View. Indication and Use in Pediatric Cardiology

PURPOSE--To evaluate the contribution and comparative value of paracoronal transgastric view compared with conventional transesophageal examination for morpho-functional assessment of different types of congenital heart disease in a pediatric group. METHODS--Fifteen patients with clinical and echocardiographic diagnosis of congenital heart disease were selected for single plane transesophageal examination. After routine evaluation, the probe was positioned to obtain a paracoronal transgastric view, and images that result from this technique were recorded and compared with those obtained in the conventional way. Eleven procedures were carried out in the cathlab and four in pediatric intensive care unit, under general anesthesia or heavy sedation. The age and weight were 32.0 months and 11.6 kg respectively. No adverse reactions were observed with this method. RESULTS--In comparison with conventional transesophageal study, the paracoronal transgastric view permitted better morpho-functional assessment of the outlets of the right and left ventricles, as well as additional informations about the left pulmonary artery. CONCLUSION--Morphological and hemodynamic informations obtained from paracoronal transgastric view is a safe method which can be used either as an alternative or a complement to conventional examination to assess the outlets of both ventricles, as well as to evaluate the subvalvar, valvar and supravalvar region in different types of congenital heart disease.

Pneumonia por Chlamydia trachomatis: descriçäo de dois casos / Chlamydia trachomatis pneumonia: report of 2 cases

Vários estudos epidemiológicos realizados nos Estados Unidos têm mostrado que as infecçöes por Chlamydia säo freqüentes em mulheres grávidas e que cerca de 1% dos recém-nascidos desenvolvem pneumonia por este agente. Através de testes sorológicos recentemente introduzidos em nosso meio passou a ser possível o diagnóstico destas infecçöes. Descreve-se em dois casos de pneumonia por Chlamydia trachomatis em recém-nascidos, adquiridas na passagem pelo canal do parto. A doença caracterizou-se por disfunçäo respiratória progressiva, associada em algum momento a uma conjuntivite, apresentando ao raio X um infiltrado intersticial difuso com hiperexpansäo pulmonar. Eosinofilia esteve presente em ambos os casos. O tratamento com eritromicina seguiu-se de rápida regressäo do quadro clínico e radiológico