Right atrial isomerism is a rare and severe isomerism. It is frequently associated with complex congenital heart disease and various extracardiac anomalies. Imaging diagnosis of right atrial isomerism is a challenge. Multisystem and complex anomalies in a 24-week-old fetus were diagnosed with prenatal ultrasound, postnatal computed tomography angiography (CTA), and autopsy. The ultrasound detected most major cardiovascular anomalies including right atrial isomerism and total anomalous pulmonary venous connection. The CTA further detected thoracic and abdominal malformations such as bilateral morphologically right bronchus, diaphragmatic hernia, asplenia, midline liver, and intestinal malrotation. The autopsy confirmed both ultrasound and CTA findings with additional findings, namely, bilateral trilobed lungs and bilateral morphological right auricles. Prenatal ultrasound and postnatal CTA can be complementary to each other in detecting multi-system complex anomalies. Their combined use can be useful for prenatal counseling and postpartum management.

A meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC).
Data bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC. Publications were screened by 2 authors independently for criteria inclusion, methodological quality assessment and data extraction. The Newcastle-Ottawa Scale and the Agency for Healthcare Research and Quality checklist were obtained to assess the quality of the studies. Data were pooled by the random effect model or the fixed effect model according to the heterogeneity test.
A total of 16 studies (2,385 participants) were included in the meta-analysis. All included studies were retrospective studies. Six potential risk factors were pooled, 5 of which were significantly associated with postoperative PVO. Patients with preoperative PVO were more likely to suffer from postoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01]. Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. A mixed type TAPVC plays a critical role in postoperative PVO [OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04]. Inverse variance analysis showed that longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO. Heterotaxy [OR = 1.18, 95% CI = 0.13, 10.45, P = 0.88] was not statistically significant as a risk factor for postoperative PVO.
This meta-analysis may provide a perspective on the risk factors for postoperative PVO after TAPVC, thus leading to more studies predicting postoperative PVO after TAPVC with our findings.

Sutureless closure has been used for primary repair of total anomalous pulmonary venous connection (TAPVC) for over 20 years but its superiority over conventional technique is still uncertain. This systematic review was conducted to compare the effectiveness of sutureless closure and conventional surgery as the primary repair for TAPVC. Systematic search was performed in June 2021 on 12 databases. All studies comparing sutureless and conventional surgery for TAPVC were included. The primary endpoints were early mortality, overall mortality, postoperative pulmonary venous stenosis (PVS), and reoperation. Meta-analysis of two-arm studies was performed with several sensitivity and subgroup analyses. Six retrospective studies with 767 patients were included in meta-analyses. Sutureless closure significantly reduced the risk of early mortality, overall mortality, postoperative PVS, and reoperation by 53%, 45%, 77%, and 67% compared to conventional technique, respectively. No heterogeneity was found and presence of publication bias was non-significant. The results were consistent in all sensitivity analyses. Subgroup analyses revealed that sutureless closure was superior to conventional technique in patients with and without preoperative pulmonary venous obstruction, and neonates and non-neonates. Sutureless closure is better than conventional closure as the primary surgery for TAPVC patients. We advocate using sutureless closure for patients with TAPVC. Future large-scale observational studies or clinical trials are required to confirm our findings.

Horseshoe lung (HL) is a rare congenital anomaly that has been classically associated with Scimitar syndrome. Very few cases have been described in the context of the VACTERL spectrum. We present a case of a newborn girl with mesocardia, tracheoesophageal fistula, and imperforated anus, who required O2 support at birth and during hospitalization. A chest CT angiography revealed a HL as an incidental finding. We suspect that HL and the VACTERL spectrum, are not separated entities but likely a further expansion of VACTERL-associated symptoms. HL might be underdiagnosed in asymptomatic patients as Chest CT angiography is not part of the routine work up for patients with VACTERL association.

Total anomalous pulmonary venous drainage (TAPVD) is caused by the absence of the congenital connection between the pulmonary vein and left atrium. This causes blood drainage into the right atrium, resulting in poor development of the left atrium and increasing the burden for the right atrium. It is accompanied by an atrial septal defect. TAPVD mostly is diagnosed during the fetal period and rarely is reported in adults. Atrial fibrillation (AF), a common arrhythmia, originates primarily from the joint of the pulmonary vein and left atrium, whereas AF originating from the right atrium has not been documented. Herein, we report the case of a 45-year-old male diagnosed with TAPVD accompanied by AF. After the correction of TAPVD and radiofrequency ablation (RFA) for AF performed under general anesthesia and cardiopulmonary bypass, the patient returned to normal sinus rhythm and showed no AF recurrence during two years of follow up.

Prior studies have reported a high mortality and incidence of post-repair pulmonary venous obstruction (PVO) in mixed total anomalous pulmonary venous connection (TAPVC). This study sought to review the surgical outcomes in this entity.
A review of 61 patients undergoing surgical repair of mixed TAPVC was conducted. Patients with a single ventricle were excluded. Patients were subdivided into 3 groups according to Chowdhury\'s classification. Predictors for death and postoperative PVO were explored by Cox regression model.
This study trended towards an older cohort with a median age of 88 days (interquartile range, 56.5-177). Twelve patients belonged to \'2 + 2\' type, 40 belonged to \'3 + 1\' type and the remaining 9 belonged to bizarre type. There were no early death and 7 late deaths. Follow-up was available in 96.7% of the patients after discharge with a median duration of 53 months (range, 1-177). Nineteen patients developed post-repair PVO among whom 2 required reintervention. Patients with preoperative PVO had a 4-fold higher risk (95% confidence interval, 1.36-12.38) of postoperative PVO than those without and were more likely to die (P = 0.009). No statistical difference was observed among the 3 subgroups in terms of mortality (P = 0.058) and postoperative PVO (P = 0.186).
Preoperative PVO was significantly associated with postoperative PVO. There was no statistical difference in terms of death and postoperative PVO among the 3 subtypes of mixed TAPVC. Mid-term results favoured a complete rechanneling of pulmonary veins in \'3 + 1\' type.

A meta-analysis was performed for a comparison of outcomes between sutureless technique and conventional surgery for primary repair for total anomalous pulmonary venous connection (TAPVC). Electronic databases including PubMed, EMbase, Scopus, and Cochrane Library were searched systematically for the single-arm studies regarding sutureless repair or conventional surgery, and two-arm studies compared the outcomes of sutureless repair and conventional surgery for TAPVC. Corresponding data were extracted and the methodological quality was assessed by two reviewers independently. 26 studies were included, involving a total of 2702 patients. It was observed that compared with conventional surgery, sutureless technique was associated with a lower occurrence rate of post-operative pulmonary veins obstruction (PVO) (4.6% vs. 13.5%, OR 0.54 in favor of sutureless technique) and re-operations due to PVO (3.4% vs. 12.4%, 0.25 in favor of sutureless technique). However, meta-analyses of post-operative early (OR 0.57; 95% CI 0.27-1.19; P = 0.13), late (OR 0.37; 95% CI 0.13-1.06; P = 0.13), and overall (OR 0.61; 95% CI 0.36-1.03; P = 0.07) mortality showed no significant difference between sutureless technique and conventional surgery. Compared with conventional surgery, sutureless technique was associated with a lower occurrence rate of post-operative PVO and re-operations due to PVO. Meanwhile, post-operative early, late, and overall mortality were not statistically different between two surgical approaches. Sutureless technique is beneficial in the primary repair of TAPVC regarding post-operative PVO and re-operations due to PVO. However, the level of evidence was low and randomized controlled trials should be designed to evaluate the safety and effectiveness of sutureless technique for TAPVC.

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, \"S\"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the \"scimitar\" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

UNASSIGNED: A meta-analysis was performed to compare the differences in outcomes between sutureless technique and conventional surgery for primary repair of Total Anomalous Pulmonary Venous Connection(TAPVC).
METHODS: Electronic databases, including PubMed, EMbase, Medline, CNKI, Wanfang Data and Weipu Data were searched systematically for the literature aimed mainly at comparing the therapeutic effects for primary repair of TAPVC administered by sutureless technique and conventional surgery. Corresponding data sets were extracted and two reviewers independently assessed the methodological quality.
RESULTS: Seven studies meeting the inclusion criteria were included, involving a total of 1293 subjects. It was observed that sutureless technique entailed a lower occurrence rate of post-operative Pulmonary Veins Obstruction (PVO) (OR, 0.52 95%CI, 0.32-0.86; P = 0.01) and re-operation due to PVO (OR, 0.28;95%CI, 0.09-0.87; P = 0.03). However, meta-analyses of hospitalization time (WMD, 5.92; 95%CI, - 7.97-19.80; P = 0.40) and post-operative mortality (OR, 0.65; 95%CI, 0.41-1.04; P = 0.07) showed no significant differences between sutureless technique and conventional surgery. Meta-analysis of Cardiopulmonary Bypass (CPB) time and aortic cross-clamp time also showed no significant differences between the two surgical approaches (WMD, 5.07; 95%CI, - 9.29-19.42; P = 0.49); (WMD, 5.73; 95%CI, - 7.76-19.23; P = 0.40), but the result remained inconclusive due to pooling result changes after sensitivity analysis.
CONCLUSIONS: Compared with conventional surgery, a lower occurrence rate of post-operative PVO and re-operation due to PVO were associated with sutureless technique. Meanwhile, hospitalization time and post-operative mortality were not statistically different between the two surgical approaches. Pooling result of CPB and aortic cross-clamp time between the two groups remained inconclusive.

Scimitar syndrome or veino-lobaire syndrome (term coined by Felson) is a very rare disease characterized by the combination of cardiopulmonary anomalies, in particular an anomalous right pulmonary venous return, located mostly in the inferior vena cava. We here report the original case of a 6-month-old female infant presenting with acute dyspnea. The diagnosis was suspected on the basis of thoracic radiograph and was confirmed by tomodensitometry which showed a large single right pulmonary vein draining into the right atrium associated with dextrocardia and pulmonary sequestration. The prognosis was based on the size of the left-right shunt and related malformations.