In dextro-transposition of the great arteries (D-TGA) and double outlet right ventricle with subpulmonary ventricular septal defect (DORV-SPV) and predicted transposition physiology, the presence of associated lesions, such as ventricular septal defect (VSD), coarctation of the aorta (CoA) or pulmonary stenosis (PS), impacts prenatal counseling and complicates neonatal surgery. This study aimed to evaluate the diagnostic accuracy of fetal echocardiography (FE) and its ability to predict the postnatal surgical plan in fetuses with D-TGA or DORV-SPV. This was a single-center retrospective cohort study of fetuses with D-TGA or DORV-SPV who were liveborn, had a recorded plan for postnatal intervention and were managed at Lucile Packard Children's Hospital, Palo Alto, CA, USA, between January 2013 and January 2024. In cases with serial FE examinations, all available FE reports were included in the analysis, in addition to the first postnatal echocardiogram report. For echocardiograms with incomplete measurement data, a single reader retrospectively obtained FE and postnatal echocardiogram measurements while blinded to clinical outcomes. Prenatal predictions for postnatal surgical repair were extracted from the fetal cardiology consultation notes and postnatal clinical data were obtained by chart review. Final FE and postnatal echocardiogram diagnoses, as well as prenatal surgical-plan predictions and subsequent postnatal surgical repairs performed, were compared and the percentage concordance was calculated to obtain FE diagnostic accuracy. Receiver-operating-characteristics (ROC) curves were generated to evaluate the association of FE aortic and pulmonary measurements with postnatal CoA repair. The study included 99 fetuses, of which 45 were diagnosed postnatally with simple D-TGA with no associated lesions (i.e. D-TGA with intact ventricular septum), 38 with complex D-TGA with associated lesions and 15 with DORV, and one neonate was diagnosed prenatally with DORV-SPV but postnatally diagnosed with truncus arteriosus. In the majority (65%) of patients, serial FE was performed. The percentage concordance between fetal and postnatal echocardiogram diagnoses was high for simple D-TGA (95% (42/44)) and D-TGA with VSD and PS (100% (3/3)). The concordance was progressively lower for diagnoses of D-TGA with VSD (93% (25/27)), DORV-SPV (60% (3/5)), DORV-SPV with CoA (40% (4/10)), D-TGA with VSD and CoA (29% (2/7)), D-TGA with isolated CoA (0% (0/2)) and DORV-SPV with PS (0% (0/1)). Similarly, surgical-plan accuracy between prenatal prediction and postnatal repair performed was highest for cases of simple D-TGA and D-TGA with VSD and PS, while it was lower for other diagnoses. Qualitative descriptions of VSD size changed frequently over serial FE examinations, with VSDs generally described as larger on later FE. However, between the final FE and postnatal echocardiogram, in those with discordance, the description of VSD size usually became smaller. Only 50% of small VSDs identified on postnatal echocardiography required repair. All patients who underwent postnatal repair of CoA had a VSD present. All area under the ROC curve values for aortic measurements and for ratios of aortic-to-pulmonary dimensions in predicting the need for postnatal CoA repair were greater than 0.9. Challenges remain in the accurate diagnosis and prediction of the postnatal surgical plan in fetuses with complex D-TGA and DORV-SPV. The assessment of qualitative VSD size description changed frequently over serial FE evaluations, and between final FE and postnatal echocardiogram, with small defects often not requiring repair. Aortic and aortic-to-pulmonary ratio measurements in fetuses with D-TGA or DORV-SPV appear to have improved the discriminatory ability in identifying cases that undergo postnatal CoA repair relative to fetuses with isolated CoA. © 2026 International Society of Ultrasound in Obstetrics and Gynecology.

A 4-week-old American Quarter Horse colt presented with a recent history of diarrhea and decreased activity level. On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. While in the hospital, the clinical condition of the foal warranted further imaging to determine the cause and extent of cardiac disease. A variety of congenital cardiac malformations were identified during echocardiographic examination and autopsy, including a double outlet right ventricle and a subpulmonary interventricular septal defect (Taussig-Bing anomaly), ventricular inversion with atrioventricular discordance, tricuspid valve atresia, a septum primum interatrial septal defect, mitral valve dysplasia with a cleft in the septal mitral valve cusp, aortic, and subaortic stenosis, tubular hypoplasia of the ascending aorta and the aortic arch, a patent ductus arteriosus, an aberrant circumflex coronary artery, and aberrant left and right subclavian arteries. Echocardiographic and postmortem findings of the cardiac defects in this foal are presented and discussed.

The neonatal arterial switch operation has become the standard of care for transposition of the great arteries, including transposition with intact ventricular septum, transposition with ventricular septal defect with or without aortic arch hypoplasia, and double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). While technically demanding, the operation is enormously gratifying and exciting for the congenital heart surgeon. In the current era, outcomes and expectations for the neonatal arterial switch operation are extremely high with many centers, including our own, reporting 30-day or hospital survivorship approaching 100%. Long-term results are also outstanding, although these patients do require lifelong follow-up and have the potential for need of remedial surgical intervention.

Objective To discuss the anatomical morphologies of the coronary arteries and frequencies of unusual coronary arteries in complete transposition of the great arteries and double outlet right ventricle (DORV) associated with a subpulmonic ventricular septal defect (VSD). Methods Between March 1999 and August 2012, 1,078 patients with complete transposition of the great arteries or DORV with subpulmonary VSD underwent arterial switch operations (ASOs) and were visually evaluated to classify their coronary artery morphology during open heart surgery. Results The coronary arteries could be classified into five patterns with several subtypes. Unusual coronary arteries were observed in 248 of the 1,078 cases, providing a frequency of 23.01%. The frequencies of the patients with transposition of the great arteries with intact ventricular septum (TGA/IVS), TGA/VSD, and DORV with subpulmonary VSD were 17.65, 23.28, and 31.84%, respectively. The most common morphologies were the right coronary artery (RCA) originating from sinus 1 and circumflex (CX) originating from sinus 2 (1R, AD; 2CX; 26.50%); the CX originating from sinus 2 (1AD; 2R, CX; 21.36%); the RCA, left anterior descending artery, and CX originating from single sinus 2 (2R, AD, CX; 13.24%). The in-hospital mortalities of the patients with or without unusual coronary arteries after ASO were 14.1 and 6.02%, respectively. Conclusion Patients with complete transposition of the great arteries or DORV with subpulmonary VSD have a high frequency of unusual coronary arteries, which might greatly impact on the mortality for ASO. Improving the preoperative diagnostic criteria for coronary artery morphology may significantly increase the success rate for ASOs.

We reviewed our 20-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) or double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) to assess the early and long-term outcomes. Between January 1995 and December 2014, 139 consecutive patients who underwent ASO for TGA or Taussig-Bing anomaly were included in this retrospective study. The median age at the operation was 9 (0-485) days, and 97 patients (70 %) underwent ASO less than 2 weeks. The median weight was 3.3 (2.1-10.3) kg. The patients were divided into three groups; simple TGA (n = 78) included patients with TGA with intact ventricular septum, complex TGA (n = 46) included those who had TGA with ventricular septal defect or other anomalies, and Taussig-Bing anomaly (n = 15). Median follow-up duration was 72.5 (0.4-230) months. There were 3(2.2 %) in-hospital deaths. One patient (0.7 %) underwent early reoperation due to coronary insufficiency. Late deaths occurred in 3 (2.2 %) of 136 survivors. The Kaplan-Meier's survival rate was 97.6 ± 1.4 % at 15 years. Twenty-three patients (16.9 %) required 26 reintervention. The freedom from reintervention rates were 82.5 ± 3.7 % at 5 years and 75.8 ± 4.7 % at 10 years, respectively. Median interval between ASO and first reintervention was 22.8 (6.4-89.2) months. The multivariate analysis showed that diagnosis of Taussig-Bing anomaly (hazard ratio, 7.09; P < 0.001) and side by side great artery relationship (hazard ratio, 7.98; P = 0.001) were independent risk factors for reoperation. Five patients (3.9 %) had developed at least moderate neo-aortic regurgitation during the follow-up and one patient underwent reoperation mainly for neo-aortic regurgitation. By multivariate analysis, Taussig-Bing anomaly was the risk factor for at least moderate neo-aortic regurgitation (P = 0.035). ASO can be performed with a low risk of early mortality and satisfactory long-term outcomes even in a small volume center. Close long-term surveillance is mandatory to detect structural or hemodynamic changes.