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Cardiac Magnetic Resonance (CMR) data regarding myocarditis presentation and disease course is still lacking in pediatric patients. We evaluate baseline CMR and evolution of functional and tissue abnormalities in children with acute myocarditis.CMR was performed in 125 patients with clinical diagnosis of acute myocarditis. Clinical follow-up was performed for a median of 498 (214-923) days.LVEF was depressed (55%) in 56 cases (45%) upon baseline CMR. LGE was found in 93 patients (77%) of cases. LGE was exclusively subepicardial in 29 patients (23%), while other LGE patterns (midwall/mixed) were present in 64 (51%). CMR was repeated in 92 (74%) patients. 67% presented recover of function at a median of 170 (70-746) days after onset of symptoms. Midwall/mixed LGE pattern had a statistically significant correlation with absent recover of function (OR 0.20 p 0.036). Thirteen patients (16%) had recovery from LV dysfunction but with persistence of LGE. Sub-epicardial pattern of LGE (OR 3.33, 95% CI 1.08-10.2, p = 0.036) and the presence of fever at admission (OR 4.67, 95% CI 1.16-18.7, p = 0.03) were associated with a significantly higher likelihood of complete normalization while midwall/mixed LGE pattern was associated with non-recovery.In pediatric myocarditis, midwall/mixed LGE pattern is associated with absent recover of function. Patients with recover of function may still have persistence of LGE, while a complete recovery from functional and tissue abnormalities is found only in a third of patients. Midwall/mixed pattern of LGE at first MRI was associated to worse outcome.

Speckle-tracking echocardiography has been used to assess atrial function. This analysis is feasible in univentricular hearts. The aim of this study was to characterize the relationship between atrial strain and functional capacity in the Fontan circulation. Apical four-chamber echocardiographic loops of 39 Fontan patients were reviewed. The dominant atrium was assessed for active (eact), conduit (econ), and reservoir (eres) strain and eact/eres ratio. Cardiopulmonary exercise test was performed on the same day and oxygen uptake (VO2) at ventilatory threshold (VT) and peak VO2 were chosen as the dependent variables. Statistical analysis was performed using SPSS® version 23. Unpaired t test was used for binomial and continuous variable correlation; single and multivariable linear regression were used for continuous variable correlation. Statistical significance was defined as p value

The authors present an image obtained from a routine cardiac magnetic resonance (CMR) examination performed on a 13-year-old boy who had undergone truncus arteriosus type I repair in infancy. Surgery had been unremarkable. An atrial septal defect (ASD) had been left open for atrial decompression. At the age of 3, percutaneous closure was proposed on the grounds of persistent cyanosis, fatigue and right ventricular dilatation. Closure of the defect was achieved with an 11 mm Amplatzer septal occluder (ASO) device, now well visualised in figure 1. Figure 1 Cardiac magnetic resonance (CMR) image, short-axis view. What serious complication was narrowly avoided in the closure procedure? 1. Erosion due to a short aortic rim. 2. Device embolisation due to suboptimal final position. 3. Device-mediated aortic dissection. 4. Myocardial ischaemia …

Background Peak oxygen uptake (peak VO2) and its decline predict death or serious cardiovascular adverse events in patients with Fontan circulation. Our aim was to study VO2 in a population of Fontan patients with variable age and contemporary surgical correction, and find predictors of functional status which could lead to management changes. Methods Prospective cardiopulmonary exercise test was performed on a treadmill. Blood tests and transthoracic echocardiogram were performed on the same day. Dependent variables were defined as: VO2 at ventilatory threshold (VT) as a percentage of the predicted peak VO2 and peak VO2 as a percentage of its predicted value. Statistical analysis was performed on SPSS® version 23 and significance was defined as a p-value Results Forty-eight patients were included for analysis. All had a lateral or extracardiac conduit. Mean age was 18.2 years (SD 6.2). Mean age at Fontan completion of 6.5 years (SD 2.3) showed no association with functional capacity. Mean percent VO2 at VT was 37.6% (SD 9.4) and percent peak VO2 was 67.8% (SD 16.7). VO2 both at ventilatory threshold and peak was associated with age, weekly sports practice, significant atrioventricular regurgitation and having a pacemaker or being on antiarrhythmic drugs. On multivariate analysis, weekly sports practice was the best predictor for VO2 values. Conclusions Sports practice is a modifiable factor that significantly impacts functional capacity in Fontan patients despite their age. Clinicians should actively prescribe and promote physical activity in this population, either with regular sports practice or engagement in cardiac rehabilitation programs.

Myocarditis, defined as inflammation of myocardial tissue, is a rare disease in the pediatric population. Cardiovascular cardiac magnetic resonance (CMR) is a powerful tool for noninvasive assessment of myocardial inflammation as defined by the Lake Louise criteria, with a sensitivity of approximately 82% [1] . CMR allows tissue characterization of the myocardium, as well as accurate definition of ventricular volumes and function. Few prognostic factors have been associated with either normalization of function or progression to dilated cardiomyopathy in pediatric myocarditis. In the present study, we aimed to describe CMR findings in a large cohort of pediatric patients with acute myocarditis and to assess disease evolution. Our study received the proper ethical oversight [CNIL (Commission nationale de l’informatique et des libertes) declaration 2130271 v 0]. Sixty-eight children younger than 18 years of age with clinical myocarditis and positive acute-phase myocardial inflammation, as defined by the Lake Louise criteria [2] , underwent follow-up CMR during a 12-year period (from March 2007 to March 2019). The first follow-up CMR study was performed at a median of 5 months after the first examination (interquartile range: 1 to 6 months), and additional CMR studies were performed in 47% of the cohort (32 patients). Patient characteristics are presented in Table 1 . Most of the cohort were males (73.5%) who presented with chest pain (68%) or new-onset heart failure (33%). Fever was found in 55% of the cohort. Full recovery from acute myocarditis, as defined by normalization of ventricular function [left ventricular ejection fraction (LVEF) > 55%, right ventricular EF > 54%], ventricular dimensions (LV end-diastolic volume index [3] . This difference might translate to a higher healing ability of pediatric patients from tissue damage, as seen in other medical conditions. This complete healing of myocardial tissue abnormalities might be clinically relevant because residual fibrosis was associated with worse prognosis independently of LVEF [3] , [4] . In our study, an LGE pattern other than sub-epicardial was associated with non-recovery. This result was concordant with a recent study by Aquaro et al. [5] , who demonstrated a worse prognosis in association with a mid-wall septal pattern of LGE compared with a sub-epicardial inferior-lateral pattern. In conclusion, we found that fever at presentation was associated with full recovery from acute myocarditis, whereas specific acute-phase CMR findings (positive T2 criteria, LGE presence, and patterns other than sub-epicardial) were associated with its absence.

We present an uncommon challenging case of spontaneous thrombosis of the arterial duct and with alloimmune thrombocytopaenia in a full-term newborn who presented with respiratory distress, hypoglycaemia dispersed petechiae on the trunk, and significant haemorrhage of the umbilical venous catheter.

The authors report the case of an 18-year-old woman with Turner Syndrome and aortic coarctation, who developed aortic dissection after percutaneous stenting. Surgical treatment was necessary as the lesion progressed. This case highlights both the importance of awareness as well as multidisciplinary management of this potential complication.

Contains fulltext : 219893.pdf (Publisher’s version ) (Closed access) BACKGROUND: Treatment of venous thromboembolism in children is based on data obtained in adults with little direct documentation of its efficacy and safety in children. The aim of our study was to compare the efficacy and safety of rivaroxaban versus standard anticoagulants in children with venous thromboembolism. METHODS: In a multicentre, parallel-group, open-label, randomised study, children (aged 0-17 years) attending 107 paediatric hospitals in 28 countries with documented acute venous thromboembolism who had started heparinisation were assigned (2:1) to bodyweight-adjusted rivaroxaban (tablets or suspension) in a 20-mg equivalent dose or standard anticoagulants (heparin or switched to vitamin K antagonist). Randomisation was stratified by age and venous thromboembolism site. The main treatment period was 3 months (1 month in children /=1 dose), were centrally assessed by investigators who were unaware of treatment assignment. Repeat imaging was obtained at the end of the main treatment period and compared with baseline imaging tests. This trial is registered with ClinicalTrials.gov, number NCT02234843 and has been completed. FINDINGS: From Nov 14, 2014, to Sept 28, 2018, 500 (96%) of the 520 children screened for eligibility were enrolled. After a median follow-up of 91 days (IQR 87-95) in children who had a study treatment period of 3 months (n=463) and 31 days (IQR 29-35) in children who had a study treatment period of 1 month (n=37), symptomatic recurrent venous thromboembolism occurred in four (1%) of 335 children receiving rivaroxaban and five (3%) of 165 receiving standard anticoagulants (hazard ratio [HR] 0.40, 95% CI 0.11-1.41). Repeat imaging showed an improved effect of rivaroxaban on thrombotic burden as compared with standard anticoagulants (p=0.012). Major or clinically relevant non-major bleeding in participants who received >/=1 dose occurred in ten (3%) of 329 children (all non-major) receiving rivaroxaban and in three (2%) of 162 children (two major and one non-major) receiving standard anticoagulants (HR 1.58, 95% CI 0.51-6.27). Absolute and relative efficacy and safety estimates of rivaroxaban versus standard anticoagulation estimates were similar to those in rivaroxaban studies in adults. There were no treatment-related deaths. INTERPRETATION: In children with acute venous thromboembolism, treatment with rivaroxaban resulted in a similarly low recurrence risk and reduced thrombotic burden without increased bleeding, as compared with standard anticoagulants. FUNDING: Bayer AG and Janssen Research & Development. 01 januari 2020

Funding Acknowledgements None Introduction Atrial function has recently emerged as a valuable parameter, particularly for evaluation of ventricular diastolic dysfunction and heart failure. There is a strong need for reliable echocardiographic predictors of exercise capacity in univentricular hearts, but their particular anatomy makes it challenging. In this work we aimed to characterize the relationship between atrial strain and exercise parameters in the Fontan population. Methods Fontan patients followed in our outpatient clinic were prospectively evaluated with cardiopulmonary exercise test and transthoracic echocardiogram. The dominant atrium, i.e. the atrium connected to the dominant atrioventricular valve, was assessed with speckle-tracking echocardiography for active (εact), conduit (εcon), and reservoir (εres) strain; and εact/εres ratio. A single cardiac loop from the 4 chamber view was selected for this analysis and the ‘zero’ strain reference for atrial deformation analysis was set at the onset of the electrocardiogram P wave. Exercise capacity defined as the percentage of peak oxygen uptake (VO2), comparing with predicted values, was chosen as the dependent variable. Independent variables were selected among clinical and echocardiographic data. Statistical analysis was performed using SPSS version 23. T-student test was used for binomial and continuous variable correlation; single and multivariable linear regression was used for continuous variable correlation. Statistical significance was defined as p-value < 0.05. Results Fifty-two Fontan patients were assessed. Nineteen (37%) were excluded due to inadequate deformation tracking of the atrial wall. Mean age was 18.0 years (SD 6.9, min. 10.0 - max. 36.0), mean age at Fontan surgery was 7.0 years (SD 2.9, min. 3.0 – max. 18.0). Peak VO2 as a percentage of the predicted value was 66.5% (SD 18.8, min. 36.4 – max. 118.6). εact was -11.1% (SD 3.7, min. -21.1 – max. -4.8), εcon was 10.6% (SD 6.5, min. -0.5 – max. 6.5), εres was 21.7% (SD 5.2, min. 13.2 – max. 34.4) and εact/εres ratio was 0.54 (SD 0.23, min. 0.22 – max. 1.04). On univariate analysis, all atrial strain variables correlated with peak VO2. After adjusting for collinearity, multivariable regression defined age (estimate -1.6, 95% CI: -2.5 to -0.9, p-value < 0.001) and εact strain (estimate 1.8, 95% CI: 0.5 to 3.2, p-value = 0.011) as the strongest predictors of peak VO2 (r2= 0.479). Conclusion Peak VO2 defines exercise capacity and is a strong marker of prognosis in Fontan patients. There are very few echocardiographic variables capable of predicting it, in part due to a variable cardiac anatomy. We showed that atrial strain rate is a novel echocardiographic parameter that predicts peak VO2. In the Fontan circulation, a higher reliance on active atrial contraction for ventricular filling predicts lower exercise capacity. Therefore, atrial strain rate, whenever measurable, may provide a new method of risk stratification in this population. Abstract 1167 Figure. Example of atrial strain curve.

Kawasaki disease (KD) with cardiac involvement can result in the development of coronary aneurysm, stenosis or thrombosis with significant cardiovascular implications. We report the case of a 23-month-old male with a late diagnosis of KD, in whom intravenous immunoglobulin treatment was not feasible. The patient's course was assessed by routine echocardiography. At the age of five years, angiographic assessment revealed an aneurysm of the anterior descending coronary artery measuring 17 mm×7 mm involving the first diagonal branch, 90% post-aneurysmal stenosis, and an aneurysm of the right coronary artery measuring 32 mm×6 mm. Due to the critical anatomy of the anterior descending artery the revascularization method of choice was coronary artery bypass surgery with an internal mammary artery graft, under cardiopulmonary bypass. There were no significant intraoperative or postoperative complications. This confirms coronary artery bypass grafting as a reliable treatment option for patients who present with coronary sequelae from KD, even at a very young age. Resumo: A doença de Kawasaki pode resultar no desenvolvimento de aneurisma, estenose e trombose coronária com importantes implicações cardiovasculares. Reportamos o caso de um doente do sexo masculino de 23 meses com o diagnóstico tardio de doença de Kawasaki, impossibilitando o tratamento atempado com imunoglobulina endovenosa. O doente foi seguido em ambulatório com controlo ecocardiográfico e angiográfico. Aos cinco anos de idade a coronariografia revelava: a) aneurisma da artéria descendente anterior com 17×7 mm, envolvendo a primeira diagonal; b) estenose de 90% pós-aneurismática: c) aneurisma da coronária direita com 32×6 mm. Tendo em consideração a anatomia crítica da lesão envolvendo a artéria descendente anterior, a estratégia de intervenção escolhida foi cirurgia de revascularização miocárdica com um enxerto de artéria mamária esquerda in situ, sob circulação extracorporal. O procedimento decorreu sem intercorrências intra ou pós-operatórias de relevo. A cirurgia de revascularização miocárdica apresenta-se como uma opção viável na presença de sequelas coronárias de doença de Kawasaki, mesmo em doentes muito jovens.

Introdução: A prevalência da síndrome de Down tem aumentado nos últimos 30 anos; 55% destas crianças apresentam cardiopatia congénita.Material e Métodos: Estudo retrospetivo longitudinal de coorte; dados clínicos obtidos em bases de dados de 1982 a 2013 com o diagnóstico de síndrome de Down ou trissomia 21 num hospital de referência em cardiologia pediátrica e cirurgia cardíaca.Objetivo: Avaliar a evolução, nas últimas três décadas, dos cuidados cardiológicos prestados às crianças com síndrome de Down ecardiopatia congénita.Resultados: Estudámos 102 doentes com síndrome de Down e cardiopatia congénita submetidos a terapêutica invasiva: cirurgiacardíaca corretiva, paliativa e cateterismo terapêutico. Em doentes referenciados no primeiro ano de vida, a referenciação foi cada vez mais precoce. O diagnóstico mais frequente foi o defeito completo do septo aurículo-ventricular (41%). Verificou-se uma tendência para cirurgia corretiva cada vez mais precoce em doentes abaixo dos 12 meses (p < 0,001). A partir de 2000, a grande maioria dos doentes foi operada antes dos seis meses de idade. As principais complicações cardíacas foram alterações de ritmo e baixo débito e as principais não cardíacas foram pulmonares e infeciosas. A taxa de mortalidade a 30 dias foi de 3/102 casos (2,9%). Dos doentes em follow-up, 89% estão em classe funcional I da NYHA.Discussão e Conclusão: A correção cirúrgica mais precoce verificada nos últimos 15 anos vai ao encontro do proposto na literatura. A taxa de mortalidade a 30 dias verificada é sobreponível aos resultados internacionais. Os doentes com síndrome de Down submetidos a cirurgia corretiva de cardiopatia congénita apresentam uma excelente capacidade funcional a longo prazo.

BACKGROUND A 30-year-old female with tricuspid valve atresia, ventricular septal defect, and atrial septal defect had a neonatal modified Blalock Taussig shunt and a Fontan-Bjork operation performed at five years of age. She did well initially but progressively developed signs of systemic congestion due to severe homograft stenosis and underwent successful percutaneous implantation of a Melody® pulmonary valve (Medtronic, Minneapolis, MN, USA) in the “tricuspid” position.

Key Clinical Message Venlofaxine, a widely used antidepressant, is known to cause a withdrawal syndrome. We present a case of neonatal transient ventricular dysfunction in a neonate exposed to venlafaxine in utero. Other causes of ventricular dysfunction were excluded. Neonatal ventricular dysfunction can be a possible side effect of maternal use of this drug.

Growth impairment in infants with unrestrictive ventricular septal defects (VSD) is common, and normalisation of growth has been reported after surgical correction. Literature is inconsistent about growth velocity after surgery in term and preterm infants. We aimed to establish the pattern of catch-up growth in term and preterm infants submitted to VSD surgical correction before 1 year of age. Fifty-two infants (41 term, 11 preterm) were studied. Anthropometric data at birth, surgery and 3, 6, 12 and 24 months after surgery were collected retrospectively. Statistic analyses were performed in SPSS® version 21. At the time of surgery, growth was severely impaired in term and preterm infants. Term infants underwent a period of fast growth within the first 6 months after surgery, achieving posteriorly a normal growth pattern, as both weight and height were not significantly different from the reference population at 24 months after surgery. Preterms caught-up later than term infants but with a significant weight gain within 3 months after surgery.Early surgical repair of VSD leads to a significant acceleration of growth within 3 to 6 months after surgery, for both groups.• Growth impairment in infants with unrestrictive ventricular septal defects is well documented in literature. • Surgical correction in the first months of life is the current option for most ventricular septal defects, leading to a more favourable growth pattern. • Rapid growth during infancy may be associated with the development of insulin resistance, metabolic syndrome, obesity and cardiovascular disease later in life. What is New: • Literature is inconsistent about catch-up growth velocities after ventricular correction for term infants. • Preterm infants have never been enrolled in previous studies that aimed to establish a pattern of growth after surgery. • This group of children, who underwent a rapid post-surgery catch-up growth that follows a period of failure to thrive, may be at a higher risk of insulin resistance, metabolic syndrome, obesity and cardiovascular disease.

A 2-month-old infant presented with poor weight gain, feeding intolerance and cardiomegaly on chest radiography. Transthoracic echocardiography showed severe left ventricular dilatation and dysfunction, left atrial dilatation, moderate mitral regurgitation and doubtful origin of left coronary artery (LCA). Cardiac catheterisation did not identify the origin of LCA from the aorta. Coronary CT angiography (128-multidetector CT, retrospective ECG-gated with restrictive dose modulation, 70 kVs, 1.2 mSv) revealed the origin of the LCA from the pulmonary artery trunk/root junction, immediately above the sinus of Valsalva and was consistent with remaining echocardiography findings (figure 1). The right coronary artery had normal origin. After surgical reimplantation of the LCA in the ascending aorta there was good …

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiovascular defect that can range from being fatal early in life to presenting in adulthood asymptomatically. We report the case of a teenager whose diagnosis was incidental and underwent surgery, consisting in coronary artery button transfer, with excellent result.

Cardiac catheterisation in congenital heart disease is a developing field. Patients’ ages range from foetus to adulthood. This document is a revision and update of the previously published recommendations and summarises the requirements for training in diagnostic and interventional cardiac catheterisation.

Aortic coarctation can be repaired surgically or percutaneously. The decision should be made according to the anatomy and location of the coarctation, age of the patient, presence of other cardiac lesions, and other anatomic determinants (extensive collaterals or aortic calcification). This article reviews the different therapeutic options available, explaining the differences between children and adults, describing different approaches to the same disease, exemplified by three cases of nonclassic surgical approach and one percutaneous treatment.

Os hemangiomas hepáticos infantis constituem o terceiro tumor hepático mais frequente na criança e o mais frequente antes dos seis meses. As opções terapêuticas são determinadas pela apresentação clínica, devendo ser individualizadas. Apresenta-se o caso de uma criança actualmente com quatro anos de idade, com diagnóstico neonatal de volumosa malformação hepática vascularizada, com critérios imagiológicos compatíveis com hemangioma hepático infantil. Destaca-se a ocorrência inicial de Síndrome de Kasabach-Merrit (trombocitopenia, anemia) e insuficiência cardíaca que resolveram espontaneamente. Ao longo do período de seguimento, o estudo imagiológico evolutivo (ecografia, doppler ressonância magnética e tomografia computorizada com administração de contraste endovenoso) confirmou a hipótese de HHI ao permitir o mapeamento vascular detalhado. A partir do primeiro ano de vida, constatou-se evolução favorável com redução progressiva da massa. Embora se tenha mantido atitude conservadora, a melhor abordagem e intervenção nesta entidade, permanece controversa. Salientam-se as particularidades deste caso, discutindo a abordagem com melhor relação custo-benefício.

Introdução: Na artroplastia total do joelho e na artroplastia total da anca ocorrem perdas de sangue consideráveis, sendo necessário recorrer frequentemente a transfusões de concentrado de eritrócitos. No entanto, para além dos potenciais riscos inerentes, o sangue homólogo é um recurso limitado e caro. No sentido de reduzir o recurso a transfusões homólogas, tem-se desenvolvido estratégias alternativas, como a recuperação pós-operatória de sangue.Objetivos: Avaliação da implementação da recuperação pós-operatória de sangue no consumo de concentrado de eritrócitos no período pós-operatório e no tempo de internamento, pela avaliação retrospetiva de doentes submetidos a artroplastia total do joelho ou a artroplastia total da anca.Material e Métodos: Os 976 doentes foram divididos em 2 grupos: CELL TRANS – doentes submetidos à intervenção após a implementação da recuperação pós-operatória de sangue e CONT – submetidos à intervenção antes da implementação da estratégia referida. O número de unidades de concentrado de eritrócitos administradas foi obtido pela análise das requisições ao Serviço de Imunohemoterapia e o tempo de internamento foi obtido através do Sistema de Apoio ao Médico. A análise estatística foi realizada com recurso à linguagem R, considerando-se diferenças significativas entre os grupos quando p < 0,05.Resultados: Após a introdução da recuperação pós-operatória de sangue, 29% dos doentes submetidos a artroplastia total do joelho e 38% dos doentes submetidos a artroplastia total da anca receberam concentrado de eritrócitos no período pós-operatório, um número consideravelmente menor ao observado antes da implementação da estratégia, 68% e 59% respetivamente. Concomitantemente, verificou-se uma redução estatisticamente significativa no tempo de internamento de 9,0 para 8,3 dias para o caso dos doentes submetidos a artroplastia total do joelho e de 9,1 para 8,8 dias para o caso dos doentes submetidos a artroplastia total da anca.Conclusões: A recuperação pós-operatória de sangue em doentes submetidos a artroplastia total do joelho ou a artroplastia total da anca reduz significativamente a necessidade de transfusão de concentrado de eritrócitos bem como o tempo de internamento.

Patients with aortic coarctation (CoAo) often have a diastolic flow in the descending aorta. The effect of arterial stiffness on CoAo flow pattern was described in vitro and with computer models. Study of Doppler flow patterns and arterial stiffness may provide helpful data to support the decision of CoAo treatment. Fifty studies were obtained in 31 patients (14 women, 21.5 ± 15.5 years). In 19 patients, studies were performed before and after percutaneous intervention. Systolic invasive gradients were measured (Sgrad). Doppler parameters included Doppler corrected gradient (Dgrad), diastolic velocity at end of T wave (DVT), end diastolic velocity (DVQ), systolic and diastolic half pressure times (SHPTc and DHPTc) and velocity runoff (VRc). In 19 patients, before intervention, arterial stiffness was assessed by measuring pulsed wave velocity (PWV) between right carotid and radial arteries. Sgrad showed correlation with Dgrad, DVT, DVQ, SHPTc, DHPTc and VRc (p 0. In the group with Sgrad below 30 mmHg, a negative correlation was found between DTail and PWV (p = 0.024), suggesting that low aortic stiffness contributes to persistent diastolic flow in the descending aorta. Doppler systolic and diastolic parameters correlated well with severity of CoAo. In mild to moderate CoAo, Doppler diastolic flow in the descending aorta was more likely in patients with lower arterial stiffness.

Resumo: Introdução: A miocardite viral pode cursar com insuficiência cardíaca refratária à medicação. Nestes casos, a assistência ventricular externa é uma alternativa que pode ser usada como ponte para transplante ou para recuperação. Descrevemos o primeiro caso em Portugal de recuperação da função ventricular após assistência ventricular por miocardite grave. Caso clínico: Apresentamos o caso de uma criança de 5 anos, sexo masculino, sem doença cardíaca prévia, com miocardite viral grave, insuficiência cardíaca refratária à medicação e serologias positivas para Parvovírus B19 e vírus Ebstein-Barr. Foi implantado o Berlin Heart Excor® 15 dias após o diagnóstico. A biopsia cardíaca, na altura da implantação, mostrava áreas de fibrose subendocárdica. Verificou-se recuperação da função miocárdica, tendo sido retirada a assistência ventricular após 40 dias da implantação. O doente teve alta 15 dias depois. Discussão: A sobrevida das crianças em assistência ventricular tem vindo a melhorar significativamente, devido ao planeamento atempado e à otimização da anticoagulação. A presença de fibrose subendocárdica no contexto de miocardite não constitui uma indicação formal para transplante. Abstract: Introduction: Viral myocarditis can lead to heart failure that is refractory to medication. In these cases, a ventricular assist device is a good therapeutic option that can be used as a bridge to transplantation or recovery. We describe the first case in Portugal of recovery with ventricular assistance after severe myocarditis. Case report: A five-year-old boy with no previous cardiac disease presented with severe viral myocarditis, refractory to medical treatment, with positive serology for parvovirus B19 and Ebstein-Barr virus. A Berlin Heart Excor® was implanted 15 days after diagnosis. A biopsy at the time of implantation showed subendocardial fibrosis. After 40 days of assistance ventricular function recovered and the device was explanted. The patient was discharged from hospital 15 days later. Discussion: Survival of children with ventricular assistance has improved significantly because of earlier implantation and coagulation monitoring. The presence of subendocardial fibrosis in the context of myocarditis is not a mandatory indication for transplantation. Palavras-chave: Assistência ventricular externa, Berlin Heart Excor®, Insuficiência cardíaca, Miocardite viral, Keywords: Assisted circulation, Circulatory assist devices, Heart failure, Viral myocarditis

Patients with Ebstein's anomaly of the tricuspid valve may have right-to-left shunt at atrial level resulting in hypoxaemia, high haematocrit and hyperviscosity syndrome. The purpose of this study was to assess the results of percutaneous closure of atrial right-to-left shunt in patients with Ebstein's anomaly.Records of patients treated between January 2002 and June 2010 were reviewed. Their condition before and after shunt closure (clinical data, oxygen saturation and haematocrit) were studied. During this period nine selected patients with Ebstein's anomaly and right-to-left shunt at atrial level were treated. Ages ranged from six to 67 years; seven were male. Mean pulmonary artery pressures were under 25 mmHg in all. Three patients had previous episodes of stroke and three had very high haematocrit, two of whom required therapeutic phlebotomies. Test occlusion of the shunt was performed in all patients with a balloon catheter, revealing an increase in systemic oxygen saturation, with right atrial pressures remaining18 mmHg in all. Percutaneous closure of atrial shunt was achieved in all. There were no major complications. Arterial oxygen saturations increased in all patients from 85.0 ± 4.5% to 96.7 ± 1.5% (mean ± standard deviation). Medium follow-up was five years. The three patients with very high haematocrit levels had a decrease in its values from 62.9 ± 2.8% to 45.5 ± 3.9% after device occlusion. Both therapeutic phlebotomy programs were discontinued. All patients reported a marked improvement in effort tolerance.Percutaneous closure of atrial right-to-left shunt in selected patients with Ebstein's anomaly offers significant improvement, abolishing hypoxaemia and hyperviscosity and preventing paradoxical embolisation.

A 2-day-old male infant required a conventional VVI pacemaker for congenital atrioventricular block. Three years later, he developed progressive heart failure due to left ventricular (LV) dysfunction and mitral regurgitation despite optimized medical treatment, and a cardiac resynchronization therapy (CRT) device was implanted. This is the first Portuguese report of CRT in a pediatric patient.One-year echocardiographic follow-up showed that LV shortening fraction had improved and LV end-diastolic dimension and mitral regurgitation had decreased. New York Heart Association class had improved from III–IV to I at 1-year follow-up. Resumo: Um recém-nascido do sexo masculino apresenta bloqueio auriculo-ventricular completo pelo que foi implantado um pacemaker VVI ao 2.° dia de vida.Três anos depois o doente desenvolve disfunção ventricular esquerda, regurgitação mitral grave e insuficiência cardíaca progressiva apesar da terapêutica médica otimizada. Nesse contexto efetuamos terapêutica de ressincronização (CRT). Este é o primeiro relato português de implantação de CRT em idade pediátrica.Um ano após a implantação o ecocardiogrma mostra melhoria da fração de ejeção, redução do diâmetro telediastólico do ventrículo esquerdo e redução muito significativa da regurgitação mitral. A classe funcional de New York Heart Association melhorou de III-IV para I. Keywords: Cardiac resynchronization therapy, Cardiomyopathy after right ventricular pacing, Reverse remodeling, Palavras-chave: Terapêutica de ressincronização cardíaca (CRT), Miocardiopatia induzida pelo pacing direito, Remodelagem reversa

Resumo: Introdução: Uma fístula coronária é uma conexão entre uma artéria coronária e uma cavidade cardíaca ou grande vaso. É um defeito raro e que geralmente ocorre de forma isolada. Apesar de a ecocardiografia bidimensional ter um papel importante no diagnóstico, a angiotomografia computorizada ou a angiografia coronária são importantes para a delineação da anatomia. A cirurgia é o tratamento tradicional, mas o encerramento percutâneo é actualmente o método recomendado, com excelentes resultados e baixa morbilidade em grupos com experiência em embolização terapêutica. População e métodos: Descrevemos a experiência do nosso centro no tratamento percutâneo de 15 fístulas coronárias em 12 doentes entre 1996 e 2011. Oito (67%) eram do sexo masculino. A idade mediana foi de 25 anos. Os motivos de referência mais frequentes foram sopro e/ou cansaço. Todas as fístulas eram de origem congénita. Cinco doentes (42%) tinham patologia cardíaca concomitante: atrésia da pulmonar com septo interventricular intacto (1), canal arterial persistente (1), comunicação interauricular ostium secundum (1), estenose aórtica em válvula aórtica biscúspide (1), estenose pulmonar crítica operada no período neonatal (1). Três doentes tinham duas fístulas coronárias e nos restantes a lesão era única. As fístulas eram hemodinamicamente significativas, com origem no território da coronária direita (n = 10), da coronária esquerda (n = 3) e da circunflexa (n = 2) e drenavam para o ventrículo direito (n = 5), artéria pulmonar (n = 6), aurícula direita (n = 2), seio coronário (n = 1) e ventrículo esquerdo (n = 1). O material de embolização incluiu coils standard, coils de libertação controlada, microcoils (simples, GDC ou IDC) e um dispositivo Amplatzer® Duct Occluder. Resultados: A embolização da fístula foi obtida em todos os doentes. Não houve mortalidade. Ocorreu enfarte do ventrículo direito num doente com fístula de grandes dimensões e coronária direita de diâmetro muito reduzido distalmente à emergência da fístula. Em três casos ocorreram complicações minor: embolização inadvertida de coil, recuperado no mesmo procedimento (1), arritmia transitória (1) e pseudo-aneurisma femoral (1).Num seguimento médio de 4,9 anos (um mês a 14 anos) não se registaram intercorrências relacionadas com o procedimento. O controlo por ecocardiograma e/ou angiografia coronária mostrou que em dez doentes ocorreu oclusão eficaz e permanente das fístulas, enquanto que dois doentes mantiveram fluxo residual mínimo, por pequenos vasos acessórios, sem repercussão hemodinâmica. Conclusão: As embolizações terapêuticas representam uma forma eficaz de abordagem de fístulas coronárias seleccionadas. Estas anomalias têm características muito variáveis, pelo que é necessário dispor de um grande leque de dispositivos para seleccionar a opção mais eficaz. Abstract: Introduction: A coronary fistula is a connection between one of the coronary arteries and a cardiac chamber or great artery. It is a rare defect and usually occurs in isolation. Two-dimensional echocardiography has an important role in diagnosis but coronary or CT angiography is essential to delineate the anatomy. Surgery is the traditional therapeutic approach but percutaneous closure is now the recommended method, with excellent results and few complications in experienced centers. Methods: We describe our experience with percutaneous treatment of 15 coronary fistulas in 12 patients between 1996 and 2011. Eight (67%) were male and median age was 25 years. The most frequent symptoms were murmur and/or fatigue. All fistulas were congenital. Five patients (42%) had concomitant cardiac disease: pulmonary atresia with intact ventricular septum (1), patent ductus arteriosus (1), ostium secundum atrial septal defect (1), stenotic bicuspid aortic valve (1), and critical pulmonary stenosis operated in the neonatal period (1). Three patients had two fistulas, while the others had a single lesion. All fistulas were hemodynamically significant. They originated in the territory of the right coronary (10), left coronary (3) and circumflex (2), draining into the right ventricle (5), pulmonary artery (6), right atrium (2) coronary sinus (1) and left ventricle (1). Embolization materials included standard coils, controlled-release coils, microcoils (standard, GDC or IDC) and an Amplatzer® duct occluder. Results: Embolization was achieved in all patients. There was no mortality. One patient with a large fistula and a very small right coronary artery distally to the origin of the fistula had a right ventricular infarction. In three patients there were minor complications: inadvertent coil embolization, recovered in the same procedure (1), transient arrhythmia (1) and femoral pseudo-aneurysm (1). In a mean follow-up of 4.9 years (one month to 14 years), there were no procedure-related complications. Echocardiographic and/or angiographic control showed complete and permanent occlusion in ten patients and minimal residual flow in two patients through small collaterals with no hemodynamic significance. Conclusion: Percutaneous embolization represents an effective form of treatment for selected coronary fistulas. A wide range of embolization devices must be available to ensure the best therapeutic approach. Palavras-chave: Fístulas coronárias, Embolização, Intervenção percutânea, Keywords: Coronary fistulas, Embolization, Percutaneous intervention

Introdução: As cardiopatias congénitas complexas são patologias graves, e o diagnóstico pré-natal poderá ter implicações sobre a morbilidade e a mortalidade. O objetivo deste trabalho foi estudar a influência do diagnóstico pré-natal e da distância do local de parto a um centro de referência, na morbilidade imediata e mortalidade precoce de um grupo de doentes com cardiopatias congénitas complexas.Material e Métodos: Análise retrospetiva dos doentes com cardiopatias congénitas complexas, seguidos no nosso hospital, nascidos entre 2007 e 2012.Resultados: Identificaram-se 126 doentes com cardiopatias congénitas complexas. Em 95% a gravidez foi vigiada desde o primeiro trimestre existindo diagnóstico pré-natal em 42%. Houve relação estatisticamente significativa entre o local do parto e a existência de diagnóstico pré-natal. A cardiopatia congénita complexa mais frequente foi a transposição das grandes artérias (45,2%), seguida da atresia da pulmonar com comunicação interventricular (17,5%) e ventrículo esquerdo hipoplásico (9,5%). Oitenta e dois doentes (65,1%) foram medicados com prostaglandinas e 38 (30,2%) foram ventilados antes de uma intervenção. A cirurgia ocorreu no período neonatal em 73%. A sobrevida atuarial aos 30 dias, 12 e 24 meses foi 85%, 80% e 75%, respetivamente. Não houve relação estatisticamente significativa entre diagnóstico pré-natal e mortalidade.Discussão: A maioria dos doentes com cardiopatias congénitas complexas não teve diagnóstico pré-natal. Nos casos com diagnóstico pré-natal houve referenciação e parto num centro terciário. Não houve associação estatisticamente significativa entre diagnóstico prénatal e mortalidade neonatal, como já descrito em séries heterogéneas de cardiopatia congénita complexa.Conclusão: A maioria dos doentes com cardiopatias congénitas complexas não teve diagnóstico pré-natal. Estes dados devem ser tomados em conta no planeamento do diagnóstico pré-natal das cardiopatias congénitas.

Pulmonary atresia with ventricular septal defect, hypoplastic pulmonary arteries and major aortopulmonary collateral arteries is a complex and heterogeneous cardiac disease. Currently, there are two different major surgical approaches: one involves major aortopulmonary collateral arteries unifocalisation, connecting them to the native pulmonary arteries circuit, connection of the right ventricle to the ‘neo-pulmonary arteries’ and concomitant/delayed ventricular septal defect closure. The other option is …

Constrictive pericarditis is an uncommon disease in children, usually difficult to diagnose. We present the case of a 14-year-old boy with a previous history of tuberculosis and right heart failure, in whom constrictive pericarditis was diagnosed. The case highlights the need to integrate all information, including clinical data, noninvasive cardiac imaging, and even invasive hemodynamic evaluation when required, in order to establish the correct diagnosis and proceed to surgical treatment.

Platypnea orthodeoxia is a rare syndrome characterized by dyspnea and decreased arterial oxygen saturation when passing from the dorsal decubitus position to the sitting or upright position1. It usually results from an intracardiac right-to-left shunt; however, the pathophysiological mechanism responsible for the positional nature of the shunt has not been clarified1,2. We report a case of dyspnea and orthostatic hypoxemia, which was attributed to a right-to-left shunt due to atrial septal defect (ASD).

Objectives: This study aimed to characterize the prevalence of congenital heart disease (CHD) in children born alive in São Miguel island from January 1992 to December 2001. Methods: Based on the Azorean Registry of CHD, which includes complete clinical and personal information, 189 patients were diagnosed. Results: During this 10-year period, the average prevalence of CHD is 9.16 per 1,000 live births (range 4.77–12.75). The most frequent cardiac malformations found were: ventricular septal defect (38.1%), atrial septal defect (12.2%) and patent ductus arteriosus (11.6%). Until now, four familial clusters were identified, representing a total of 13 patients. Conclusions: This first epidemiological study of CHD in the Azorean population reveals evidence for familial aggregation, which is of great interest for understanding the genes involved in these complex pathologies.

Aortic coarctation is occasionally detected only in adulthood, usually in the context of systemic hypertension. It is frequently associated with other malformations, but the presence of severe disease of the aortic or mitral valves is rare. Such associated lesions, nonetheless, have important implications regarding the type and timing of therapeutic interventions. We describe an adult patient with severe aortic coarctation, aortic valvar regurgitation, and impaired left ventricular systolic function. We treated the aortic coarctation first by means of percutaneous dilation and implantation of a stent. Four days later, we proceeded to treat the aortic regurgitation surgically, using the Bentall procedure. Our experience documents a safe and efficient therapeutic approach to this association of lesions.

Lipomatous hamartoma of cardiac valves is a very rare entity, with only three reported cases in children. We describe the case of a 9-year-old girl with a mass in the mitral valve, which was detected in an echocardiogram performed for heart murmur investigation. At surgery, a white round-shaped tumour was removed and histopathological examination revealed a lipomatous hamartoma.

A case of ventricular septal tumor diagnosed in 7-month-old boy is reported. An echocardiogram was performed for investigation of a heart murmur in an otherwise healthy infant. He remained asymptomatic, and the tumor had a very slow growth. When the boy was 9 years old, partial surgical excision was performed for right ventricular outflow tract obstruction. Histology showed a primary cardiac leiomyoma. To the authors’ knowledge, a primary cardiac leiomyoma of the ventricular septum has not been reported previously.

Platypnea-orthodeoxia syndrome (POS) is an uncommon syndrome characterized by dyspnea and hypoxemia triggered by orthostatism and relieved by recumbency. It is often associated with an interatrial shunt through a patent foramen ovale (PFO). We report the case of a 92-year-old woman initially admitted in the setting of a traumatic femoral neck fracture (successfully treated with hip replacement surgery) in whom a reversible decline in transcutaneous oxygen saturation from 98% (in the supine position) to 84% (in the upright position) was noted early post-operatively. Thoracic multislice computed tomography excluded pulmonary embolism and severe parenchymal lung disease. The diagnosis of POS was confirmed by tilt-table contrast transesophageal echocardiography, which demonstrated a dynamic and position-dependent right-to-left shunt (torrential when semi-upright and minimal in the supine position) through a PFO. The patient underwent percutaneous closure of the PFO with an Amplatzer device, which led to prompt symptom relief and full functional recovery. Resumo: A síndrome platipneia-ortodeoxia (SPO) é uma entidade rara caracterizada por dispneia e hipoxemia desencadeadas pelo ortostatismo e aliviadas pelo decúbito. Está frequentemente associada à presença de um shunt inter-auricular através de um foramen ovale patente (FOP). Relata-se o caso de uma mulher de 92 anos, internada inicialmente por fratura traumática do colo do fémur. Foi submetida a artroplastia da anca sem complicações. No período pós operatório inicial observou-se um declínio reversível da saturação de oxigênio de 98% em decúbito dorsal para 84% na posição ortostática. A angio-tomografia computorizada do tórax excluiu trombo-embolia pulmonar e doença grave do parênquima pulmonar. O diagnóstico de SPO foi confirmado por ecocardiografia transesofágica contrastada (soro agitado) com inclinação na mesa de tilt, que demonstrou um shunt direito-esquerdo dinâmico e posicional (torrencial a 45° e mínimo a 0°) através de um FOP. A doente foi submetida a encerramento percutâneo do FOP com dispositivo Amplatzer, que proporcionou alívio sintomático imediato e permitiu uma recuperação funcional total. Keywords: Platypnea-orthodeoxia syndrome, Tilt table, Transesophageal echocardiography, Percutaneous closure, Patent foramen ovale, Palavras-chave: Síndrome platipneia-ortodeoxia, Inclinação dinâmica, Ecocardiografia transesofágica, Encerramento percutâneo, Foramen ovale patente

We report a rare case of CHD and abdominal aorta interruption. Renal hypertension was the first sign of the abdominal aortic malformation. The aetiology of abdominal aortic interruption remains unclear, it could be congenital or acquired.

Pulmonary artery anatomy was studied in 36 patients (age range 12 days to 12.83 years, mean 2.9 years) with right ventricular outflow tract obstruction over a 3-year period using a 1.5 Tesla Magnetic Resonance system. Gated spin-echo sequences were obtained in each patient. Image planes used were straight and oblique transverse, oblique coronal, and oblique sagittal. The studies were analyzed retrospectively and comparative angiographic, surgical, and postmortem data was available in all cases. As previously noted with other imaging modalities, multiple views and oblique cuts were found to increase significantly the quality and accuracy of the information acquired. In 12 of the 15 patients who had axial and coronal imaging of both branch pulmonary arteries, the pulmonary arteries were unequivocally demonstrated to at least the first hilar branch. However, early in our experience we missed a number of branch stenoses that developed following surgical anastomoses. These mistakes were caused by inadequate or inappropriate slice acquisition. Magnetic resonance is an important technique for imaging pulmonary arteries. Careful use of the appropriate imaging planes is essential for accurate demonstration of the anatomy.

Acute kidney injury (AKI) is a common complication in children after surgery for congenital heart disease, and peritoneal dialysis (PD) is usually the renal replacement therapy (RRT) of choice, especially in very young children. The aim of the present study was to describe our experience of using PD to treat AKI after cardiac surgery. We retrospectively analyzed children 1 week to 16 years of age undergoing cardiac surgery during 2000 – 2008 and found the incidence of AKI treated with PD to be 2.3%. In the 23 patients treated with PD (13 male; average age: 29 ± 48.4 months; weight: 9.1 ± 8.1 kg), the indications for PD initiation were oliguria ( n = 13), anuria ( n = 9), and acidosis ( n = 1). The average time between cardiac surgery and AKI was 4.8 ± 16.8 hours, and between AKI and PD initiation, it was 12 ± 16.8 hours. Patients were treated for a mean of 4.8 ± 3.8 days. Two patients developed peritonitis, and mechanical dysfunction of the PD catheter occurred in 1 patient. In-hospital mortality was 43.4%. Patients treated with PD weighed less ( p = 0.004) and had longer bypass time ( p = 0.004), inotrope use ( p = 0.000), and mechanical ventilation ( p = 0.000). However, in a regression analysis, only cardiopulmonary bypass time (odds ratio: 1.021; 95% confidence interval: 0.998 to 1.027; p = 0.032) remained predictive of a subsequent need for PD. We conclude that PD is an efficacious RRT for AKI in children undergoing cardiac surgery and that, in this setting, bypass time is the strongest predictor of a subsequent need for RRT.

Introduction: A coronary fistula is a connection between one of the coronary arteries and a cardiac chamber or great artery. It is a rare defect and usually occurs in isolation. Two-dimensional echocardiography has an important role in diagnosis but coronary or CT angiography is essential to delineate the anatomy. Surgery is the traditional therapeutic approach but percutaneous closure is now the recommended method, with excellent results and few complications in experienced centers. Methods: We describe our experience with percutaneous treatment of 15 coronary fistulas in 12 patients between 1996 and 2011. Eight (67%) were males and the median age was 25 years. The most frequent symptoms were murmur and/or fatigue. All fistulas were congenital. Five patients (42%) had concomitant cardiac disease: pulmonary atresia with intact ventricular septum (1), patent ductus arteriosus (1), ostium secundum atrial septal defect (1), stenotic bicuspid aortic valve (1), and critical pulmonary stenosis operated in the neonatal period (1). Three patients had two fistulas, while the others had a single lesion. All fistulas were hemodynamically significant. They originated in the territory of the right coronary (10), left coronary (3) and circumflex (2), draining into the right ventricle (5), pulmonary artery (6), right atrium (2) coronary sinus (1) and left ventricle (1). Embolization materials included standard coils, controlled-release coils, microcoils (standard, GDC or IDC) and an Amplatzer® duct occluder. Results: Embolization was achieved in all patients. There was no mortality. One patient with a large fistula and a very small right coronary artery distally to the origin of the fistula had a right ventricular infarction. In three patients there were minor complications: inadvertent coil embolization, recovered in the same procedure (1), transient arrhythmia (1) and femoral pseudo-aneurysm (1). In a mean follow-up of 4.9 years (1 month to 14 years), there were no procedure-related complications. Echocardiographic and/or angiographic control showed complete and permanent occlusion in ten patients and minimal residual flow in two patients through small collaterals with no hemodynamic significance. Conclusion: Percutaneous embolization represents an effective form of treatment for selected coronary fistulas. A wide range of embolization devices must be available to ensure the best therapeutic approach. Resumo: Introdução: Uma fístula coronária é uma conexão entre uma artéria coronária e uma cavidade cardíaca ou grande vaso. É um defeito raro e que geralmente ocorre de forma isolada. Apesar da ecocardiografia bidimensional ter um papel importante no diagnóstico, a angiotomografia computorizada ou a angiografia coronária, é essencial para a delineação da anatomia. A cirurgia é o tratamento tradicional, mas o encerramento percutâneo é actualmente o método recomendado, com excelentes resultados e baixa morbilidade em grupos com experiência em embolização terapêutica. População E Métodos: Descrevemos a experiência do nosso centro no tratamento percutâneo de 15 fístulas coronárias em 12 doentes entre 1996 e 2011. Oito (67%) eram do sexo masculino. A idade mediana foi 25 anos. Os motivos de referência mais frequentes foram sopro e/ou cansaço. Todas as fistulas eram de origem congénita. Cinco doentes (42%) tinham patologia cardíaca concomitante: atrésia da pulmonar com septo interventricular intacto (1), canal arterial persistente (1), comunicação interauricular ostium secundum (1), estenose aórtica em válvula aórtica biscúspide (1), estenose pulmonar crítica operada no período neonatal (1). Três doentes tinham duas fístulas coronárias e nos restantes a lesão era única. As fístulas eram hemodinamicamente significativas, com origem no território da coronária direita (n=10), da coronária esquerda (n=3) e da circunflexa (n=2) e drenavam para o ventrículo direito (n=5), artéria pulmonar (n=6), aurícula direita (n=2), seio coronário (n=1) e ventrículo esquerdo (n=1). O material de embolização incluiu coils standard, coils de libertação controlada, microcoils (simples, GDC ou IDC) e um dispositivo Amplatzer® Duct Occluder. Resultados: A embolização da fístula foi obtida em todos os doentes. Não houve mortalidade. Ocorreu enfarte do ventrículo direito num doente com fístula de grandes dimensões e coronária direita de diâmetro muito reduzido distalmente à emergência da fístula. Em três casos ocorreram complicações minor: embolização inadvertida de coil, recuperado no mesmo procedimento (1), arritmia transitória (1) e pseudo-aneurisma femoral (1).Num seguimento médio de 4,9 anos (um mês a 14 anos) não se registaram intercorrências relacionadas com o procedimento. O controlo por ecocardiograma e/ou angiografia coronária mostrou que em dez doentes houve ocorreu oclusão eficaz e permanente das fístulas, enquanto que dois doentes mantiveram fluxo residual mínimo, por pequenos vasos acessórios, sem repercussão hemodinâmica. Conclusão: As embolizações terapêuticas representam uma forma eficaz de abordagem de fístulas coronárias seleccionadas. Estas anomalias têm características muito variáveis, pelo que é necessário dispor de um grande leque de dispositivos para seleccionar a opção mais eficaz. Keywords: Coronary fistulas, Embolization, Percutaneous intervention, Palavras-chave: Fístulas coronárias, Embolização, Intervenção percutânea

OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations.

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology, which is the main cause of acquired heart disease in children in developed countries. The main complications result from the development of coronary aneurysms which can lead to ischemic heart disease.We present the case of a teenage boy with a diagnosis of KD at the age of seven. He was treated with gammaglobulin and aspirin and echocardiographic evaluation in the acute phase was apparently normal. At the age of 11, he developed chest pain and exertional dyspnea. Nuclear perfusion scans with exercise revealed hypoperfusion of the left anterior descending (LAD) and right coronary artery (RCA) territories. Cardiac catheterization showed occlusion of the proximal segments of both arteries. He underwent coronary artery bypass graft surgery (internal mammary artery bypass graft to the LAD and saphenous vein graft to the RCA), with a good clinical result.This case report highlights the importance of early diagnosis and treatment of KD and regular cardiological follow-up, bearing in mind the potential late complications of this pediatric disease. Resumo: A doença de Kawasaki (DK) é uma vasculite sistémica, de etiologia desconhecida, constituindo a principal causa de cardiopatia adquirida em idade pediátrica em países desenvolvidos. As principais complicações resultam do aparecimento de aneurismas coronários que podem evoluir para doença coronária isquémica.Apresenta-se o caso clínico de um adolescente com diagnóstico de DK aos 7 anos. Efetuou terapêutica com imunoglobulina e ácido acetilsalicílico e a avaliação ecocardiográfica na fase aguda foi aparentemente normal. Aos 11 anos de idade desenvolveu quadro de angor e dispneia de esforço. A cintigrafia de perfusão miocárdica com prova de esforço revelou hipoperfusão dos territórios correspondentes às artérias descendente anterior esquerda (DA) e coronária direita (CD). O cateterismo cardíaco demonstrou oclusão dos segmentos proximais de ambas as artérias. Foi submetido a cirurgia de revascularização coronária (artéria mamária interna para a DA e veia safena interna para a CD) com boa evolução clínica e desaparecimento das alterações isquémicas na cintigrafia.Este caso clínico vem alertar para a importância do diagnóstico e terapêutica atempados e seguimento posterior na DK, salientando-se a potencial gravidade das complicações cardiovasculares a longo prazo, desta doença pediátrica. Keywords: Kawasaki disease, Coronary aneurysm, Coronary artery bypass, Palavras-chave: Doença de Kawasaki, Aneurismas coronários, Cirurgia de revascularização coronária

Introduction: Viral myocarditis can lead to heart failure that is refractory to medication. In these cases, a ventricular assist device is a good therapeutic option that can be used as a bridge to transplantation or recovery. We describe the first case in Portugal of recovery with ventricular assistance after severe myocarditis. Case report: A five-year-old boy with no previous cardiac disease presented with severe viral myocarditis, refractory to medical treatment, with positive serology for parvovirus B19 and Ebstein-Barr virus. A Berlin Heart Excor® was implanted 15 days after diagnosis. A biopsy at the time of implantation showed subendocardial fibrosis. After 40 days of assistance ventricular function recovered and the device was explanted. The patient was discharged from hospital 15 days later. Discussion: Survival of children with ventricular assistance has improved significantly because of earlier implantation and coagulation monitoring. The presence of subendocardial fibrosis in the context of myocarditis is not a mandatory indication for transplantation. Resumo: Introdução: A miocardite viral pode cursar com insuficiência cardíaca refractária à medicação. Nestes casos, a assistência ventricular externa é uma alternativa que pode ser usada como ponte para transplante ou para recuperação. Descrevemos o primeiro caso em Portugal de recuperação da função ventricular após assistência ventricular por miocardite grave. Caso Clínico: Apresentamos o caso de uma criança de cinco anos, sexo masculino, sem doença cardíaca prévia, com miocardite viral grave, insuficiência cardíaca refractária à medicação e serologias positivas para Parvovírus B19 e vírus Ebstein-Barr. Foi implantado o Berlin Heart Excor® 15 dias após o diagnóstico. A biopsia cardíaca, na altura da implantação, mostrava áreas de fibrose subendocárdica. Verificou-se recuperação da função miocárdica, tendo sido retirada a assistência ventricular após 40 dias da implantação. O doente teve alta 15 dias depois. Discussão: A sobrevida das crianças em assistência ventricular tem vindo a melhorar significativamente, devido ao planeamento atempado e à optimização da anti-coagulação. A presença de fibrose subendocárdica no contexto de miocardite não constitui uma indicação formal para transplante. Keywords: Assisted circulation, Circulatory assist devices, Heart failure, Viral myocarditis, Palavras-chave: Assistência ventricular externa, Berlin Heart Excor®, Insuficiência cardíaca, Miocardite viral

We present a patient in whom the heart was right-sided, with usual atrial position, absence of the right-sided atrioventricular connexion, and a single outlet via the aorta from a dominant morphologically right ventricle. We use the case to illustrate potential problems in the terminology as used to describe the absence of one atrioventricular connexion. Our case shows why absence of one atrioventricular connexion must be classified according to the side of the missing connexion, and not the anatomy of the atrial or ventricular chambers involved. To simplify the approach to certain hearts, the use of the concept of one concordant or discordant atrioventricular connexion may be justified when the other atrioventricular connexion is absent.