Isolated unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly in which one branch pulmonary artery has no connection to the main pulmonary trunk (most often there is ductal origin). Without treatment, it may lead to ipsilateral pulmonary hypoplasia and contralateral pulmonary artery hypertension. To avoid these complications, early surgical repair of UAPA is necessary. Surgical strategies include direct anastomosis between the "isolated" branch pulmonary artery (PA) and the main pulmonary trunk or creation of an interposition graft using prosthetic material or flap techniques. We describe a surgical technique using a totally autologous interposition tube graft.

As vantagens da plastia valvar mitral são menor morbimortalidade, redução do risco de tromboembolismo e endocardite, melhora na sobrevida e melhor preservação da função ventricular esquerda. A causa mais comum de regurgitação mitral degenerativa é a válvula mixomatosa com prolapso segmentar do folheto posterior; esta lesão pode ser corrigida por técnicas clássicas, como a ressecção quadrangular, mas outras lesões podem exigir técnicas cirúrgicas mais complexas, por exemplo, correção de prolapso do folheto anterior ou doença de Barlow. A plastia valvar [...]

INTRODUCTION:Veno-arterial extracorporeal membrane oxygenation is well-established for pediatric patients with post-cardiotomy heart failure. However, extracorporeal membrane oxygenation support is associated with major complications, that is, hemorrhage and thromboembolism. We seek to report our experience with delayed systemic heparinization during neonatal cardiac extracorporeal membrane oxygenation and its impact on bleeding and thromboembolism.METHODS:We retrospectively identified 15 consecutive neonates who were placed on extracorporeal membrane oxygenation after congenital heart surgery during a period of 3 years (2015-2017). Our anticoagulation protocol consisted of full heparin reversal by protamine after switching from cardiopulmonary bypass to extracorporeal membrane oxygenation (target activated clotting time: 120 ± 20 seconds). Administration of systemic heparinization was delayed until postoperative drainage volume declined to <1 mL/kg/h. Primary study endpoints were thromboembolism, bleeding, and requirement of blood products on extracorporeal membrane oxygenation.RESULTS:Our cohort (mean age: 13 ± 2.6 days; mean weight: 3.1 ± 0.3 kg; 66.7% male) required post-cardiotomy extracorporeal membrane oxygenation with a mean support time of 4.5 ± 2.2 days. Systemic heparinization was delayed averagely for 18.1 ± 9.3 hours. No thromboembolic events were observed on extracorporeal membrane oxygenation or after weaning. Relevant surgical site bleeding occurred in two patients (13.3%) requiring re-thoracotomy on the first postoperative day. Analysis of transfusion volumes revealed 24.5 ± 21.9 mL/kg/d mean packed red blood cells, 9.6 ± 7.1 mL/kg/d mean fresh frozen plasma, and 7.5 ± 5.7 mL/kg/d mean platelets. In-hospital survival was 86.6% (n = 13).CONCLUSION:In this retrospective analysis, the results of delayed systemic heparinization in neonatal post-cardiotomy extracorporeal membrane oxygenation could lead one to conclude that this routine is safe and favorable with low risk for thromboembolic events, reduced postoperative hemorrhage, and reduced blood product utilization.

The long-term outcome of patients with single ventricles improved over time, but remains poor compared to other congenital heart lesions with biventricular circulation. Main cause for this unfavourable outcome is the unphysiological hemodynamic of the Fontan circulation, such as subnormal systemic cardiac output and increased systemic-venous pressure. To overcome this limitation, we are developing the concept of a contractile extracardiac Fontan-tunnel. In this study, we evaluated the survival and structural development of a tissue-engineered conduit under in vivo conditions. Engineered heart tissue was generated from ventricular heart cells of neonatal Wistar rats, fibrinogen and thrombin. Engineered heart tissues started beating around day 8 in vitro and remained contractile in vivo throughout the experiment. After culture for 14 days constructs were implanted around the right superior vena cava of Wistar rats (n = 12). Animals were euthanized after 7, 14, 28 and 56 days postoperatively. Hematoxylin and eosin staining showed cardiomyocytes arranged in thick bundles within the engineered heart tissue-conduit. Immunostaining of sarcomeric actin, alpha-actin and connexin 43 revealed a well -developed cardiac myocyte structure. Magnetic resonance imaging (d14, n = 3) revealed no constriction or stenosis of the superior vena cava by the constructs. Engineered heart tissues survive and contract for extended periods after implantation around the superior vena cava of rats. Generation of larger constructs is warranted to evaluate functional benefits of a contractile Fontan-conduit.

OBJECTIVES: Conventional aortic valve replacement (AVR) in young, active patients represents a suboptimal solution in terms of long-term survival, durability and quality of life. The aim of the present work is to present an update on the multicentre experience with the pulmonary autograft procedure in young, adult patients.METHODS: Between 1990-2013, 1779 adult patients (1339 males; 44.7 +/- 11.6 years) underwent the pulmonary autograft procedure in 8 centres. All patients underwent prospective clinical and echocardiographic examinations annually. The mean follow-up was 8.3 +/- 5.1 years (range 0-24.3 years) with a total cumulative follow-up of 14 288 years and 662 patients having a follow-up of at least 10 years.RESULTS: The early (30-day) mortality rate was 1.1% (n = 19). Late (>30 day) survival of the adult population was comparable with the age- and gender-matched general population (observed deaths: 101, expected deaths: 91; P = 0.29). Freedom from autograft reoperation at 5, 10 and 15 years was 96.8, 94.7 and 86.7%, respectively, whereas freedom from homograft reoperation was 97.6, 95.5 and 92.3%, respectively. The overall freedom from reoperation was 94.9, 91.1 and 82.7%, respectively. Longitudinal modelling of functional valve performance revealed a low (<5%) probability of a patient being in higher autograft regurgitation grades throughout the first decade. Similarly, excellent homograft function was observed throughout the first 15 years.CONCLUSION: The autograft principle results in postoperative long-term survival comparable with that of the age-and gender-matched general population and reoperation rates within the 1%/patient-year boundaries and should be considered in young, active patients who want to avoid the shortcomings of conventional prostheses.

BACKGROUND AND AIM OF THE STUDY An increasing number of young adult patients are choosing bioprostheses for aortic valve replacement (AVR). In this context, the Ross operation deserves renewed consideration as an alternative biological substitute. After both the Ross procedure and bioprosthetic AVR, reoperation rates remain a concern and may be related to age at surgery. Herein are reported details of freedom from reoperation after the Ross procedure for different age groups. METHODS The reoperation rates of 1,925 patients (1,444 males, 481 females; mean age 41.2 ± 15.3 years) from the German Ross registry with a mean follow up of 7.4 ± 4.7 years (range: 0.00-18.51 years; total 12,866.6 patient-years) were allocated to three age groups: group I < 40 years; group II 40-60 years; and group III > 60 years. RESULTS At 10 years (respectively 15 years) of follow up, freedom from reoperation was 86% (76%) in group I, 93% (85%) in group II, and 89% (83%) in group III. CONCLUSION There is some evidence that, at least during the first 10 and 15 years after AVR, the Ross procedure provides a significantly lower reoperation rate in young adult and middle-aged patients aged < 60 years. This information may be of interest to the patients' or physicians' decision-making for aortic valve surgery.

Introduction: Fontan palliation is the treatment of choice for patients with morphological or functional univentricular hearts. The unphysiologic and non-pulsatile pulmonary blood flow results in multiorgan complications and a poor long-term outcome. We evaluated graft survival and histomorphology of a pulsatile Fontan conduit generated from Engineered Heart Tissue (EHT) after implantation in a rat model. Hypothesis: We hypothesized that EHT matures and remains contractile in the setting of venous (Fontan-like) preload. Methods: EHT was generated from ventricular cardiomyocytes of neonatal Wistar rats, fibrinogen, thrombin and DMEM. After culture for 14 days constructs were implanted around the right superior vena cava of Wistar rats (n=12, 300-350 g). Immunosupression was achieved by daily subcutaneous injection of Cyclosporin A (25 mg/kg BW) and Methylprednisolone (2 mg/kg BW). MRI (Bruker) was used to assess condensation of EHTs in vivo. Animals were euthanized after 7, 14, 28 and 56 days postoperatively for histomorphological analysis. Transmission electron microscopy was used to evaluate sarcomeric integrity of cardiomyocytes within the construct. Results: In culture, EHTs started beating around day 8 and remained contractile in vivo throughout the experiment (d7=3/3, d14=2/3, d28=3/3, d56=2/3). All animals survived circumferential implantation of EHTs around the right SVC via a right thoracotomy. MRI (d14, n=3) revealed no constriction or stenosis of the SVC by the constructs. Hematoxylin and Eosin staining showed densely packed bundles of cardiomyocytes within the EHT conduit and intense vascularisation. Immunolabeling of actinin and connexin 43 indicated adequate maturation of cardiomyocytes after grafting around the right SVC in rats. Conclusions: EHTs placed around the superior caval vein of Wistar rats survive and contract for a considerable time after implantation. Histomorphology revealed a matured phenotype of grafted cardiomyocytes and an adequate vascularisation. The functional benefit of a contractile neo-ventricle to propel pulmonary blood flow in Fontan patients remains to be evaluated.

Introduction: Fontan palliation is the treatment of choice for patients with morphological or functional univentricular hearts. Despite improved outcomes over the past decades the unphysiologic hemodynamics of the Fontan circulation, i.e., high central vein pressure and non-pulsatile pulmonary blood flow results in early and late multiorgan complications. Thus, as a first step, we evaluated the feasibility to create pulsatile flow in a cavopulmonary anastomosis utilizing Engineered Heart Tissue (EHT) in a rat model.

BACKGROUND:Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability. METHODS:A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013. RESULTS:Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts. CONCLUSIONS:Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding.

BACKGROUND:The advancement of noninvasive imaging methods has resulted in the increase in diagnosis of heart neoplasms. However, the literature has few studies involving primary cardiac neoplasms in children.OBJECTIVE:To retrospectively review cases of primary heart neoplasms in children, considering the initial clinical manifestations, diagnostic tests used, surgical indication, histopathological types and immediate postoperative course.METHODS:The retrospective study was based on the assessment of medical records from 1983 to 2011. Only cases that were referred for surgical treatment during that period were included. Age at admission, prenatal diagnosis, family history, initial symptoms and the results of performed tests were assessed. Moreover, the date and indication of the surgery, intraoperative findings, the result of the histopathological analysis, as well as the immediate postoperative complications were recorded.RESULTS:Of the 18 patients studied, the most frequent clinical manifestations were dyspnea and heart murmur (7 and 6 patients, respectively); the most often used adjunct diagnostic method was echocardiography (18 patients); cavitary obstruction or ventricular inflow or outflow tract obstruction was the main indication for surgery (12 cases); the most common histological profile was rhabdomyoma (7 patients) and most patients showed good clinical outcome.CONCLUSIONS:In this study, imaging diagnosis was basically attained through echocardiography, with good correlation with intraoperative findings. Histopathological findings were consistent with the literature, with rhabdomyoma being the most common neoplasm in children. The evolution after surgical treatment was favorable in most cases.

FUNDAMENTO: Avanço dos métodos não invasivos de imagem proporcionou o aumento no número de diagnóstico de tumores cardíacos. Apesar disso, a literatura apresenta poucos trabalhos envolvendo tumores cardíacos primários em crianças. OBJETIVO: Avaliar retrospectivamente casos de tumores cardíacos primários em crianças, considerando manifestações clínicas iniciais, exames utilizados para o diagnóstico, indicação cirúrgica, tipos histopatológicos encontrados e evolução pós-operatória imediata. MÉTODOS: O estudo foi retrospectivo, baseado na avaliação de prontuários no período de 1983 a 2011. Incluímos somente casos que foram orientados para tratamento cirúrgico no período. Avaliaram-se a idade na admissão, o diagnóstico pré-natal, a história familial, os sintomas iniciais e os resultados de exames realizados. Foram coletados, ainda, a data e indicação de cirurgia, os achados intraoperatórios, o resultado do exame histopatológico, assim como as complicações imediatas no pós-operatório. RESULTADOS: Dos 18 pacientes estudados, as manifestações clínicas mais encontradas foram dispneia e sopro cardíaco (7 e 6 pacientes, respectivamente); o método de complemento diagnóstico mais usado foi o ecocardiograma (18 pacientes); a obstrução cavitária ou do trato de entrada ou saída ventricular foi a principal indicação de cirurgia (12 casos); o perfil histológico mais encontrado foi rabdomioma (7 pacientes); a maioria dos pacientes apresentou boa evolução clínica. CONCLUSÃO: Neste estudo o diagnóstico por imagem foi basicamente ecocardiográfico, com boa correlação com os achados intraoperatórios. Os achados histopatológicos foram concordantes com a literatura, com o rabdomioma apresentando-se como o tumor mais comum em crianças. A evolução após tratamento cirúrgico mostrou-se favorável na maior parte dos casos.

BACKGROUND:The increasing number of children with evolving congenital heart diseases demands greater preparation of professionals and institutions that handle them.OBJECTIVE:To describe the profile of patients aged over 16 years with congenital heart disease, who have undergone surgery, and analyze the risk factors that predict hospital mortality.METHOD:One thousand five hundred twenty patients (mean age 27 ± 13 years) were operated between January 1986 and December 2010. We performed a descriptive analysis of the epidemiological profile of the study population and analyzed risk factors for hospital mortality, considering the complexity score, the year in which surgery was performed, the procedure performed or not performed by the pediatric surgeon and reoperation.RESULTS:There was a significant increase in the number of cases from the year 2000. The average complexity score was 5.4 and the septal defects represented 45% of cases. Overall mortality was 7.7% and most procedures (973 or 61.9%) with greater complexity were performed by pediatric surgeons. Complexity (OR 1.5), reoperation (OR 2.17) and pediatric surgeon (OR 0.28) were independent risk factors influencing mortality. Multivariate analysis showed that the year in which the surgery was performed (OR 1.03), the complexity (OR 1.44) and the pediatric surgeon (OR 0.28) influenced the result.CONCLUSION:There is an increasing number of patients aged 16 years which, despite the large number of simple cases, the most complex ones were referred to pediatric surgeons, who had lower mortality, especially in recent years.

FUNDAMENTO: O número crescente de crianças com cardiopatias congênitas em evolução demanda maior preparo dos profissionais e das instituições que as manuseiam. OBJETIVO: Descrever o perfil dos pacientes com idade superior a 16 anos com cardiopatia congênita operados e analisar os fatores de risco preditivos de mortalidade hospitalar. MÉTODOS: Mil, quinhentos e vinte pacientes (idade média 27 ± 13 anos) foram operados entre janeiro de 1986 e dezembro de 2010. Foram realizadas análise descritiva do perfil epidemiológico da população estudada e análise dos fatores de risco para mortalidade hospitalar, considerando escore de complexidade, ano em que a cirurgia foi realizada, procedimento realizado pelo cirurgião pediátrico ou não e presença de reoperação. RESULTADOS: Ocorreu um crescimento expressivo no número de casos a partir do ano 2000. A média do escore de complexidade foi 5,4 e os defeitos septais corresponderam a 45% dos casos. A mortalidade geral foi 7,7% e o maior número de procedimentos (973 ou 61,9%) com maior complexidade foi realizado por cirurgiões pediátricos. Complexidade (OR 1,5), reoperação (OR 2,17) e cirurgião pediátrico (OR 0,28) foram fatores de risco independentes que influenciaram a mortalidade. A análise multivariada mostrou que o ano em que a cirurgia foi realizada (OR 1,03), a complexidade (OR 1,44) e o cirurgião pediátrico (OR 0,28) influenciaram no resultado. CONCLUSÃO: Observa-se um número crescente de pacientes com idade superior a 16 anos e que, apesar do grande número de casos simples, os mais complexos foram encaminhados para os cirurgiões pediátricos, que apresentaram menor mortalidade, em especial nos anos mais recentes. (Arq Bras Cardiol. 2012; [online].ahead print, PP.0-0)

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 Coracao da Faculdade de Medicina da Universidade de Sao 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.

BACKGROUND:Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction. METHODS:Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period. RESULTS:Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31). CONCLUSION:Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-term follow-up.