Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular anomaly, often associated with other defects such as interrupted aortic arch (IAA) or patent ductus arteriosus (PDA). We report a 12-day-old male neonate presenting with respiratory distress, edema, and oliguria. Physical examination revealed a grade 2/6 systolic murmur at the left sternal border. Echocardiography identified PFAA with coarctation, IAA, PDA, and an atrial septal defect (ASD), confirmed by cardiac CT. Surgical intervention included aortic arch repair, fifth arch excision, and ASD closure. The postoperative recovery was smooth, and the patient remained clinically well with normal cardiac function during the 1-year follow-up period. This case underscores the critical role of echocardiography in early diagnosis and surgical planning for complex congenital heart disease.

Central venous catheterization is crucial for the perioperative and postoperative management of neonatal cardiac surgery patients. The procedure can be challenging due to the small size of the vessels, and it carries a high risk of morbidity and mortality. The most common sites for catheter insertion are the jugular or femoral veins; however, jugular access is typically avoided in patients with univentricular heart disease. The most frequent complications associated with central venous catheters are thromboembolic events and infections. Here, we present a case of lactescent urine in the postoperative period following cardiac surgery, attributed to the migration of a central venous catheter into the renal vein. We report the case of a newborn with an antenatal diagnosis of type B aortic arch interruption, who underwent complete surgical correction on day 9 of life. The infant presented with lactescent urine on postoperative day 5. Extensive investigations revealed that the central venous catheter had migrated into the right renal vein. Lactescent urine is an uncommon presentation in children and is most often associated with parasitic infections. In our case, this condition was related to the diffusion of parenteral nutrition into the renal vein. This case illustrates a rare postoperative complication, where a central venous catheter migrated into the renal vein following cardiac surgery.

The arterial switch operation (ASO) is the standard surgical intervention to correct transposition of the great arteries (TGA). The aim of this study is to describe long-term outcomes after the ASO in patients with TGA, and to explore anatomical and clinical factors potentially associated with adverse outcomes, comparing simple and complex TGA. Since December 2001 to October 2023, 233 patients underwent arterial switch operation (158 simple TGA and 75 complex TGA). Kaplan-Meier analysis assessed survival and reoperation risk, Chi-square test and T-student test have been performed in order to analyse pre-, intra- and post-operative data among the two groups. In-hospital mortality was 1.7%. Overall survival rates were 97.9%, 96.8% and 96.8% at 5, 10 and 15 years, respectively. Freedom from surgical reoperation at 15 years was 98.3% in simple TGA and 91% in complex TGA at 15 years (p = 0.039). Reinterventions were infrequent and mainly related to pacemaker implantation and right ventricular outflow tract obstruction. Coronary artery stenosis occurred in 5 patients (2.2%). Risk factors analysis suggested that anatomical complexity such as intramural coronary artery (p = 0.003), aortic arch hypoplasia (p = 0.004), aortic arch interruption (p = < 0.001) and coronary reimplantation with button technique (p = 0.018) are predictive factors for mortality. In addition, complex TGA (p = 0.022) and post-operative aortic regurgitation (p < 0.001) are identified as potential predictive factors for reintervention. Arterial switch operation provides excellent long-term survival and low reintervention rates in TGA. These results support tailored long-term surveillance, particularly for patients with complex anatomy or coronary anomalies.

Truncus arteriosus (TA) is a major cyanotic heart malformation infrequently associated with aortic arch interruption and isolation of the branch pulmonary artery. Anomalous superior vena caval drainage to the left atrium (LA) is hitherto unreported in TA. In a 45-day-old infant diagnosed with TA and cyanosis, anomalous drainage of the right superior vena cava into the LA was not identified preoperatively or intraoperatively. It was detected postoperatively due to persistent hypoxemia. The anomaly posed unique challenges in the subsequent management. This case report highlights the unusual association of systemic venous drainage in TA.

Interruption of the aortic arch is a rare congenital cardiac malformation with rare cases described in adulthood. Survival in adulthood relies on developing collateral networks to maintain distal flow. CHD occurs in almost 50% of Turner syndrome and is the most frequent cause of early mortality. Also, they have an increased risk factor for thoracic aortic dilatation, and elective surgery should be considered according to body surface area. Surgical correction is the preferred treatment for the interrupted aortic arch and aortic root dilatation. We present the case of a 46-year-old patient with Turner syndrome with a diagnosis of interrupted aortic arch and aortic root aneurysm who underwent the Bentall procedure and interposition of a Dacron graft in the descending aorta. Post-procedural recovery was uneventful with a good haemodynamic response.