三尖弁閉鎖症

CLINICAL CONDITION: A 13-year-old child with unrepaired thoracoabdominal ectopia cordis presented with cyanosis, effort intolerance, and recurrent respiratory infections. Evaluation demonstrated tricuspid atresia, a hypoplastic right ventricle, pulmonary atresia with hypoplastic pulmonary arteries supplied by major aortopulmonary collateral arteries, a large subaortic ventricular septal defect, an unrestricted atrial septal defect, bilateral superior vena cavas, and complete atrioventricular block. KEY QUESTIONS: What is the spectrum and natural history of thoracoabdominal ectopia cordis with complex congenital heart disease? What is the role of multimodality imaging in defining the feasibility of intervention? What therapeutic options should the multidisciplinary team consider, and what key information is required before choosing among them? What anatomical and physiological factors influenced the choice between intracardiac repair, palliation, and conservative management, and how does published experience inform these decisions? How does the presence of complete atrioventricular block modify management considerations in this setting? OUTCOME: Multimodality imaging defined severe anatomical constraints, including limited intrathoracic space and a major aortopulmonary collateral artery-dependent pulmonary circulation. After multidisciplinary discussion, the family elected to continue conservative management. TAKE-HOME MESSAGES: Prolonged survival to adolescence is possible in unrepaired thoracoabdominal ectopia cordis with severe single-ventricle physiology and complete atrioventricular block through a precarious physiological equilibrium. Multimodality imaging and multidisciplinary assessment are essential for identifying cases suitable for intervention vs those best managed conservatively.

OBJECTIVES: The bidirectional Glenn (BDG) procedure, a key palliative intervention for single-ventricle physiology, is conventionally performed using cardiopulmonary bypass (CPB) despite its associated complications and costs. However, the comparative impact of CPB versus off-pump techniques on perioperative safety, neurological risk and long-term outcomes remains controversial. Therefore, this meta-analysis aimed to determine whether performing the BDG procedure without CPB improves perioperative outcomes (including morbidity, mortality, and neurological events), resource utilisation, and long-term survival compared to the conventional on-pump approach. METHODS: This review systematically searched PubMed, EMBASE and Cochrane Central from inception until January 2025 for comparative randomised and observational studies reporting outcomes in patients undergoing the BDG procedure with versus without CPB. The primary outcome was perioperative mortality (≤30 days or discharge); secondary outcomes included operative duration, recovery parameters (ventilation time, vasoactive support, intensive care unit (ICU)/hospital stay) and postoperative complications (infection, bleeding, neurological events, arrhythmias). RESULTS: A total of 5 studies (2 randomised controlled trials, 3 observational studies; N = 339, 55% with CPB, 45% without CPB) were included. Patients had a mean age range of 6-66 months and mean weight of 6-16 kg. Common anomalies included double-outlet right ventricle and tricuspid atresia. Perioperative mortality did not differ significantly between groups (odds ratio [OR] = 1.15, 95% confidence interval [CI] = 0.31-4.20; P = 0.83). Significant benefits favouring the off-pump approach were observed for operation duration (mean difference [MD] = 31.98 min, 95% CI = 14.97-48.99; P = 0.0002), duration of inotropic support (MD = 7.05 hours, 95% CI = 5.48-8.63; P <0.00001), duration of mechanical ventilation (MD = 4.09 hours, 95% CI = 3.26-4.92; P <0.00001) and ICU length-of-stay (MD = 0.73 days, 95% CI = 0.55-0.91; P <0.00001). No significant differences were found for hospital length-of-stay, postoperative bleeding, neurological complications, oxygen saturation, superior vena cava pressure, infection or arrhythmias. Risk of bias was assessed via the ROBINS-I and ROB2 assessment tools for non-randomised and randomised trials, respectively; a funnel plot was constructed to assess for publication bias. CONCLUSION: The findings suggest that, although off-pump BDG procedure is associated with shorter operative and recovery AU: Please provide the accepted date. times than the on-pump approach, it does not demonstrate superior perioperative mortality or hospital discharge outcomes. Given the risk of conversion and lack of extended follow-up in reporting studies, further randomised multicentre studies with standardised outcomes are warranted before off-pump is deemed a superior option.

A neonate with prenatally suspected complex single ventricle physiology was found after birth to have tricuspid atresia with a large ventricular septal defect, double outlet right ventricle, L-malposed great arteries, and severe subpulmonary stenosis. The infant presented at term via cesarean delivery for breech presentation with cyanosis, poor tone, and absent spontaneous respirations, requiring immediate positive pressure ventilation and initiation of prostaglandin E₁ to support ductal patency during hemodynamic stabilization. Early transthoracic echocardiography confirmed complex single ventricle anatomy with a hypoplastic right ventricle, L-malposed great arteries, and a restrictive subpulmonary outflow tract, consistent with a Type IIIb tricuspid atresia subtype with reduced pulmonary blood flow. The patient underwent staged surgical palliation beginning with a 3 mm modified Blalock-Taussig shunt via central sternotomy at one month of age, which was complicated by medically managed necrotizing enterocolitis but otherwise followed by a stable interstage period with appropriate oxygen saturations. The patient had low-normal branch pulmonary arteries, and there was no patent ductus arteriosus. At five and a half months, she underwent a hemi-Fontan procedure with bilateral pulmonary artery patch augmentation, atrial septectomy, and shunt takedown, resulting in a patent cavopulmonary connection and adequate systemic output despite transient postoperative ventricular dysfunction. The child was subsequently discharged on standard heart failure and antiplatelet therapy, with gradual weaning of afterload reduction and diuretics as ventricular function improved and oxygen saturation targets were maintained. This case illustrates how detailed anatomical characterization of tricuspid atresia, particularly the relationship of the great arteries and degree of pulmonary outflow obstruction, guides selection and timing of staged palliation. It also underscores the value of coordinated prenatal and postnatal evaluation, early initiation of prostaglandin therapy, and close interstage monitoring in achieving a favorable early outcome in a rare and complex tricuspid atresia subtype that remains eligible for eventual Fontan completion.