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OBJECTIVES: Surgical management of mitral valve disease is challenging in infants <1 year old. We aimed at reviewing the French experience with Melody mitral valve replacement in critically ill infants. METHODS: A retrospective cohort study reporting the French experience with Melody mitral valve replacement. RESULTS: Seven symptomatic infants [complete atrioventricular septal defect (n = 4, Down syndrome: n = 3), hammock valve (n = 3)] underwent Melody mitral valve replacement [age: 3 months (28 days to 8 months), weight: 4.3 kg (3.2-6.4 kg)] because of severe mitral valve regurgitation (6) or mixed valve disease (1) and 14 mm (11-16 mm) mitral valve annulus. In 2 patients whose valve was felt irreparable, Melody mitral valve replacement was performed straightaway. The others underwent 2 (1-3) previous attempts of valve repair; 3 were on extracorporeal membrane oxygenation. Melody mitral valve replacement led to competent valve and low gradient [3 mmHg, (1-4 mmHg)]. One patient died 3 days post-implant from extracorporeal membrane oxygenation-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, 1 underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical mitral valve replacement at 2 years; another is currently planned for transcatheter Melody valve dilation. CONCLUSIONS: Melody mitral valve replacement may be considered in selected infants with small mitral valve annulus as an alternative to mechanical mitral valve replacement. Our experience highlights a high-risk of late infective endocarditis that deserves further consideration.
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First described in 1760 by the anatomist Morgagni, coarctation of the aorta (CoA) is a congenital heart defect characterized by narrowing the aorta, typically distal to the left subclavian artery. It accounts for approximately 5-8% of all congenital heart diseases, with an incidence estimated at 4 per 10,000 live births. In 1944, the Swedish surgeon Clarence Crafoord achieved the first successful surgical CoA repair by performing an aortic end-to-end anastomosis on two patients aged 12 and 27 years old. Presently, the most prevalent techniques for surgical repair, particularly in infants and neonates with isolated coarctation, involve resection with end-to-end anastomosis (EEA) and the modified Crafoord technique (extended resection with end-to-end anastomosis (EEEA)). Subclavian flap aortoplasty (SCAP) is an alternative surgical option for CoA repair in patients under two years of age. In cases where the stenosis extends beyond resection and end-to-end anastomosis feasibility, patch aortoplasty (PP) employing a prosthetic patch can augment the stenotic region, especially for older patients. Despite advances in pediatric cardiology and cardiac surgery, recoarctation remains a significant concern after surgical or interventional repair. This comprehensive review aims to provide a thorough analysis of coarctation management, covering the pioneering techniques introduced by Crafoord using end-to-end anastomosis and now extending to the contemporary era marked by percutaneous interventions as well as the recoarctation rate associated with each type.
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Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.
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Comunicação Interatrial , Veias Pulmonares , Humanos , Veia Cava Superior/diagnóstico por imagem , Resultado do Tratamento , Comunicação Interatrial/terapia , Comunicação Interatrial/cirurgia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Veias Pulmonares/anormalidadesRESUMO
Percutaneous interventions have completely refashioned the management of children with congenital heart diseases (CHD) and the use of non-invasive imaging has become the gold standard to plan and guide these procedures in the modern era. We are now facing a dual challenge to improve the standard of care in low-risk patients, and to shift our strategies from the classic open chest surgery to imaging-guided percutaneous interventions in high-risk patients. Such rapid evolution of ultrasound technologies over the last 20 years have permitted the integration of transthoracic, transesophageal and intracardiac echocardiography into the interventional workflow to improve image guidance and reduce radiation burden from fluoroscopy and angiography. Specifically, miniaturization of transesophageal probe and advances in three-dimensional (3D) imaging techniques have enabled real-time 3D image guidance during complex interventional procedure, In addition, multimodality and fusion imaging techniques harness the strengths of different modalities to enhance understanding of anatomical and spatial relationship between different structures, improving communication and coordination between interventionalists and imaging specialists. In this review, we aim to provide an overview of 3D imaging modalities and multimodal fusion in procedural planning and live guidance of percutaneous interventions. At the present times, 3D imaging can no longer be considered a luxury but a routine clinical tool to improve procedural success and patient outcomes.
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The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (20182020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.
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Insuficiência Cardíaca , Comunicação Interventricular , Dispositivo para Oclusão Septal , Cateterismo Cardíaco , Criança , Pré-Escolar , Comunicação Interventricular/epidemiologia , Comunicação Interventricular/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Estudos Observacionais como Assunto , Estudos Prospectivos , Resultado do TratamentoRESUMO
AIMS: To investigate the healing process and nickel release of the Hyperion occluder (Comed BV, Netherlands), as compared to the Amplatzer septal occluder (ASO) (St. Jude Medical Inc., St. Paul, MN, USA) in a chronic swine model. BACKGROUND: Some long-term complications occurring after percutaneous atrial septal defect (ASD) closure may be partially associated with an inappropriate healing of the device and increased nickel release. There is no direct comparative study of different occluders for healing and nickel release. METHODS: After percutaneous ASD creation, 12 pigs were implanted with 15 mm Hyperion (n = 6) and 15 mm ASO (n = 6) devices. After 1 month (n = 3 for each device) and 3 months (n = 3 for each device) of follow-up, device explantation was performed and healing was assessed using histopathological workup. Systemic and tissular nickel release was performed. RESULTS: Implantation was successful in 100% without complications. Device coverage was observed as early as 1 month after implantation and was almost complete after 3 months. A granulation tissue with a predominantly mononuclear inflammatory reaction was observed in contact with nitinol wires while an inflammatory reaction was seen in contact with textile fibers. We found no statistically significant difference between the 2 devices whether for histological grading scores or systemic nickel release, regardless to follow-up duration. CONCLUSIONS: In this preclinical study, we demonstrated that Amplatzer septal occluder and Hyperion occluder were not significantly different for device healing and nickel release processes.
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Ligas/farmacologia , Comunicação Interatrial/cirurgia , Efeitos Adversos de Longa Duração/induzido quimicamente , Teste de Materiais/métodos , Complicações Pós-Operatórias/induzido quimicamente , Implantação de Prótese , Dispositivo para Oclusão Septal/efeitos adversos , Ligas/efeitos adversos , Animais , Pesquisa Comparativa da Efetividade , Efeitos Adversos de Longa Duração/prevenção & controle , Níquel/efeitos adversos , Níquel/farmacologia , Avaliação de Resultados em Cuidados de Saúde , Complicações Pós-Operatórias/prevenção & controle , Desenho de Prótese , Implantação de Prótese/efeitos adversos , Implantação de Prótese/instrumentação , Suínos , Oligoelementos/efeitos adversos , Oligoelementos/farmacologia , Resultado do TratamentoRESUMO
Stenting the arterial duct emerged in the early 1990s as an alternative to a variety of surgical interventions in neonates with a duct-dependent pulmonary or systemic circulation complex defect. Furthermore, palliative ductal stenting has been applied in older children with severe suprasystemic pulmonary arterial hypertension, as an alternative to surgical shunts, such as Potts anastomosis. Early results of this technique were discouraging, but by learning from the failures of the past, ductal stenting has become a reliable palliative therapy. In this review, we aim to describe the historical evolution of ductal stenting, its different clinical applications and outcomes, and future perspectives for this strategy in congenital cardiac catheterization.
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Cateterismo Cardíaco/instrumentação , Canal Arterial , Cardiopatias Congênitas/terapia , Cuidados Paliativos , Stents , Cateterismo Cardíaco/efeitos adversos , Pré-Escolar , Tomada de Decisão Clínica , Canal Arterial/anormalidades , Canal Arterial/diagnóstico por imagem , Canal Arterial/fisiopatologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Seleção de Pacientes , Desenho de Prótese , Fatores de Risco , Resultado do TratamentoRESUMO
The healing process, occurring after intra-cardiac and intra-vascular device implantation, starts with fibrin condensation and attraction of inflammatory cells, followed by the formation of fibrous tissue that slowly covers the device. The duration of this process is variable and may be incomplete, which can lead to thrombus formation, dislodgement of the device or stenosis. To better understand this process and the neotissue formation, animal models were developed: small (rats and rabbits) and large (sheep, pigs, dogs and baboons) animal models for intra-vascular device implantation; sheep and pigs for intra-cardiac device implantation. After intra-vascular and intra-cardiac device implantation in these animal models, in vitro techniques, i.e. histology, which is the gold standard and scanning electron microscopy, were used to assess the device coverage, characterize the cell constitution and detect complications such as thrombosis. In humans, optical coherence tomography and intra-vascular ultrasounds are both invasive modalities used after stent implantation to assess the structure of the vessels, atheroma plaque and complications. Non-invasive techniques (computed tomography and magnetic resonance imaging) are in development in humans and animal models for tissue characterization (fibrosis), device remodeling evaluation and device implantation complications (thrombosis and stenosis). This review aims to (1) present the experimental models used to study this process on cardiac devices; (2) focus on the in vitro techniques and invasive modalities used currently in humans for intra-vascular and intra-cardiac devices and (3) assess the future developments of non-invasive techniques in animal models and humans for intra-cardiac devices.
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Vasos Sanguíneos/patologia , Desfibriladores Implantáveis , Procedimentos Endovasculares/instrumentação , Miocárdio/patologia , Marca-Passo Artificial , Intervenção Coronária Percutânea/instrumentação , Implantação de Prótese/instrumentação , Stents , Cicatrização , Animais , Biópsia , Vasos Sanguíneos/diagnóstico por imagem , Procedimentos Endovasculares/efeitos adversos , Fibrose , Humanos , Modelos Animais , Intervenção Coronária Percutânea/efeitos adversos , Implantação de Prótese/efeitos adversos , Fatores de Risco , Fatores de TempoRESUMO
We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.
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Implante de Prótese de Valva Cardíaca , Estenose da Valva Mitral , Valva Mitral , Modelagem Computacional Específica para o Paciente , Impressão Tridimensional , Obstrução do Fluxo Ventricular Externo/prevenção & controle , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Lactente , Valva Mitral/anormalidades , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Estenose da Valva Mitral/congênito , Estenose da Valva Mitral/fisiopatologia , Estenose da Valva Mitral/cirurgia , Modelos Anatômicos , Cuidados Pré-Operatórios/métodos , Desenho de Prótese/métodos , Reoperação/métodos , Risco Ajustado/métodos , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/etiologiaRESUMO
BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a routine treatment for dysfunctional right ventricular outflow tract. Infective endocarditis (IE) is a major concern. AIM: To report French experience with the Melody™ valve (Medtronic Inc., Minneapolis, MN, USA). METHODS: All patients who underwent PPVI were recorded in a multicentre French national survey. Demographic and procedural data were collected from patients with IE. Bacterial identification, diagnostic tools and outcome were recorded. RESULTS: Forty-five cases of IE were diagnosed in 43 patients. The cumulative IE incidence was 11.8% (95% confidence interval [CI] 8.5-15.9). The annualized IE incidence was 3.6% (95% CI 0-4.8). Freedom from IE was 96.3% and 85.8% at 12 months and 60 months, respectively. IE incidence did not change during the study period. The mean interval between PPVI and IE was 2.6±2.1 years (range, 5 days to 7.3 years). Fifteen patients with IE required intravenous antibiotics only. Seven patients had early interventional cardiac catheterization to relieve severe right ventricular outflow tract obstruction. Twenty-four patients had surgical valve replacement (six urgently; nine semi-urgently; nine electively). Staphylococcus aureus IE required surgery in all but one patient. Three patients died before any treatment. Three additional patients died, giving a mortality rate of 14%. Global survival in the total cohort of patients who received a Melody valve was excellent (96.5% at 5 years). When comparing survival curves between the IE and non-IE groups, death and cardiovascular events were statistically significantly higher in the IE group (log-rank P<0.0001). CONCLUSION: Melody valve IE is a severe complication following PPVI. The annualized IE incidence in this cohort was similar to rates reported in other studies. With rapid diagnosis and adequate treatment, outcome has improved, and unfavourable outcome is mainly associated with S. aureus.
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Cateterismo Cardíaco/efeitos adversos , Endocardite Bacteriana/epidemiologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Próteses Valvulares Cardíacas/efeitos adversos , Infecções Relacionadas à Prótese/epidemiologia , Valva Pulmonar/cirurgia , Infecções Estafilocócicas/epidemiologia , Obstrução do Fluxo Ventricular Externo/cirurgia , Administração Intravenosa , Adolescente , Adulto , Antibacterianos/administração & dosagem , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/métodos , Cateterismo Cardíaco/mortalidade , Remoção de Dispositivo , Endocardite Bacteriana/microbiologia , Endocardite Bacteriana/mortalidade , Endocardite Bacteriana/terapia , Feminino , França/epidemiologia , Pesquisas sobre Atenção à Saúde , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/métodos , Implante de Prótese de Valva Cardíaca/mortalidade , Humanos , Incidência , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Desenho de Prótese , Infecções Relacionadas à Prótese/microbiologia , Infecções Relacionadas à Prótese/mortalidade , Infecções Relacionadas à Prótese/terapia , Fatores de Risco , Infecções Estafilocócicas/microbiologia , Infecções Estafilocócicas/mortalidade , Infecções Estafilocócicas/terapia , Fatores de Tempo , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/mortalidade , Adulto JovemRESUMO
BACKGROUND: Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated. OBJECTIVE: We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF. METHODS: We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014). RESULTS: Mean age at initial complete correction and at PVR was 20.1 ± 17.2 months and 26.3 ± 9.5 years, respectively. Forty-one patients had significant preoperative TR: 24 were moderate (group M) and 17 were severe (group S). A total of 16 TV repair were performed (8 for each group). Moderate and severe tricuspid regurgitation observed in the first year following the initial complete repair were significantly associated with severe TR at PVR (P < .001). In group M patients, TR was improved regardless of TV repair, whereas, in group S, residual significant TR was reported in 7 patients who did not have TV repair. No cases were observed for patients who underwent concomitant TV repair (P = .002). Among these patients with residual significant TR, 2 needed a tricuspid valve replacement. The functional status (New York Heart Association classification) of group S patients was significantly improved by concomitant TV repair. CONCLUSIONS: In adults with repaired TOF, TV repair is a safe procedure when performed at the time of PVR. If, at mid-term follow-up, there is probably no benefit of TV repair when preoperative TR is moderate, TV repair may improve both tricuspid valve function and functional status of the patients in case of severe preoperative TR.
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Complicações Pós-Operatórias/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: We report our experience of an unexpected complication of internalization of a pulmonary artery (PA) band in the vascular lumen, which occurred in a chronic porcine model of repaired tetralogy of Fallot (TOF). METHODS: Twelve piglets were divided into 3 groups: (1) TOF model animals (PA band plus pulmonary valvotomy, n = 4), (2) pulmonary insufficiency (PI) animals (pulmonary valvotomy, n = 4), and (3) control animals (n = 4). A nonabsorbable, coated braided polyester tape was used to perform the main pulmonary artery banding. Echocardiography was performed 4 months postoperatively. After each animal was euthanized, PA histological analysis was performed in animals with band internalization. RESULTS: Significant postsurgical pulmonary regurgitation and right ventricular enlargement were present in the TOF and PI, compared with control animals, whereas no significant pulmonary stenosis was observed in TOF animals when compared with PI group. Postmortem examination of all TOF animals revealed the constricting band to be intact but partially internalized into the PA lumen, allowing blood flow around the stenosis. Histological sections of the banded PA in the area of internalization showed a significant disorganization of the medial layer, with significant scarring and fibrotic reaction surrounding the outside of the band and the presence of inflammatory cells suggesting a significant inflammatory response during band internalization. CONCLUSIONS: Band internalization may occur after PA banding using a nonabsorbable, coated braided polyester tape in a chronic porcine model of repaired TOF. This unusual complication was likely due to the type of material used for banding.
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Complicações Pós-Operatórias/etiologia , Artéria Pulmonar/cirurgia , Suturas/efeitos adversos , Tetralogia de Fallot/cirurgia , Função Ventricular Direita/fisiologia , Animais , Animais Recém-Nascidos , Modelos Animais de Doenças , Ecocardiografia , Falha de Equipamento , Ligadura/efeitos adversos , Ligadura/instrumentação , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/fisiopatologia , Suínos , Tetralogia de Fallot/fisiopatologiaRESUMO
As preoperative morbi-mortality remains significant, care of newborns with transposition of the great arteries is still challenging. In this review of the literature, we discuss the different treatments that could improve the patient's condition into the preoperative period. Instead of a standardized management, we advocate personalized care of these neonates. Considering the deleterious effects of hypoxia, special attention is given to the use of non-invasive technologies to assess oxygenation of the tissues. As a prolonged preoperative time with low cerebral oxygenation is associated with cerebral injuries, distinguishing neonates who should undergo early surgery from those who could wait longer is crucial and requires full expertise in the management of neonatal congenital heart disease. Finally, to treat these newborns as soon as possible, we support a planned delivery policy for foetuses with transposition of the great arteries.
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Gerenciamento Clínico , Cuidados Pré-Operatórios/métodos , Transposição dos Grandes Vasos/cirurgia , HumanosRESUMO
Liver stiffness (LS) is associated with the presence of fibrosis; however, hepatic congestion due to elevated central venous pressure has also been shown to correlate with LS values. We report here the case of a 35-year-old woman with operated pulmonary stenosis and chronic pericarditis in whom longitudinal follow-up of LS was correlated with the changes in her hemodynamic conditions before and after surgical or percutaneous treatment of residual lesions. This case highlights the potential interest of LS as a reliable marker of hepatic congestion and elevated right ventricle (RV) filling pressures.
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Valvuloplastia com Balão/métodos , Fígado/fisiopatologia , Pericardite/complicações , Estenose da Valva Pulmonar/complicações , Adulto , Cateterismo Cardíaco , Técnicas de Imagem por Elasticidade , Feminino , Seguimentos , Hemodinâmica , Humanos , Fígado/diagnóstico por imagem , Pericardite/fisiopatologia , Pericardite/cirurgia , Período Pós-Operatório , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/cirurgiaRESUMO
BACKGROUND: Left ventricular (LV) dysfunction may complicate paediatric cardiac surgery with cardiopulmonary bypass, notably after long aortic cross-clamping (ACC). Assessment of occult myocardial injury by conventional echocardiographic variables may be difficult in the postoperative period. AIMS: To evaluate the feasibility of two-dimensional (2D) strain in the postoperative period, and to assess the effect of ACC duration on this variable. METHODS: Thirty-three paediatric patients (age<18years) with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass were included in this prospective single-centre study. Daily echocardiography was performed from the day before surgery to the fifth postoperative day. LV ejection fraction and LV 2D strain were measured. The cohort was divided into three groups according to ACC duration (group 1:<30minutes; group 2: 30-80minutes; group 3:>80minutes). RESULTS: Mean age and weight were 4.2±2.5years and 15.1±5.2kg, respectively. Feasibilities of longitudinal, circumferential and radial strains were good, and quite similar to conventional variables. Compared with conventional variables, intra- and interobserver agreements regarding 2D strain were better (r=0.916, P<0.001 and r=0.855, P<0.001 for longitudinal strain versus r=0.156, P=0.54 and r=0.064, P=0.80 for LV ejection fraction by Simpson's method). Postoperative evolution of longitudinal and circumferential strains was significantly different between the three groups (P<0.001), whereas there was no difference using conventional variables. CONCLUSION: Postoperative LV 2D strain is a feasible and reproducible method. Strain measurements seem to indicate correlation with ACC duration.
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Procedimentos Cirúrgicos Cardíacos , Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Função Ventricular Esquerda/fisiologia , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Seguimentos , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Prognóstico , Estudos ProspectivosRESUMO
The management of double-outlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Between 1993 and 2011, 36 consecutive patients presenting with double-outlet right ventricle or noncommitted ventricular septal defect (21 inlet, 10 muscular, and 5 central perimembranous) and 2 adequately sized ventricles underwent surgical repair at 2 centers. Right ventricular outflow tract obstruction was present in 18 of 36 patients (50%). A total of 21 patients had undergone previous palliative procedures. Anatomical repair (group I) by means of intraventricular baffle construction was performed in 24 (associated right ventricular outflow tract reconstruction in 12 and arterial switch in 5) at a median age of 10.5 months. Ventricular septal defect was surgically enlarged in 12 (50%) patients. The remaining 12 patients underwent univentricular repair (group II). There were 4 hospital deaths (11%), all in group I (P = 0.30 vs group II). A total of 8 of 20 patients in group I survivors underwent 13 reoperations after a median delay of 24 months: subaortic stenosis was the main cause for reoperation in 6 of 8 patients. There was 1 late death in group I and 2 late deaths in group II. The median follow-up was 5.6 years (95% CI: 0.2-9.8). The 10- year actuarial survival rate and freedom from reoperation were 74.7 ± 5% and 58 ± 5% in group I and 71 ± 7% and 70 ± 7% in group II, respectively. At the last visit, all survivors were in New York Heart Association class I-II. Univariate analysis showed that atrioventricular septal defect and isolated mitral cleft were associated with death (P = 0.04) and need for reoperation (P = 0.038). In conclusion, anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution. Associated atrioventricular septal defect and isolated mitral cleft were the only risk factors for mortality and reoperation.
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Anormalidades Múltiplas , Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Comunicação Interventricular/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Pré-Escolar , Intervalo Livre de Doença , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/mortalidade , Ecocardiografia Doppler em Cores , Feminino , Defeitos dos Septos Cardíacos/mortalidade , Defeitos dos Septos Cardíacos/cirurgia , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Valva Mitral/anormalidades , Valva Mitral/cirurgia , Paris , Reoperação , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVES: Surgical management of various forms of double-outlet right ventricle uses a variety of approaches depending on the underlying anatomic form. In this study, we sought to determine the risk factors of mortality and reoperation in those with double-outlet right ventricle undergoing biventricular repair, according to anatomic characteristics and initial surgical strategy. METHODS: Between 1992 and 2013, 433 patients were included in the study. Double-outlet right ventricle was classified as double-outlet right ventricle with subaortic ventricular septal defect associated with subpulmonary obstruction in 33% of patients (n = 141), with subaortic ventricular septal defect without subpulmonary obstruction in 30% of patients (n = 130), with subpulmonary ventricular septal defect in 32% of patients (n = 139), and with noncommitted ventricular septal defect in 5% of patients (n = 23). Three types of repairs were performed: (1) intraventricular baffle repair, n = 149 (34%); (2) intraventricular baffle repair with right ventricular outflow tract reconstruction, n = 163 (38%); and (3) intraventricular baffle repair with arterial switch operation, n = 121 (28%). RESULTS: Thirty-day overall mortality was 7.4%. Early reoperation was needed in 6% of the cases. Early mortality was higher in the intraventricular baffle repair with arterial switch operation group (P = .01). Survival at 10 years was 86.2%, and freedom from reoperation at 10 years was 61.4%. At last follow-up (median, 5.7 years; 95% confidence interval, 4.5-6.6), mortality and reoperation rates were similar in the different surgical strategy groups. Late reoperation and late mortality were significantly higher in the double-outlet right ventricle with noncommitted ventricular septal defect group (P < .01). In multivariate analyses, risk factors for reoperation were concomitant surgical procedures (P = .03) and duration of cardiopulmonary bypass (P < .01). Risk factors for mortality were restrictive ventricular septal defect (P = .01), mitral cleft (P < .01), and associated coronary artery anomalies (P = .01). CONCLUSIONS: Those with the anatomic type of double-outlet right ventricle with noncommitted ventricular septal defect were at higher risk for reoperation and mortality. Intraventricular baffle repair with arterial switch operation was the surgical strategy in patients at higher risk of early death. Initial surgical strategy did not influence the late outcomes.