Introduction: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to in uence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival.Methods: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period , with a focus on those with the combination of exomphalos major and major CHD (EMCHD).Results: There were 123 patients with exomphalos identi ed, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin).In the ExoMaj group; 17% had major CHD (10/59), M: F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention.Conclusion: EMCHD is associated with high mortality. The most signi cant nding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Aims To evaluate comparative outcomes of the repair of pulmonary atresia with ventricular septal defect (PAVSD), done as staged repair (SR) or single-stage complete repair (CR). Methods A systematic online search was conducted using the following databases: PubMed, Scopus, Cochrane database, The Virtual Health Library, Clinical trials.gov, and ScienceDirect for studies comparing SR versus CR for treatment of PAVSD. Total mortality rate, operative and early post-operative mortality rate, post-operative ventilation duration, duration of postoperative intensive care unit (ICU) stay, need for post-operative extra-corporeal membrane oxygenation (ECMO) support rate, transcatheter re-intervention rate, unplanned re-operation rate, freedom from right ventricular outflow tract (RVOT) re-intervention, and length of hospital stay (LOS) were the evaluated outcome parameters. Results Four comparative studies reporting a total of 264 patients who underwent SR (167 patients) or CR (97 patients) were included. Total mortality was higher in the SR group compared to the CR group (odds ratio (OR) 2.58, P = 0.03). Two groups were comparable regarding operative and early post-operative mortality (OR 1.37, P = 0.62), post-operative ventilation duration (mean difference (MD) − 1.58, P = 0.43), need for post-operative ECMO support rate (OR 4.72, P = 0.16), transcatheter re-intervention rate (OR 0.60, P = 0.53), unplanned re-operation rate (OR 0.73, P = 0.57), and LOS (MD − 3.39, P = 0.45). Higher rate of freedom from RVOT re-intervention was observed in the SR group (OR 4.16, P = 0.00001). Conclusion Single-staged early CR of PAVSD significantly reduced total mortality rate as compared with SR. However, there is life-long risk of frequent need for RVOT re-interventions. Future high-quality randomised studies with robust methodological qualities are encouraged to evaluate the optimal timing and technique for repair of PAVSD, by analysing more outcomes in large patient groups and multi-centre studies.

Aim: To evaluate outcomes of interventions for severe aortic valve stenosis (AS), whether it is done by surgical aortic valvotomy (SAV) or balloon aortic dilatation (BAD). Results: Eleven studies with total number of 1733 patients; 743 patients had SAV, while 990 patients received BAD. There was no significant difference in early mortality (odds ratio [OR]: 0.96, p = 0.86), late mortality (OR: 1.28, p = 0.25), total mortality (OR: 1.10, p = 0.56), and freedom from aortic valve replacement (OR: 1.00, p = 1.00). Reduction of aortic systolic gradient was significantly higher in the SAV group (OR: 2.24, p = 0.00001), and postprocedural AR rate was lower in SAV group (OR: 0.21, p = 0.00001). Conclusion: SAV is associated with better reduction of aortic systolic gradient and lesser post procedural AR which reduce when compared with BAD.

Introduction: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. Methods: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990–2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). Results: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M: F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. Conclusion: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Introduction: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. Methods: The hospital records, echocardiographic and other imaging modality data, outpatients’ records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. Results: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2–60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months – 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. Conclusions: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.