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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

Resumo Fundamento Algumas síndromes têm características específicas e facilmente reconhecíveis, enquanto outras podem ser mais complexas de se identificar e podem apresentar diferentes manifestações fenotípicas, por exemplo. Um diagnóstico etiológico é importante para entender a natureza da doença, para estabelecer o prognóstico e para começar o tratamento, permitindo a inclusão de pacientes na sociedade e reduzindo o custo financeiro dessas doenças. Objetivo A proposta inicial deste estudo foi a triagem citogenética para detectar a síndrome de deleção 22q11.2 (SD22q11.2) em recém-nascidos e crianças com doença cardíaca congênita utilizando a técnica da amplificação multiplex de sondas dependente de ligação (MLPA). Assim, por meio da pesquisa, outras mudanças genômicas foram identificadas nesses pacientes cardíacos. Nosso objetivo se estendeu a investigar essas outras mudanças citogenéticas. Métodos Investigamos 118 recém-nascidos com doenças cardíacas congênitas nascidos consecutivamente durante um ano, utilizando a técnica da MLPA. Resultados A técnica da MLPA permitiu a detecção da SD22q11.2 em 10/118 pacientes (8,5%). Outras alterações genômicas foram identificadas em 6/118 pacientes (5%): 1p36 del, 8p23 del (2 casos), 7q dup, 12 dup e 8q24 dup. Conclusão Este estudo ressalta a relevância da detecção de alterações genômicas que estão presentes em recém-nascidos e crianças com doenças cardíacas congênitas por meio de ferramentas citogenômicas.

Abstract Background The Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome (OMIM 253200) is an autosomal recessive lysosomal disorder, caused by the deficiency of the enzyme N-acetylgalactosamine 4-sulfatase (also known as arylsulfatase B) due to mutations of the ARSB gene. Cardiologic features are well recognized, and are always present in MPS VI patients. Generally, the onset and the progression of the cardiologic symptoms are insidious, and just a few patients have developed a rapidly progressive disease. Cardiac involvement in MPS VI is a common and progressive feature. For MPS patients, cardiac evaluations are recommended every 1 to 2 years, including blood pressure measurement, electrocardiography and echocardiography. However, congestive heart failure and valvular surgical repair are not frequently seen, and if so, they are performed in adults. Here we report on an atypical MPS VI case with ascites fetalis and a rapidly progressive cardiac disease. Case presentation A 6-month-old Brazilian male, only child of a Brazilian healthy non-consanguineous couple. During pregnancy, second trimester ultrasonography observed fetal ascites and bilateral hydrocele. Physical exam at 6 months-old revealed a typical gibbus deformity and MPS was suspected. Biochemical investigation revealed a diagnosis of MPS type VI, confirmed by molecular test. Baseline echocardiogram revealed discrete tricuspid regurgitation and a thickened mitral valve with posterior leaflet prolapse, causing moderate to severe regurgitation. The patient evolved with mitral insufficiency and congestive heart failure, eventually requiring surgical repair by the first year of age. Conclusions We report the first case of MPS VI whose manifestations started in the prenatal period with fetal ascites, with severe cardiac valvular disease that eventually required early surgical repair. Moreover, in MPS with neonatal presentation, including fetal hydrops, besides MPS I, IVA and VII, clinicians should include MPS VI in the differential diagnosis.

Objective: The objective of this study was to describe the clinical course of a newborn who developed dilated cardiomyopathy (DCM) after COVID-19 infection.Methods: We retrospectively assessed the clinical notes of a pediatric patient with decompensated heart failure and who was previously positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).Results: A 23-day-old newborn presented with diarrhea, hypoactivity, tachypnea, and lethargy. The infant progressed to develop respiratory failure and required orotracheal intubation due to apnea. A nasopharyngeal swab tested positive for SARS-COV-2. An echocardiogram (ECHO) demonstrated severe left ventricular dysfunction. The patient was discharged after 18 days with furosemide and angiotensin-converting enzyme inhibitors. During the follow-up period, the infant had two episodes of decompensated heart failure, with evidence of DCM. Investigations for known causes of secondary DCM were negative. The infant was promptly referred for heart transplantation.Conclusion: Although rare, we have observed a case of DCM in a newborn following COVID-19 disease. DCM may be a complication following COVID-19 disease in newborns.

Abstract Introduction: Post-cardiotomy myocardial dysfunction requiring mechanical circulatory support occurs in about 0.5% of cases. In our environment, the use of extracorporeal membrane oxygenation has been increasing in recent years. Objective: To evaluate the impact of investment in professional training and improvement of equipment in the rate of weaning from extracorporeal membrane oxygenation and survival. Methods: A retrospective study. Fifty-six pediatric and/or congenital heart patients underwent post-cardiotomy extracorporeal membrane oxygenation at our institution between November 1999 and July 2014. We divided this period into two phases: phase I, 36 cases (before the structuring of the extracorporeal membrane oxygenation program) and phase II, 20 cases (after the extracorporeal membrane oxygenation program implementation) with investment in training and equipment). Were considered as primary outcomes: extracorporeal membrane oxygenation weaning and survival to hospital discharge. The results in both phases were compared using Chi-square test. To identify the impact of the different variables we used binary logistic regression analysis. Results: Groups were comparable. In phase I, 9 patients (25%) were weaned from extracorporeal membrane oxygenation, but only 2 (5.5%) were discharged. In phase II, extracorporeal membrane oxygenation was used in 20 patients, weaning was possible in 17 (85%), with 9 (45%) hospital discharges (P

Background: Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure. Objective: To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy. Methods: Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy. Results: We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and causeof death in group A while neurologic complications were more prevalent en group B. Conclusion: Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2.

Background: To alert for the diagnosis of the 22q11.2 deletion syndrome (22q11.2DS) in patients with congenital heart disease (CHD). Objective: To describe the main CHDs, as well as phenotypic, metabolic and immunological findings in a series of 60 patients diagnosed with 22q11.2DS. Methods: The study included 60 patients with 22q11.2DS evaluated between 2007 and 2013 (M:F=1.3, age range 14 days to 20 years and 3 months) at a pediatric reference center for primary immunodeficiencies. The diagnosis was established by detection of the 22q11.2 microdeletion using FISH (n = 18) and/or MLPA (n = 42), in association with clinical and laboratory information. Associated CHDs, progression of phenotypic facial features, hypocalcemia and immunological changes were analyzed. Results: CHDs were detected in 77% of the patients and the most frequent type was tetralogy of Fallot (38.3%). Surgical correction of CHD was performed in 34 patients. Craniofacial dysmorphisms were detected in 41 patients: elongated face (60%) and/or elongated nose (53.3%), narrow palpebral fissure (50%), dysplastic, overfolded ears (48.3%), thin lips (41.6%), elongated fingers (38.3%) and short stature (36.6%). Hypocalcemia was detected in 64.2% and decreased parathyroid hormone (PTH) level in 25.9%. Decrease in total lymphocytes, CD4 and CD8 counts were present in 40%, 53.3% and 33.3%, respectively. Hypogammaglobulinemia was detected in one patient and decreased concentrations of immunoglobulin M (IgM) in two other patients. Conclusion: Suspicion for 22q11.2DS should be raised in all patients with CHD associated with hypocalcemia and/or facial dysmorphisms, considering that many of these changes may evolve with age. The 22q11.2 microdeletion should be confirmed by molecular testing in all patients.

Heart transplantation is an option for children with complex congenital heart disease and cardiomyopathies. A patient's quality of life and long-term survival depend on successful management of the surgical complications and adverse side effects of immunosuppression. The purpose of this review was to summarize the practical management of postoperative care in this patient population and to make recommendations for the future.

OBJETIVO: Analisar uma série de 34 pacientes adultos submetidos ao tratamento cirúrgico da persistência do canal arterial. MÉTODOS: Estudo retrospectivo, com coleta de dados dos prontuários de 34 pacientes consecutivos, com idade superior a 18 anos, com persistência do canal arterial submetidos a correção cirúrgica, no período de 1997 a 2008, no Instituto do Coração da Faculdade de Medicina da Universidade de São Paulo. RESULTADOS: A idade média foi de 28,7 (18 a 53) anos e 22 (64,7%) pacientes eram do sexo feminino. O sintoma mais frequente foi dispneia (76,5%). A toracotomia lateral esquerda foi utilizada em 33 (97,1%) pacientes e o canal arterial foi seccionado e suturado em 25 (73,5%). A circulação extracorpórea (CEC) foi necessária em um paciente. Observou-se calcificação em oito (23,5%) pacientes e 12 (35,3%) haviam sido submetidos à tentativa de fechamento percutâneo. A incidência de complicações foi de 32%, sendo uma permanente, com paralisia de corda vocal (2,9%). Dois (5,8%) pacientes permaneceram com shunt residual e três (8,8%) apresentaram paralisia de corda vocal esquerda transitória. A cirurgia realizada efetivamente levou à melhora da classe funcional (P< 0,0001). Não houve óbitos. CONCLUSÃO: Nesta série de pacientes, o tratamento cirúrgico do canal arterial com técnica convencional em adultos pode ser realizado com baixa morbidade e baixa incidência de complicações.OBJECTIVE: To analyze 34 patients submitted to surgical treatment of patent arterial duct with age beyond 18 years old. METHODS: Retrospective data collected from patient's charts with more than eighteen years old, submitted to surgical correction of patent arterial duct between 1997 and 2008 at Instituto do Coração da Faculdade de Medicina da Universidade de São Paulo. RESULTS: The mean age was 28.7 (18 a 53) years and 22 (64.7%) were female. The more prevalent symptom was dyspnea (76.5%). Left lateral thoracotomy was used in 33 (97.1%); the DA was sectioned and sutured in 25 (73.5%) cases and one patient needed cardiopulmonary bypass support. There were eight (23.5%) calcified arterial duct and 12 (35.3%) previous treatment with transcatheter devices were performed. The complication rate was 32%, with one (2.9%) permanent vocal cord palsy. Two (5.8%) patients had residual shunt less than 2mm. Transient left cord voice palsy was observed in 3 (8.8%) The procedure improves functional class (P< 0.0001) and no mortality was observed. CONCLUSION: In this series, the surgical treatment of patent arterial duct in adults could be done without mortality and low incidence of complications.

OBJETIVO: Reportar a experiência no tratamento cirúrgico da coarctação da aorta (CoAo) em pacientes adultos, avaliando os resultados imediatos e a médio prazo. MÉTODOS: Entre janeiro de 1987 e março de 2000, 50 pacientes consecutivos adultos foram submetidos a tratamento cirúrgico da coarctação de aorta, por toracotomia lateral esquerda. Destes, 42 (84%) eram hipertensos, com pressão arterial sistólica média de 170,6 mmHg (125-220 mmHg). O gradiente médio no local da coarctação era de 51,4 mmHg (18-123 mmHg). A abordagem de doenças cardiovasculares associadas não foi realizada no mesmo tempo cirúrgico, com exceção de dois casos de persistência do canal arterial (PCA). Ressecção da CoAo e anastomose término-terminal foi realizada em 20 (40%) pacientes, ampliação da área de CoAo com retalho de pericárdio bovino em 22 (44%) e interposição de um tubo sintético em oito (16%). RESULTADOS: A morbidade operatória foi baixa, ocorrendo apenas uma reoperação por sangramento; a ocorrência mais frequente nas primeiras horas de pós-operatório foi hipertensão, observada em 98% dos pacientes, controlada com medicamentos endovenosos. Não houve óbito hospitalar. O gradiente residual médio foi de 18,7 mmHg (8-33 mmHg). O tempo médio de internação hospitalar foi de 9,5 dias (5-30 dias). O tempo médio de seguimento foi de 46,8 meses (1145 meses) em 45 (91,8%) pacientes; destes, 41 (91,1%) encontravam-se normotensos, sendo que 75,6% sem medicamentos. Em 93,3% dos pacientes, não ocorreram sintomas de qualquer natureza. Quatro pacientes foram reoperados neste período (um para implante de marca-passo definitivo, dois para troca valvar, e outro por endocardite), ocorrendo um óbito tardio por endocardite e sepse. CONCLUSÃO: O tratamento cirúrgico da CoAo, mesmo em pacientes adultos, impõe-se como método terapêutico eficaz, nesta série, independentemente da técnica cirúrgica utilizada, com baixa morbidade e mortalidade, e sobretudo reduzindo os níveis pressóricos a curto e médio prazosOBJECTIVE: The aim of this study is to describe our experience in aortic coarctation surgery in adult patients by assessing the immediate and mid-term outcomes. METHODS: From January 1987 to March 2000, 50 consecutive adult patients underwent surgery for correction of aortic coarctation, through left lateral thoracotomy. Of these, forty two (84%) patients presented high blood pressure, with mean systolic arterial pressure of 170.56 mmHg (125-220 mmHg). The mean of pressure gradient in the coarctation area was 51.4 mmHg (18-123 mmHg). Other associated surgical cardiovascular diseases were not treated in the same operative act, except in two cases of patent ductus arteriosus (PDA). Three different techniques were used: aortic coarctation resection with end-to-end anastomosis was performed in 20 (40%) patients, coarctation enlargement with bovine pericardial patch was performed in 22 (44%) patients and synthetic tube interposition was performed in eight (16%) patients. RESULTS: Operative morbidity was low; there was one case of bleeding who required reoperation. The most common immediate postoperative event was high blood pressure (98%), but it was easily controlled by intravenous drugs. There was no hospital death. Mean residual pressure gradient was 18.7 (8-33 mmHg). Patients were discharged in 9.5 days (5-30). Postoperative follow-up mean was 46.8 months (1-145 months) in 45 (91.8%) patients. Forty one (91.1%) of these followed-up patients had normal blood pressure, whereas 75.6% of them without drugs intake. 93.3% of these followed-up patients were asymptomatic. Four of them required further surgical operation, one needed a pacemaker implant, other two patients needed a cardiac valve replacement and one had endocarditis. There was one related death due to sepsis secondary to endocarditis. CONCLUSION: Surgical treatment of aortic coarctation, even in adult patients, is an efficient therapeutic choice, regardless of the applied surgical technique, with low morbidity and mortality. It reduces efficiently the arterial pressure levels in both immediate and mid-term follow-up

FUNDAMENTO: A transposição das grandes artérias é a cardiopatia congênita cianogênica mais freqüente no período neonatal, correspondendo a 7% de todas as cardiopatias congênitas. Dentre as operações para tratamento cirúrgico, a operação de Jatene, com correção arterial, é o tratamento escolhido. Durante a evolução pós-operatória tardia, alguns problemas foram observados, sendo o mais comum a ocorrência de estenose supravalvar na neopulmonar, independentemente do tipo da técnica cirúrgica utilizada. OBJETIVO: Estudar e analisar a prevalência da estenose, bem como descrever o tratamento cirúrgico e propor manobras técnicas para prevenir seu aparecimento. MÉTODOS: Dentre 553 pacientes operados, 409 tiveram alta hospitalar e 281 seguidos tardiamente; 59 (20,9%) apresentaram diferentes graus de estenose supravalvar pulmonar e 21 gradiente médio superior a 60 mmHg, necessitando tratamento cirúrgico. Dependendo da localização e da anatomia da estenose, o tratamento cirúrgico constou de aplicação de diferentes técnicas, como ampliação das áreas de estenose com remendos de pericárdio bovino, ressecção de áreas estenóticas e anastomose término-terminal, substituição de remendos retraídos e de tubos sintéticos. RESULTADOS: Houve boa evolução em 20 pacientes, com óbito em um dos casos. CONCLUSÃO: Conclui-se que a estenose supravalvar pulmonar pós-operação de Jatene para transposição das grandes artérias teve prevalência de 20,9%. Uma vez identificada e com indicação de tratamento, pode ser tratada cirurgicamente com baixa mortalidade, mediante diferentes técnicas cirúrgicas. Para prevenir a ocorrência de estenose, propõem-se ampla dissecção e liberação dos ramos pulmonares, anastomoses amplas, remendos amplos de pericárdio autólogo e cuidado na reconstrução da neoaorta, evitando compressão da neopulmonar.BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7% of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9%) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9%; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary.

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%) (positive FISH), and in 3 patients deletion was not detected (negative FISH). Sixteen patients with a positive FISH (94%) 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.

We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment.

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%) and single ventricle was prevalent in group II (14-66%). 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% in G-I and 4.7% in G-II. Significant pleural effusion occurred in 41.4% of G-I patients and in 23.8% of G-II patients. Significant pericardial effusion occurred in 29.2% and 14.2%, 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%) were in functional class I. Sinus rhythm was present in 94%, 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.

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 solitus. 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 DeVega-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.

OBJETIVO: Avaliação de uma nova abordagem cirúrgica para fechamento da comunicação interatrial. MÉTODOS: A abordagem da comunicação interatrial através da janela xifóidea foi realizada em 6 pacientes do sexo masculino e 1 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 acirculação extracorpórea, através de canulação da artéria femoral e átrio direito e videotoracoscopia para auxiliar a visibilização da aorta e do canal arterial, assim como das estruturas correspondentes às cavidades esquerdas. RESULTADOS: Não houve complicações intra-operatórias ou pós-operatórias e, em todos os pacientes, foi possível a extubação na sala de operações. CONCLUSÃO: A janela xifóidea, sem a abertura do esterno, permitiu a correção da comunicação interatrial com bons resultados, podendo constituir-se em uma nova abordagem, minimamente invasiva.PURPOSE: Evaluate a new access for surgical correction of atrial septal defects. METHODS: The access by xiphoid window was done in 7 cases with atria! septal defect type ostium secundum. The age ranged from 6 months to 14 years, mean-age 5.1 years old. In all cases the extracorporeal circulation was done with femoral artery and right atrial cannulation and aortic clamping. The video-assisted endoscopy was utilized to help the visualization of the aorta, ductus arteriosus and the anatomy of the left intracardiac structures. RESULTS: There was no intraoperative or postoperative complications and in our 7 cases the extubation at the operating room was possible. CONCLUSION: The xiphoid window without opening the sternum permited correction of the atrial septal defect with good results and without complications, and could be used as a new technique, less invasive, to correct these defects.

A síndrome do QT longo é caracterizada por prolongação 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 1 paciente sobrevivente. Nos 3 casos foi necessária, após a operação, a utilização de marcapasso átrio-atrial ou atrioventricular. No InCór, entre outubro de 1993 e maio de 1994, 3 pacientes portadores desta síndrome, 1 do sexo feminino e 2 do masculino, com idades, respectivamente, de 10, 11 e 13 anos, foram submetidos a desnervação por autotransplante. No pré-operatório, apesar de dose máxima de β-bloqueadores, os freqüentes episódios de taquicardia ventricular não foram evitados. Com carga mínima o teste ergométrico provocava TVR. A retirada do órgão foi feita seccionando-se as veias cavas, aorta, tronco pulmonar e as veias pulmonares, com remanescente de átrio esquerdo. Assim, o sistema de condução 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 desnervação pode ser empregada em pacientes portadores da síndrome do QT longo, com baixo risco cirúrgico e preservação do sistema de condução.The long QT syndrome is characterized by intermittent or permanent prolongation of the QT interval, ventricular tachyarrhythmias and frequent sudden death. There is three previous reports in the literature, two unsuccessful. In all these patients a postoperative pacemaker was implanted. In our Institute, in October 1993 to May 1994, three patients with this syndrome, one female and two male aged 10,11 and 13 years were submitted to total denervation of the heart through autotransplantation. Despite full dosis of propranolol these patients were severaly symptomatic, with recurrent ventricular tachycardia. The technique was orientated to avoid lesion of the conduction system. The superior and inferior vena cava, the aortic and the pulmonary trunk were transected. Only the posterior wall of the left atrium between the pulmonary veins was incised. After surgery the patients remained in sinus rhythm without episodes of ventricular tachycardia even during serial ergometric test. Considering these preliminary results we recommend the described technique for patients with the long QT syndrome refractarles to adequate medication.

This special issue is dedicated to the common challenge topics in pediatric transplantation. It contains 11 chapters, ranging from clinical research in pediatric transplantation to translational research (from bench to bedside). It includes comprehensive reviews from renowned scientists, clinicians and surgeons from five countries from the International Pediatric Transplantation Association (IPTA), Harvard University, the University of Miami and the University of São Paulo Medical School. The clinical management of specific issues, such as sensitized patients and ABO blood type-incompatible transplantation, is addressed. In addition, the challenges facing this patient population and the future perspectives for clinical research are discussed.

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.Referimos el caso atípico de asociación de bronquitis plástica (BP) con enteropatía perdedora de proteínas (EPP) en una menina de 4 años y 9 meses de edad, con doble vía de entrada de ventrículo único tipo izquierdo y concordancia ventrículo-arterial sometida a la cirugía cavopulmonar total, con túnel lateral intracardiaco a los 3 años. Se consideraron como llamativas la eliminación de molde bronquial de 10 cm formado por fibrina (BP) y la elevación de alfa-1-antitripsina de 52 mg/g de heces. En uso de sildenafila, se programó, en caso de continuidad del proceso, la ligadura del conducto torácico y trasplante 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.

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Os autores relatam o caso de uma criança de 11 anos portadora de bloqueio atrioventricular total, desenvolvido durante correçao cirúrgica de comunicaçoes interatrial e interventricular. Após sete anos de evoluçao assintomática com marcapasso ventricular endocárdico, a criança desenvolveu endocardite bacteriana em valva tricúspide. Quatro meses depois de curada por antibioticoterapia, apresentou estenose tricúspide severa que necessitou de substituiçao valvar. Foram identificadas quatro fatores possivelmente implicados na gênese da estenose tricúspide: traumatismo direto do aparelho valvar pelo cabo-eletrodo, trombose, obliteraçao do orifício valvar por

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.

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.