完全大血管転位症

BACKGROUND: Congenital heart disease (CHD) is the most prevalent congenital abnormality worldwide and is the primary contributor to neonatal mortality. Obstetricians in China continue to face uncertainties about optimal delivery timing, appropriate delivery mode, perinatal interventions, and postnatal surgical outcomes for fetuses with complex CHD. OBJECTIVE: To investigate integrated pregnancy and perinatal management strategies for fetuses diagnosed with complex CHD by prenatal ultrasound in China. METHODS: A retrospective analysis was performed on 123 fetuses with complex CHD diagnosed by prenatal ultrasound at Peking University People's Hospital between 2016 and 2023. RESULTS: The mean gestational age at ultrasound diagnosis was 23.7 ± 3.4 weeks. Among the 123 cases, 11 (8.9%) were total anomalous pulmonary venous connection (TAPVC), 14 (11.4%) pulmonary valve abnormalities (PVA), 7 (5.7%) right ventricular double outlet (DORV), 13 (10.6%) aortic arch abnormalities, 69 (56.1%) complete transposition of the great arteries (D-TGA), and 9 (7.3%) other types. Following prenatal counseling, all pregnancies were continued. Among the 72 fetuses undergoing prenatal genetic testing, 9 (9/72) showed chromosomal variants. Fifty-five pregnant women (44.72%) attempted trial of labor, of whom 46 (37.40%) achieved vaginal delivery, including 6 (4.88%) assisted with forceps. Trial of labor failed in 9 cases, requiring cesarean section. In total, 77 pregnancies were delivered by cesarean section, yielding a cesarean rate of 62.6%. The mean gestational age at delivery was 37.9 ± 1.4 weeks, and the mean neonatal birth weight was 3099.0 ± 479.6 g. The mean umbilical arterial blood gas pH was 7.31 ± 0.05. Postnatally, 86 infants (69.9%) received prostaglandin. Average oxygen saturation increased from 72.8 ± 6.0% before alprostadil administration to 80.5 ± 5.0% afterward, a statistically significant improvement (p = 0.000). A total of 114 neonates underwent surgical intervention; 98.2% achieved favorable outcomes, whereas two infants (1.8%) died postoperatively. Approximately 13.0% were anticipated to require secondary surgery. CONCLUSION: Pregnancies involving fetuses with complex CHD should avoid preterm birth whenever possible. Complex fetal CHD alone is not an indication for cesarean delivery; the delivery mode should instead be determined by standard obstetric considerations, and individualized management may be adopted according to their opinions and preferences. When both maternal and fetal status remain stable, scheduling delivery at or beyond 39 weeks may be considered. Newborns who present with low postnatal oxygen saturation should receive prostaglandin to keep the ductus arteriosus open, followed by urgent transfer to pediatric cardiac surgery for definitive management. Coordinated, multidisciplinary care supports favorable clinical outcomes.

OBJECTIVES: To characterize hospital survival and risk factors after aortic root replacement performed by congenital units in adult patients, and to compare risk profiles of repaired conotruncal defects to other underlying diagnoses. METHODS: Adult patients after aortic root replacement in a congenital cardiac unit were identified in the European Congenital Heart Surgeons Association (ECHSA) database. Underlying pathology, prior surgeries, and types of index aortic root surgery were characterized. Primary endpoints were hospital and 30-day survival. Secondary endpoints included circulatory arrest, length of intensive care unit and hospital stays, and discharge to chronic care facility. Multiple logistic regression analyses were performed to test for independent risk factors. RESULTS: N=2544 patients after full aortic root replacement, performed in 101 different hospitals (1999 to 2024), were identified. N=249 patients (10%) with primary conotruncal defects included double-outlet-right ventricle (n=14, 6%), pulmonary atresia with VSD (n=42, 17%), transposition of great arteries (n=71, 29%), Tetralogy of Fallot (n=88, 35%), Truncus arteriosus communis (n=34, 14%). Mechanical Bentall was performed in n=163 cases (65%), valve-sparing in n=43 cases (17%), biological Bentall in n=20 (8%), homograft replacement in n=23 (9%). Conotruncal diagnosis was linked to longer CPB times (p<0.001), longer aortic cross-clamp times (p<0.001), longer ICU stay (p<0.001) and longer hospital stay (p<0.001). Multiple logistic regression revealed duration of CPB (p<0.001) and conotruncal lesion (OR 2.1, p=0.037) as independent risk factors for hospital mortality. CONCLUSIONS: Among adult congenital heart defect patients undergoing aortic root replacement, those with conotruncal defects represent a distinct subgroup characterized by a high-risk operative profile.

BACKGROUND: Sensitive fetal prediction of acute neonatal compromise at birth following a prenatal diagnosis of dextro-transposition of the great arteries/intact ventricular septum (dTGA/IVS) remains challenging. Using a large multicenter prospectively collected cohort, we aimed to determine if fetal echocardiogram findings could sensitively and specifically predict neonatal hypoxia. METHODS: Twenty-three centers enrolled maternal/infant dyads. Demographics and clinical information were collected. Previously reported fetal predictors were evaluated from the 3rd trimester by a core lab blinded to outcome. Primary outcome was preoperative neonatal hypoxia (PaO2 <30 mmHg or SpO2 <75% if PaO2 was unavailable). Univariate and multivariate analyses of predictors with the primary outcome were performed. Sensitivity analyses were explored. Incidence of balloon atrial septostomy (BAS) and mortality were calculated. RESULTS: Of the 179 maternal/infant pairs with a fetal diagnosis of dTGA/IVS and postnatal data, 132 had data at all time points and were included in the final cohort, 50 (38%) with neonatal hypoxia and 38/50 (76%) underwent balloon atrial septostomy. Univariate and multivariate analyses showed no significant anatomic or physiologic fetal echocardiography predictors of neonatal hypoxia. Sensitivity analysis showed that an increasing Foramen ovale:Total septal length ratio and increasing atrial septal excursion ratio decreased the likelihood of combined outcome of hypoxia and BAS, but still did not provide useful discrimination to predict outcome. Survival to initial hospital discharge occurred in 131/132(99%). CONCLUSIONS: Our multicenter prospective study did not identify any sensitive prenatal predictors of neonatal hypoxia in infants prenatally diagnosed with TGA/IVS. High risk delivery planning for the possibility of neonatal hypoxia and urgent BAS is needed for all infants with prenatally diagnosed TGA/IVS.

BACKGROUND: Myocardial bridging in adult patients with congenital heart disease is rare. CASE SUMMARY: A 21-year-old man with d-transposition of the great arteries who underwent the Senning procedure presented with exertional fatigue and chest pain. Medical examination revealed ascending aortic stenosis and myocardial bridging of the left anterior descending artery. The patient underwent ascending aortic replacement and coronary artery bypass grafting by saphenous vein-free right internal mammary artery graft. Postoperative examination demonstrated excellent graft patency and improvement of ischemia. DISCUSSION: This case highlights a rare presentation of symptomatic myocardial bridging decades after the Senning procedure. Careful graft selection and design are essential to achieve durable revascularization in young patients. TAKE-HOME MESSAGES: Myocardial bridging should be considered as a potential cause of myocardial ischemia in adult patients with congenital heart disease. When myotomy is technically challenging or high-risk, carefully planned coronary artery bypass grafting with an appropriate graft strategy can provide effective revascularization.

Adults with dextro-transposition of the great arteries (d-TGA) repaired with an atrial switch operation frequently develop systemic atrioventricular valve regurgitation (SAVVR), which is strongly associated with progressive systemic right ventricular (RV) failure and adverse outcomes. Surgical reintervention carries substantial risk in this population, while experience with transcatheter edge-to-edge repair (TEER) remains limited, particularly in atrial-switch anatomy requiring transbaffle access. We report a technically novel strategy using right internal jugular delivery and electrosurgery-assisted transbaffle puncture to facilitate systemic AV valve TEER after Mustard repair when transfemoral access was unsuitable. We report a 32-year-old man with atrial-switch d-TGA, prior systemic atrioventricular valve annuloplasty, transvenous implantable cardioverter-defibrillator leads, and severe SAVVR causing advanced heart failure. At baseline, he was in NYHA class III, with 6-min walk distance of 290 m and NT-proBNP of 7100 pg/mL. Preprocedural imaging demonstrated hostile iliofemoral venous anatomy, precluding transfemoral access. A multimodality imaging-guided strategy using right internal jugular venous access and electrosurgery-assisted transbaffle traversal was employed to reach the pulmonary venous atrium. TEER of the systemic AV valve was successfully performed with implantation of two PASCAL ACE devices, resulting in SAVVR reduction from severe to mild without procedural complications or need for inotropic or intravenous vasodilator support. At 6-month follow-up, the patient remained in NYHA class II, 6-min walk distance improved to 365 m, NT-proBNP decreased to 1670 pg/mL, and SAVVR reduction remained sustained. This case demonstrates that SAVV TEER in atrial-switch d-TGA can be performed via a jugular approach using electrosurgery-assisted transbaffle access when transfemoral routes are unsuitable. An anatomy-driven, Heart Team-based approach integrating advanced imaging may expand transcatheter treatment options for highly selected adults with complex congenital heart disease and limited surgical alternatives.