Assess prenatal ultrasound's diagnostic value in fetal cardiac anomalies using an abnormal number of pulmonary artery (PA) branches as an initial clue. Retrospective analysis of 20 fetuses with an abnormal number of PA branches on ultrasound, comparing prenatal ultrasonic findings with postnatal echocardiography, computed tomography (CT), surgery, autopsy, and genetic tests. Summarized ultrasonographic characteristics and occurrence frequencies in 3-vessel trachea (3VT), 3-vessel PA branch, and innominate artery (INA) coronary section. In total, abnormal PA branches included: 1 branch (15 cases: 7 pulmonary artery sling [PAS], 4 unilateral absence of pulmonary artery [UAPA], 4 anomalous origin of 1 pulmonary artery from ascending aorta [AOPA]); 3 branches (2 cases: 1 isolated left subclavian artery [ILSA], 1 isolated left innominate artery [ILINA], absent right ductus arteriosus); 4 branches (3 cases: 2 ILSA, 1 ILINA, double ductus arteriosus). Associated anomalies: tetralogy of Fallot, persistent left superior vena cava, ventricular septal defect, Berry syndrome, and nasal bone dysplasia. Postnatal confirmations were achieved via autopsy (9 cases), imaging/surgery (10 cases), or lost (1 case). Genetic tests (14 cases) were normal. Detection: 63.2% (3VT section), 100% (3-vessel PA branch section), 57.9% (INA coronary section). The 3VT, 3-vessel PA branch, and INA coronary section are key for the diagnosis of fetal cardiac malformations with abnormal PA branches. The spatiotemporal image correlation with high-definition (STIC-HD) live flow sonography can aid in visualizing the vascular connection linked to these anomalies. Identifying features of 1/3/4 PA branches facilitates a systematic assessment of fetal cardiac defects primarily characterized by aberrant PA branching.

Pulmonary artery sling (PAS) is a rare congenital vascular anomaly in which the left pulmonary artery (LPA) originates from the right pulmonary artery (RPA), forming a ring around the tracheobronchial tree. Due to non-specific respiratory symptoms, it is frequently misdiagnosed, leading to significant delays in diagnosis. This report emphasizes the crucial role of quantitative multimodal imaging in establishing a definitive diagnosis, stratifying risk, and guiding optimal surgical planning. A 4-year-and-7-month-old boy presented with a 4-year history of recurrent cough and wheezing that was refractory to standard medical therapy. Echocardiography revealed a dilated main pulmonary artery (MPA) measuring 1.9 cm (Z-score +3.2) and an anomalous origin of the LPA from the RPA, with an elevated peak flow velocity of 1.75 m/s. Contrast-enhanced computed tomography angiography (CTA) subsequently confirmed a Type I PAS, enabling precise quantification of a severe focal stenosis of the LPA (minimal diameter 2.8 mm) and demonstrating the absence of significant intrinsic tracheobronchial stenosis. Based on these findings, the patient underwent successful reimplantation of the LPA onto the MPA with an autologous pericardial patch. The postoperative course was uneventful, with complete resolution of respiratory symptoms, and follow-up imaging confirmed a patent anastomosis with successful hemodynamic outcomes. This case of isolated PAS underscores the indispensable role of a multimodal imaging strategy. While echocardiography can provide initial clues, quantitative CTA is paramount for definitive anatomical classification, precise stenosis quantification, and comprehensive preoperative planning. Early consideration of PAS in children presenting with refractory respiratory symptoms, coupled with advanced imaging, can prevent misdiagnosis and optimize outcomes.

While meso/dextrocardia, a cardiac axis abnormality, is associated with various complications and a poor prognosis, few studies have been reported. We aimed to identify and review patients at our hospital who had been diagnosed with fetal meso/dextrocardia. The medical records of 29 patients diagnosed with fetal meso/dextrocardia between April 1, 2014 and March 31, 2024 were reviewed. We identified eight cases of mesocardia and 21 cases of dextrocardia (17 dextropositions and four dextroversions). Right lung hypoplasia (including 3q trisomy, esophageal bronchopleural fistula, and left pulmonary artery sling) was identified in three cases. Five cases of persistent left superior vena cava (PLSVC) were identified [isolated PLSVC (n = 2), VACTERL association (n = 1), trisomy 13 (n = 2)]. Dextroposition was linked to congenital pulmonary airway malformation (eight cases), left pulmonary sequestration (one case), congenital diaphragmatic hernia (six cases), right lung hypoplasia (one case), and VACTERL association with right lung aplasia and esophageal atresia (one case). Dextroversion was associated with asplenia syndrome (two cases), single-ventricle (one case), and Temple syndrome with PLSVC and bilateral hypoplastic pulmonary arteries (one case). Among 29 newborns, six (20.7%) died during the early neonatal period and seven (24.1%) required postnatal multidisciplinary treatment, highlighting a poor prognosis in many cases. While some patients, such as those with isolated PLSVC, had favorable outcomes, several cases involved severe complications requiring intensive perinatal management. When fetal meso/dextrocardia is detected, it is critical to evaluate fetal anomalies comprehensively and not limit assessment to the heart and lungs.

Although pulmonary artery sling (PAS) is typically a congenital vascular ring anomaly detected in early childhood, its isolated form can also be diagnosed in adulthood. While respiratory symptoms are commonly expected, chest pain should also raise suspicion for PAS.

Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.