RESUMO
A transseptal coronary artery course, also known as a transconal course, is an anomalous course of the left main coronary artery (LMCA) or the left anterior descending artery (LAD) through the conal septal myocardium. The conal septal myocardium is the posterior wall of the right ventricular outflow tract (RVOT), acting as a dividing myocardial wall between the subaortic and subpulmonary outflow tracts. The initial segment of a transseptal coronary artery has an extraconal course between the aorta and the RVOT cranial to the true intramyocardial segment. The transseptal coronary artery then emerges out of the conal septal myocardium at the epicardial surface on the lateral aspect of the RVOT. Many consider the transseptal coronary artery to be a benign entity. However, there are few case reports of severe cardiac symptoms such as myocardial ischemia, arrhythmia, and even sudden cardiac deaths due to potential coronary artery compression in the systolic phase.â In this article, we seek to describe the imaging findings of transseptal coronary artery course on coronary computed tomography angiography (CTA), discuss their clinical analysis, and briefly discuss the management of these lesions.
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Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários , Humanos , Anomalias dos Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/cirurgia , Angiografia por Tomografia Computadorizada/métodos , Angiografia Coronária/métodos , Criança , Septos Cardíacos/diagnóstico por imagemRESUMO
OBJECTIVES: To describe the techniques used for percutaneous veno-arterial extracorporeal membrane oxygenation (VA-ECMO) cannulation and decannulation in children with the pediatric interventional cardiologist (PIC) as the primary operator, and present outcomes of this initial clinical experience. BACKGROUND: Percutaneous VA-ECMO during cardiopulmonary resuscitation (CPR) has been successfully performed in adults, but currently, not much data exists on children. METHODS: This is a single-center study including VA-ECMO cannulations performed by the PIC between 2019 and 2021. Efficacy was defined as the successful initiation of VA-ECMO without surgical cutdown. Safety was defined as the absence of additional procedures related to cannulation. RESULTS: Twenty-three percutaneous VA-ECMO cannulations were performed by PIC on 20 children with 100% success. Fourteen (61%) were performed during ongoing CPR, and nine for cardiogenic shock. The Median age was 15 (0.15-18) years, and the median weight was 65 (3.3-180) kg. All arterial cannulations were via the femoral artery except in one, 8-week-old infant who was cannulated in the carotid artery. A distal perfusion cannula was placed in the ipsilateral limb in 17 (78%). The median time from initiating cannulation to ECMO flow was 35 (13-112) minutes. Two patients required arterial graft placement at the time of decannulation and one needed below-knee amputation. ECMO support was maintained for a median of 4 (0.3-38) days. Thirty-day survival was 74%. CONCLUSION: Percutaneous VA-ECMO cannulations can be effectively performed, even during CPR with the Pediatric Interventional Cardiologist being the primary operator. This is an initial clinical experience. Future outcome studies compared with standard surgical cannulations are necessary to advocate routine percutaneous VA-ECMO in children.
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Cateterismo Periférico , Oxigenação por Membrana Extracorpórea , Adulto , Humanos , Criança , Adolescente , Oxigenação por Membrana Extracorpórea/efeitos adversos , Cateterismo Periférico/efeitos adversos , Cateterismo Periférico/métodos , Resultado do Tratamento , Estudos Retrospectivos , Choque CardiogênicoRESUMO
Anomalous origin of the coronary arteries, though uncommon, is of great clinical concern. It can be the cause of sudden cardiac death and abnormal cardiac hemodynamics. Advances in electrocardiographically (ECG)-gated multi-detector CT have increased diagnostic accuracy in detecting anomalous origin of coronary arteries and their interarterial and intramural courses. Recent advances in multi-detector CT image processing software have allowed the creation of virtual endoluminal views of the aortic root and improved assessment of the intramural course (the length and relationship to the intercoronary commissure) of the coronary artery, which is of considerable surgical importance. We review our experience with virtual endoluminal imaging in our first 19 cases of interarterial coronary artery anomalies (17 cases of interarterial with intramural segment and 2 cases of purely interarterial course) diagnosed preoperatively and proven surgically.
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Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/cirurgia , Humanos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Patent ductus arteriosus (PDA) is common in extremely low birth weight (ELBW) infants. The objectives of this study were to describe our early clinical experience of transcatheter PDA closure (TCPC) in ELBW infants, compare outcomes with surgical ligation of PDA (SLP), and identify risk factors for prolonged respiratory support. METHODS: A retrospective review was performed comparing infants born <27 weeks, weighing <1 kg at birth and < 2 kg during TCPC with 2:1 propensity-score matched group of infants that underwent SLP. Change in respiratory severity scores (RSS) immediately post-procedure and the time taken for return to pre-procedure RSS for TCPC versus SLP was compared. Factors contributing to prolonged elevation of RSS were identified. RESULTS: Eighty ELBW infants (median procedure weight: 1060 [range 640-2000] grams) that underwent successful TCPC were compared with 40 infants that underwent SLP (procedure weight 650-1760 g). There was greater increase in RSS following SLP compared to TCPC (76% vs. 18%; P < 0.01). It took longer for RSS to return to pre-procedural scores post-SLP compared to post-TCPC (28 vs. 8.4 hr; P < 0.01). Elevated pulmonary artery pressure (PAP) and TCPC at >8 weeks of age were associated with prolonged (>30-days) elevation of RSS ≥ 1 (OR = 5.4, 95%CI: 2.2-9.4, P < 0.01 and OR = 2.86, 95%CI: 1.5-4.2, P = 0.05 respectively). Overall complication rate for TCPC was 3.7%. CONCLUSIONS: TCPC is feasible in infants as small as 640-2000 g and can be performed safely in the majority. TCPC may offer faster weaning of respiratory support compared to SLP when performed earlier in life, and before the onset of elevated PAP.
Assuntos
Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Permeabilidade do Canal Arterial/terapia , Recém-Nascido de Peso Extremamente Baixo ao Nascer , Peso ao Nascer , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/fisiopatologia , Feminino , Hemodinâmica , Humanos , Lactente , Recém-Nascido , Ligadura , Pulmão/fisiopatologia , Masculino , Respiração , Respiração Artificial , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Desmame do RespiradorRESUMO
BACKGROUND: A surgical pulmonary artery band (PAB) is used to control excessive pulmonary blood flow for certain congenital heart diseases. Previous attempts have been made to develop a transcatheter, implantable pulmonary flow restrictor (PFR) without great success. We modified a microvascular plug (MVP) to be used as a PFR. The objectives of this study were to demonstrate feasibility of transcatheter implantation and retrieval of the modified MVP as a PFR, and compare PA growth while using the PFR versus PAB. METHODS AND RESULTS: The PFR was implanted in eight newborn piglets in bilateral branch pulmonary arteries (PAs). Immediately post-PFR implantation, the right ventricular systolic pressure increased from a median of 20-51 mmHg. Transcatheter retrieval of PFR was 100% successful at 3, 6, and 9 weeks and 50% at 12-weeks post-implant. A left PAB was placed via thoracotomy in four other newborn piglets. Debanding was performed 6-weeks later via balloon angioplasty. On follow-up, the proximal left PA diameters in the PFR and the PAB groups were similar (median 8 vs. 7.1 mm; p = 0.11); albeit the surgical band sites required repeat balloon angioplasty secondary to recurrent stenosis. By histopathology, there was grade II vessel injury in two pigs immediately post-retrieval of PFR that healed by 12 weeks. CONCLUSIONS: Transcatheter implantation and retrieval of the MVP as a PFR is feasible. PA growth is comparable to surgical PAB, which is likely to require reinterventions. The use of the MVP as a PFR in humans has to be trialed before recommending its routine use.
Assuntos
Procedimentos Endovasculares/instrumentação , Artéria Pulmonar/cirurgia , Circulação Pulmonar , Dispositivos de Acesso Vascular , Procedimentos Cirúrgicos Vasculares , Angioplastia com Balão , Animais , Animais Recém-Nascidos , Velocidade do Fluxo Sanguíneo , Remoção de Dispositivo , Procedimentos Endovasculares/efeitos adversos , Estudos de Viabilidade , Ligadura , Modelos Animais , Artéria Pulmonar/crescimento & desenvolvimento , Recidiva , Fatores de Risco , Estenose de Artéria Pulmonar/etiologia , Estenose de Artéria Pulmonar/fisiopatologia , Estenose de Artéria Pulmonar/terapia , Sus scrofa , Fatores de Tempo , Procedimentos Cirúrgicos Vasculares/efeitos adversosRESUMO
OBJECTIVE: Shunt-related adverse events are frequent in infants after modified Blalock-Taussig despite use of acetylsalicylic acid prophylaxis. A higher incidence of acetylsalicylic acid-resistance and sub-therapeutic acetylsalicylic acid levels has been reported in infants. We evaluated whether using high-dose acetylsalicylic acid can decrease shunt-related adverse events in infants after modified Blalock-Taussig. METHODS: In this single-centre retrospective cohort study, we included infants ⩽1-year-old who underwent modified Blalock-Taussig placement and received acetylsalicylic acid in the ICU. We defined acetylsalicylic acid treatment groups as standard dose (⩽7 mg/kg/day) and high dose (⩾8 mg/kg/day) based on the initiating dose. RESULTS: There were 34 infants in each group. Both groups were similar in age, gender, cardiac defect type, ICU length of stay, and time interval to second stage or definitive repair. Shunt interventions (18 versus 32%, p=0.16), shunt thrombosis (14 versus 17%, p=0.74), and mortality (9 versus 12%, p=0.65) were not significantly different between groups. On multiple logistic regression analysis, single-ventricle morphology (odds ratio 5.2, 95% confidence interval of 1.2-23, p=0.03) and post-operative red blood cells transfusion ⩾24 hours [odds ratio 15, confidence interval of (3-71), p<0.01] were associated with shunt-related adverse events. High-dose acetylsalicylic acid treatment [odds ratio 2.6, confidence interval of (0.7-10), p=0.16] was not associated with decrease in these events. CONCLUSIONS: High-dose acetylsalicylic acid may not be sufficient in reducing shunt-related adverse events in infants after modified Blalock-Taussig. Post-operative red blood cells transfusion may be a modifiable risk factor for these events. A randomised trial is needed to determine appropriate acetylsalicylic acid dosing in infants with modified Blalock-Taussig.
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Aspirina/administração & dosagem , Procedimento de Blalock-Taussig/efeitos adversos , Cardiopatias Congênitas/cirurgia , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/prevenção & controle , Trombose/prevenção & controle , Administração Oral , Angiografia por Tomografia Computadorizada , Relação Dose-Resposta a Droga , Ecocardiografia Doppler , Feminino , Seguimentos , Humanos , Lactente , Masculino , Inibidores da Agregação Plaquetária/administração & dosagem , Complicações Pós-Operatórias/diagnóstico , Prognóstico , Estudos Retrospectivos , Trombose/diagnósticoRESUMO
OBJECTIVE: To evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation. METHODS: A retrospective review of all patients who survived to the second stage following Norwood-Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt. The size of pulmonary arteries was compared at pre-Glenn catheterisation. RESULTS: A total of 48 neonates with the diagnosis of hypoplastic left heart syndrome or its variants comprised the study population. Femoral vein homograft of 5-6 mm diameter was used in 14 and polytetrafluoroethylene graft of 5 mm was used in 34 patients. The two groups were comparable in terms of preoperative demographics and age at time of pre-Glenn catheterisation (3.9±0.7 versus 3.4±0.8 months, p=0.06). Patients who received femoral vein homograft demonstrated a significantly higher pre-Glenn Nakata index [264 (130-460) versus 165 (108-234) mm2/m2, p=0.004]. The individual branch pulmonary arteries were significantly larger in the femoral vein group (right, 7.8±3.6 versus 5.0±1.2, p=0.014; left, 7.2±2.1 versus 5.6±1.9, p=0.02). There were no differences in cardiac index, Qp:Qs, ventricular end-diastolic pressure or systemic oxygen saturations. CONCLUSIONS: Utilisation of a valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit during Norwood-Sano operation confers better interstage growth of the pulmonary arteries. Further studies are needed to evaluate the impact of femoral vein homograft on single ventricle function.
Assuntos
Prótese Vascular , Veia Femoral/transplante , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood , Artéria Pulmonar/cirurgia , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Recém-Nascido , Masculino , Politetrafluoretileno , Próteses e Implantes , Estudos Retrospectivos , Transplante Homólogo , Pressão VentricularRESUMO
Studies in adult HT have demonstrated improved cardiac function in the recipient following administration of T3 to the donor. The purpose of this experiment was to assess the effects of T3 on the function of the immature donor heart following HT in a piglet model. A total of 32 piglets were divided into 16 donors and 16 recipients. Following creation of brain death, half of the donor piglets were randomized to receive three doses of T3 (0.2 µg/kg) along with hydrocortisone (1 mg/kg). The donor hearts were then transplanted into the recipient piglets on CPB. Duration of survival off CPB, inotrope score, and EF of heart following CPB were evaluated. There were no differences between the two groups in age, weight, pre-brain death EF, T3 levels, and CPB times. Post-CPB survival times were inversely related to the ischemic times in both groups (Pearson r=-0.80, P<.001), and this relationship was not influenced by T3. There was no difference in inotrope score, EF, or biochemical assessment between the two groups. Administration of T3 in combination with hydrocortisone to the brain-dead donor confers no beneficial effect on myocardial function or survival following HT in a piglet model.
Assuntos
Cardiotônicos/farmacologia , Transplante de Coração , Coração/efeitos dos fármacos , Coleta de Tecidos e Órgãos/métodos , Tri-Iodotironina/farmacologia , Animais , Morte Encefálica , Cardiotônicos/administração & dosagem , Esquema de Medicação , Feminino , Coração/fisiologia , Transplante de Coração/mortalidade , Humanos , Hidrocortisona/administração & dosagem , Hidrocortisona/farmacologia , Masculino , Distribuição Aleatória , Suínos , Doadores de Tecidos , Tri-Iodotironina/administração & dosagemRESUMO
Left thoracic sympathectomy has been shown to be an effective treatment for adults with long QT syndrome who are refractory to medical therapy. We report the successful use of left thoracic sympathectomy for the management of a 10-week-old premature baby with long QT syndrome and heart failure from a large ventricular septal defect and patent ductus arteriosus.
Assuntos
Insuficiência Cardíaca/complicações , Doenças do Prematuro/terapia , Recém-Nascido Prematuro , Síndrome do QT Longo/terapia , Simpatectomia/métodos , Eletrocardiografia , Seguimentos , Humanos , Lactente , Síndrome do QT Longo/complicações , Vértebras TorácicasRESUMO
BACKGROUND: The porcine small intestinal extracellular matrix reportedly has the potential to differentiate into viable myocardial cells. When used in tetralogy of Fallot repair, it may improve right ventricular function. We evaluated right ventricular function after repair of tetralogy of Fallot with extracellular matrix versus bovine pericardium. METHOD: Subjects with non-transannular repair of tetralogy of Fallot with at least 1 year of follow-up were selected. The extracellular matrix and bovine pericardium groups were compared. We used three-dimensional right ventricular ejection fraction, right ventricle global longitudinal strain, and tricuspid annular plane systolic excursion to assess right ventricular function. RESULTS: The extracellular matrix group had 11 patients, whereas the bovine pericardium group had 10 patients. No differences between the groups were found regarding sex ratio, age at surgery, and cardiopulmonary bypass time. The follow-up period was 28±12.6 months in the extracellular matrix group and 50.05±17.6 months in the bovine pericardium group (p=0.001). The mean three-dimensional right ventricular ejection fraction (55.7±5.0% versus 55.3±5.2%, p=0.73), right ventricular global longitudinal strain (-18.5±3.0% versus -18.0±2.2%, p=0.44), and tricuspid annular plane systolic excursions (1.59±0.16 versus 1.59±0.2, p=0.93) were similar in the extracellular matrix group and in the bovine pericardium group, respectively. Right ventricular global longitudinal strain in healthy children is reported at -29±3% in literature. CONCLUSION: In a small cohort of the patients undergoing non-transannular repair of tetralogy of Fallot, there was no significant difference in right ventricular function between groups having extracellular matrix versus bovine pericardium patches followed-up for more than 1 year. Lower right ventricular longitudinal strain noted in both the groups compared to healthy children.
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BACKGROUND: Numerous advances in surgical techniques and understanding of single-ventricle physiology have resulted in improved survival. We sought to determine the influence of various demographic, perioperative, and patient-specific factors on the survival of single-ventricle patients following stage 1 palliation at our institution. METHODS: We conducted a retrospective study of all single-ventricle patients who had undergone staged palliation at our institution over an 8-year period. Data were collected from the Society of Thoracic Surgeons Congenital Heart Surgery database and from patient charts. Information on age, weight at stage 1 palliation, prematurity, genetic abnormalities, non-cardiac anomalies, ventricular dominance, and type of palliation was collected. Information on mortality and unplanned reinterventions was also collected. RESULTS: A total of 72 patients underwent stage 1 palliation over an 8-year period. There were 12 deaths before and one death after stage 2 palliation. There was no hospital mortality following Glenn or Fontan procedures. On univariate analysis, low weight at the time of stage 1 palliation and prematurity were found to be risk factors for mortality following stage 1 palliation. However, multivariable Cox regression analysis revealed weight at stage 1 palliation to be a strong predictor of mortality. The type of stage 1 palliation did not have any influence on the outcome. No difference in survival was noted following the Glenn procedure. CONCLUSION: Low weight has a deleterious impact on survival following stage 1 palliation. This is mitigated by stage 2 palliation. The type of stage 1 palliation itself has no bearing on the outcome.
Assuntos
Peso Corporal/fisiologia , Cardiopatias Congênitas/mortalidade , Ventrículos do Coração/anormalidades , Procedimentos Cirúrgicos Cardíacos/métodos , Bases de Dados Factuais , Feminino , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Masculino , Complicações Pós-Operatórias , Nascimento Prematuro , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Sobrevida , Tennessee/epidemiologia , Resultado do TratamentoRESUMO
Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.
Assuntos
Veias Pulmonares/anormalidades , Anormalidades Congênitas/diagnóstico por imagem , Evolução Fatal , Feminino , Humanos , Recém-Nascido , MasculinoRESUMO
UNLABELLED: Introduction Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes. Materials and methods A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation. RESULTS: The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58-1; specificity, 0.99, 95% CI, 0.96-1) was predictive of the need for emergent left atrial decompression. CONCLUSION: Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.
Assuntos
Septo Interatrial/cirurgia , Cateterismo Cardíaco/métodos , Átrios do Coração/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Pré-Escolar , Ecocardiografia Doppler , Feminino , Transplante de Coração , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Análise Multivariada , Veias Pulmonares/diagnóstico por imagem , Estudos Retrospectivos , Ultrassonografia Pré-NatalRESUMO
The CentriMag ventricular assist device is an extracorporeal, third-generation, continuous flow device. The rapidity and simplicity of operation along with low priming volume make it attractive for use in children with refractory heart failure. We report the successful use of CentriMag as a bridge to recovery in a child and discuss issues that are unique to its use in children.
Assuntos
Ecocardiografia Transesofagiana , Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Criança , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Resultado do TratamentoRESUMO
OBJECTIVE: Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. METHODS: A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. RESULTS: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). CONCLUSIONS: Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.
Assuntos
Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/cirurgia , Atresia Pulmonar/mortalidade , Atresia Pulmonar/cirurgia , Gerenciamento Clínico , Anomalia de Ebstein/complicações , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Atresia Pulmonar/complicações , Valva Pulmonar/cirurgia , Valva Tricúspide/cirurgiaRESUMO
Hypoplastic left heart complex presents a diverse spectrum of variants. Traditional management has been dichotomous, involving either single ventricle palliation or high-risk biventricular repair. Surgical approaches to achieve biventricular repair in children with borderline left ventricle are continually evolving. One such method is the staged left ventricular recruitment (SLVR) strategy. Here, we illustrate a unique surgical approach to SLVR, achieving biventricular repair over 22 months in a neonate with hypoplastic left heart variant.
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OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.
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Consenso , Anomalia de Ebstein , Humanos , Recém-Nascido , Anomalia de Ebstein/cirurgia , Anomalia de Ebstein/fisiopatologia , Lactente , Medição de Risco , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fatores de Risco , Resultado do Tratamento , Cirurgia Torácica/normasRESUMO
Pediatric cardiac surgery in Jehovah's Witness patients who refuse the use of blood products remains a challenge because of the extreme hemodilution caused by priming the circuit and subsequent cardiopulmonary bypass. We report our successful strategy for reducing the prime volume for a 2-year-old Jehovah's Witness patient who required open heart surgery. We modified our conventional bypass circuit requirements for this size child by incorporating a lower prime oxygenator and reducing the size of the venous line and circuit, which decreased the circuit prime volume. We managed to reduce our initial sanguineous prime volume from 315 to 210 mL. The prime was further reduced to 160 mL by minimizing circuit length at the field and with venous prime sequestration prebypass. The postbypass hematocrit was 31%. Bloodless pediatric cardiac surgery in Jehovah's Witness patients can be performed safely. Incorporating a lower prime oxygenator into a revised circuit alleviated the need for blood transfusion and allowed us to achieve our calculated flow rate of 2.6 L/min/m2 while maintaining a hematocrit of 31%.
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Procedimentos Médicos e Cirúrgicos sem Sangue , Ponte Cardiopulmonar/métodos , Testemunhas de Jeová , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Religião e MedicinaRESUMO
Cardiopulmonary bypass is an integral and indispensable part of surgical repair of congenital heart defects. While the complications and morbidity secondary to the use of cardiopulmonary bypass has decreased considerably, there remains a significant incidence of clinically relevant renal and neurological injury. To provide more physiological delivery of oxygenated blood to the end-organs, our center has been successfully using a high-flow, high hematocrit cardiopulmonary bypass strategy since 2006. The essential components of this strategy include maintaining high flows (typically 200 mL/kg/min in neonates, 150-175 mL/kg/min in older infants weighing <10 kg, and 2.6 L/min/m2 in older children) throughout the duration of cardiopulmonary bypass irrespective of patient temperature, as well as maintaining a hematocrit of at least 32% on cardiopulmonary bypass. The incidence of post-operative acute kidney injury (around 3%) and clinical acute neurological events (<1%) with this strategy is considerably less when compared to other contemporary publications using the conventional cardiopulmonary bypass strategy. In this review, we discuss the rationale behind our approach and present evidence to support the high-flow, high-hematocrit strategy. We also discuss the practical aspects of our strategy and describe the adjuncts we use to derive additional benefits. These adjuncts include the use of a hybrid pH/alpha stat strategy during cooling/rewarming, aggressive use of conventional ultrafiltration during cardiopulmonary bypass, a terminal hematocrit of 40-45%, and avoidance of milrinone and albumin in the early peri-operative period. This results in a very low incidence of post-operative bleeding, facilitates chest closure in the operating room even in most neonates, helps in reducing the need for post-operative blood product transfusion and helps in achieving a favorable post-operative fluid balance early after surgery.