Resultados Perinatais e Seguimento em Longo Prazo de Tumores Cardíacos Fetais: Estudo de Coorte Histórica de 30 Anos / Perinatal Results and Long-Term Follow-Up of Fetal Cardiac Tumors: A 30-Year Historical Cohort Study

Resumo Fundamento: Seguimento de coorte retrospectiva de 30 anos que se aproxima da história natural dos tumores cardíacos diagnosticados no feto uma vez que nenhum caso foi submetido à interrupção da gestação. Objetivo: Avaliar a morbidade e mortalidade perinatal e em longo prazo em fetos com diagnóstico de tumor cardíaco. Como objetivo secundário avaliar os fatores que influenciaram os resultados perinatais e pós-natais. Método: Estudo de coorte retrospectiva envolvendo 74 gestantes com diagnóstico ecocardiográfico fetal de tumor cardíaco acompanhadas em dois serviços de referência no período de maio de 1991 a novembro de 2021. Foi realizada análise descritiva dos dados por meio de frequências absolutas (n) e relativas (%), mediana e intervalos interquartis. Para avaliar a associação entre as características ecocardiográficas e as manifestações clínicas com os resultados perinatais e pós-natais, foi aplicado o teste exato de Fisher. O cálculo da sobrevida global foi realizado pelo método de Kaplan-Meier e a comparação de curvas pelo teste de log-rank. O tempo de seguimento, calculado em meses, foi definido a partir da data de alta do hospital à data do status atual (vivo/censura ou óbito). O nível de significância considerado foi de 5% (p<0,05). Resultados: o rabdomioma é o tipo mais frequente (85%) de tumor cardíaco; apresenta alta morbidade (79,3%) e mortalidade geral de 17,4%; a presença de hidropisia fetal preditiva de óbito. Conclusão: A presença de hidropisia fetal teve impacto na mortalidade, sendo fator importante para aconselhamento e estabelecimento de prognóstico. A maioria dos óbitos ocorrem antes da alta hospitalar.

Abstract Background: This was a 30-year retrospective cohort study that approximates closely to the natural history of cardiac tumors diagnosed in the fetus, since there was no case of pregnancy interruption Objective: To assess morbidity and mortality in the perinatal period and at long term in fetuses diagnosed with cardiac tumor. Our secondary objective was to assess the evaluating factors of perinatal and postnatal results. Methods: This was a retrospective cohort study with 74 pregnant women with an echocardiographic diagnosis of fetal cardiac tumor at two referral centers between May 1991 and November 2021. A descriptive analysis was performed, and data were expressed as absolute (n) and relative (%) frequencies, median and interquartile range. Fisher's exact test was used to evaluate the association of echocardiographic characteristics and clinical manifestations with perinatal and postnatal results. Global survival was calculated using the Kaplan-Meier method and the curves were compared by the log-rank test. The time of follow-up, calculated in months, corresponded to the time elapsed from hospital discharge to current status (survived/ censoring or death). The level of significance was set at 5% (p<0.05). Results: Rhabdomyoma is the most common type of cardiac tumor (85%), with a high morbidity (79.3%) and overall mortality of 17.4%. The presence of fetal hydrops was a predictor of death. Conclusion: The presence of fetal hydrops had an impact on mortality, and hence is an important factor in counselling and determining the prognosis. Most deaths occurred before hospital discharge.

Collaborative quality improvement in the congenital heart defects: development of the assist consortium and a preliminary surgical outcomes report

Abstract Objective: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. Methods: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. Results: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (≤ 30 days, and 30 days - 1 year). Conclusion: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.

Associação de bronquite plástica com enteropatia perdedora de proteínas após operação de Fontan / Association of plastic bronchitis to protein-losing enteropathy after fontan operation

Relatamos um caso incomum de associação de bronquite plástica (BP) com enteropatia perdedora de proteínas (EPP) em menina de 4 anos e 9 meses de idade. com dupla via de entrada de ventrículo único tipo esquerdo e concordância ventrículo-arterial submetida à cirurgia cavopulmonar total. com túnel lateral intracardíaco aos três anos. Tornaram-se chamativas a eliminação de molde brônquico de fibrina de 10 cm (BP) e a elevação de alfa-1-antitripsina de 52 mg/g de fezes. Em uso de sildenafila. programou-se. em caso de continuidade do processo. a ligadura do ducto torácico e transplante cardíaco.

We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.

Síndrome de Williams-Beuren: anomalias cardiovasculares em 20 pacientes diagnosticados pela hibridazação in situ por fluorescência / Williams-Beuren syndrome: cardiovascular abnormalities in 20 patients diagnosed with fluorescence in situ hybridization

OBJECTIVE: To evaluate the cardiovascular findings and clinical follow-up of patients with Williams-Beuren syndrome. METHODS: We studied 20 patients (11 males, mean age at diagnosis: 5.9 years old), assessed for cardiovascular abnormalities with electrocardiography and Doppler echocardiography. Fluorescence in situ hybridization (FISH) was used to confirm the diagnosis of the syndrome. RESULTS: Elastin gene locus microdeletion was detected in 17 patients (85 percent) (positive FISH), and in 3 patients deletion was not detected (negative FISH). Sixteen patients with a positive FISH (94 percent) had congenital cardiovascular disease (mean age at diagnosis: 2,3 years old). We observed isolated (2/16) supravalvular aortic stenosis and supravalvular aortic stenosis associated (11/16) with pulmonary artery stenosis (4/11); mitral valve prolapse (3/11); bicuspid aortic valve (3/11); aortic coarctation (2/11), thickened pulmonary valve (2/11); pulmonary valvular stenosis (1/11); supravalvular pulmonary stenosis (1/11); valvular aortic stenosis (1/11); fixed subaortic stenosis (1/11); pulmonary artery stenosis (2/16) associated with pulmonary valvar stenosis (1/2) and with mitral valve prolapse (1/2); and isolated mitral valve prolapse (1/16). Four patients with severe supravalvular aortic stenosis underwent surgery (mean age: 5.7 years old), and 2 patients had normal pressure gradients (mean follow-up: 8.4 years). CONCLUSION: A detailed cardiac evaluation must be performed in all patients with Williams-Beuren syndrome due to the high frequency of cardiovascular abnormalities

Fontan operation and the cavopulmonary technique. Immediate and late results according to the presence of atrial fenestration

OBJECTIVE: To compare immediate and late results in patients with or without fenestration who underwent cavopulmonary anastomosis so that we could assess the efficiency of the technique. METHODS: Sixty-two patients underwent surgery between 1988 and 1999, 41 with fenestration (group I -G I) and 21 without fenestration (group II -G II). Tricuspid atresia was prevalent in group I (23-56 percent) and single ventricle was prevalent in group II (14-66 percent). Mean ages at the time of operation were 7.3 years in group I and 7.6 in group II. At late follow-up, mean ages were 10.6 years in group I and 12.8 years in group II. RESULTS: Immediate and late mortality were 7.3 percent in G-I and 4.7 percent in G-II. Significant pleural effusion occurred in 41.4 percent of G-I patients and in 23.8 percent of G-II patients. Significant pericardial effusion occurred in 29.2 percent and 14.2 percent, respectively, in groups I and II. Central venous pressure was greater in G-II, 17.7 cm in H2O, as opposed to 15 cm in G-I. Hospital stay was similar between the groups, 26.3 and 21.8 days, respectively. Cyanosis and arterial insaturation occurred in 5 patients, and 4 patients were in functional class II, all from G-I. At late follow-up, 58 (93.5 percent) were in functional class I. Sinus rhythm was present in 94 percent, and pulmonary perfusion was similar in both groups. Eleven patients who underwent spirometry had good tolerance to physical effort. CONCLUSION: Atrial fenestration did not improve the immediate or late follow-up of patients who underwent cavopulmonary anastomosis, and is, therefore, dispensable

Anomalia de UhL. Diagnóstico diferencial e indicaçäo de transplante cardíaco em lactente / Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed

Anomalia de Ebstein com coarctaçäo de aorta: uma associaçäo rara / Ebstein's anomaly with coarctation of the aorta: an unusual association

Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solittus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a De Vega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.

Defeito total do septo atrioventricular: correlaçäo anatomofuncional entre pacientes com e sem Síndrome de Down / Complete atrioventricular canal: clinical anatomic correlations between patients with and without Down's Syndrome

OBJETIVOS: Comprarar o grau de repercussäo, fatores responsáveis e época de aparecimento dos sintomas do defeito do septo atrioventricular (DSAV), em pacientes com e sem síndrome de Down...

Abordagem transxifóidea sem esternotomia para correçäo da comunicaçäo interatrial / Transxiphoid approach without sternotomy for the correction of atrial septal defect

Objetivo: Avaliaçao de uma nova abordagem cirúrgica para fechamento da comunicaçao interatrial. Métodos: A abordagem da comunicaçao interatrial através da janela xifóidea foi realizada em 6 pacientes do sexo masculino e l do sexo feminino e a idade variou de 6 meses a 14 anos, com média de idades de 5,1 anos. Em todos, foi utilizada a circulaçao extracorpórea, através de canulaçao da artéria femoral e átrio direito e videotoracoscopia para auxiliar a visibilizaçao da aorta e do canal arterial, assim como das estruturas correspondentes às cavidades esquerdas. Resultados: Nao houve complicaçoes intra-operatórias ou pós-operatórias e, em todos os pacientes, foi possível a extubaçao na sala de operaçoes. Conclusao: A janela xifóidea, sem a abertura do esterno, permitiu a correçao da comunicaçao interatrial com bons resultados, podendo constituir-se em uma nova abordagem, minimamente invasiva.

Tratamento da síndrome do QT longo com a técnica de autotransplante cardíaco com preservaçäo do sistema de conduçäo / Treatment of long QT syndrome with the technique of heart transplantation with the conduction system preservation

A síndrome do QT longo é caracterizada por prolongaçao intermitente ou permanente do intervalo QT, taquicardias paroxísticas ventriculares (TVR) e morte súbita. Existem três casos publicados na literatura tratados cirurgicamente com a técnica do autotransplante cardíaco, com l paciente sobrevivente. Nos 3 casos foi necessária, após a operaçao, a utilizaçao de marcapasso átrio-atrial ou atrioventricular. No InCór, entre outubro de 1993 e maio de 1994, 3 pacientes portadores desta síndrome, l do sexo feminino e 2 do masculino, com idades, respectivamente, de lO, ll e 13 anos, foram submetidos a desnervaçao por autotransplante. No pré-operatório, apesar de dose máxima de beta-bloqueadores, os freqüentes episódios de taquicardia ventricular nao foram evitados. Com carga mínima o teste ergométrico provocava TVR. A retirada do órgao foi feita seccionando-se as veias cavas, aorta, tronco pulmonar e as veias pulmonares, com remanescente de átrio esquerdo. Assim, o sistema de conduçao permaneceu íntegro e o ritmo, sinusal. No pós-operatório os testes ergométricos realizados mostraram ausência de taquicardia ventricular. Considerando os resultados imediatos, julgamos que a técnica relatada de autotransplante cardíaco como método de completa desnervaçao pode ser empregada em pacientes portadores da síndrome do QT longo, com baixo risco cirúrgico e preservaçao do sistema de conduçao.

Atresia pulmonar com septo interventricular íntrego: desenvolvimento natural do ventrículo direito / Pulmonary atresia with intact ventricular septum: natural development of the right ventricule

Lactente, portador de atresia pulmonar com septo interventricular íntegro e ventrículo direito (VD) hipoplásico, contendo 3 porçöes bem definidas, que se tornou naturalmente bem desenvolvido até 2 anos de idade, após anastomose de Blalock-Taussig, realizada precocemente. O valor Z da valva tricúspide, inicialmente de 0,3 e sobrecarga de ventrículo esquerdo no eletrocardiograma transformaram-se em anel tricúspide de 22mm e sobrecarga de VD após 2 anos de evoluçäo, motivando correçäo cirúrgica. Assim, a demonstraçäo de fenômeno, até hoje ignorado, do aumento natural de uma cavidade cardíaca hipoplástica, torna possível pospor a cirurgia nesta anomalia à fase ulterior, diminuindo riscos

Transposição das grandes artérias com aorta posterior / Tronsposition of the Great Arteries with Posterior Aorta

Säo relatados dois caso de transposiçäo das grandes artérias (TGA) com aorta posterior. O 1§ paciente tinha o diagnóstico feito no pré-opertório e foi submetido a cirurgia de Senning com bom resultado. O 2§ teve seu diagnóstico pré-operatório de dupla via de saída de ventrículo direito (DVSVD) com estenose pulmonar infundíbulo-valvar (EPIV) exigindo cirurgia tipo Blalock-Taussing de urgência, devido a sua acentuada hipoxemia. Evoluiu para o óbito, sendo constatado, em estudo necroscópico, TGA com aorta posterior. Ambos os casos possuíam infundíbulo subpulmonar e comunicaçäo interventricular, esta permitindo continuidade fibrosa mitroaórtica, dados estes que väo de encontro áqueles vistos mais freqüentemente na literatura. Discutem-se aqui os aspectos de propedêutica clínica e armada de importância nesta rara variante de TGA

Two cases of transposition of the great arteries(TGA) with posterior aorta are reported. The first was submitted to the Senning procedure with good outcome; the other had the diagnosis of double outlet right ventricle with severe pulmonary stenosis, and a Blalock Taussig shunt was accomplishedior hypoxic crisis. The patient died 11 days after surgery and the necropsy demontrated TGA with posterior aorta. In both cases there was a sub-pulmonary infundibulum. The presence of ventricular septal defects allowed aortic-mitral fibrous continuity, a finding previously described in anatomical reports. Clinical and angiographic aspects are discussed

Miocardiopatia isquêmica na criança: relato de caso com acentuada coronariopatia / Ischemic cardiomyophathy in childhood. case report of advanced coronary artery disease

A miocardiopatia isquêmica na criança está relacionada com uma série de anomalias congênitas e adquiridas, que podem levar a seqüelas cardíacas importantes, como infarto do miocárdio e à morte súbita. Eventualmente, torna-se dificil a caracterizaçäo etiológica, pela ausência de testes diagnósticos específicos, em situaçöes em que näo há malformaçöes das artérias coronárias. Relatam-se os aspectos clínicos de uma criança de seis anos de idade, portadora de coronariopatia severa: aneurisma de tronco da coronária esquerda com oclusäo no terço proximal de artéria descendente anterior e no terço proximal de artéria coronária direita dominante, além de acentuada disfunçäo miocárdica. O diagnóstico provisório neste caso foi de doença de Kawasaki. Säo analisados os principais elementos de interesse no diagnóstico da coronariopatia, bem como a importância da identificaçäo de casos enquanto assintomáticos, porém, com alteraçöes significativas das artérias coronárias, situaçäo presente no paciente relatado

Ischemic cardiomyopathy in childhood is related with congenital and acquired pathologies which could lead to serious cardiac sequelae as myocardial infarction and sudden death. Unfortunately, when coronary artery malformations are excluded, it is difficult in some cases to be completely certain on the etiology of the arterial coronary disease, due the lack of pathognomonic diagnostic tests. Case report of 6 year old child, with severe coronary artery disease: aneurysm of main branch of left coronary artery; left anterior descending branch and right coronary artery occlusions with acentuated myocardial dysfunction. We believe that the diagnosis of Kawasaki disease is presumptive in this patient. The principal aspects to elucidate the etiology of coronary artery disease are analysed, as well as the importance of identifying patients without known previous illness, but with severe coronary artery lesions, as in this reported case