Common arterial trunk in functionally univentricular hearts: a case series.

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.

Surgical valvotomy versus balloon dilatation for children with severe aortic valve stenosis: a systematic review.

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.

Congenital aortic valve stenosis is disease in which in which babies are born with narrowing of their aortic valve (the valve leading to main body artery). This study aims to evaluate best outcomes for the two main interventions to treat this disease which are; balloon dilatation (keyhole) and open-heart surgery. Our study results showed that there was no significant difference in mortality between the two treatment strategies; however, there is better immediate results in reliving valve narrowing after and less valve leak after open heart surgery than after key hole procedure. These results remain operator dependent and can differ between centers; therefore, more high-quality studies are encouraged to determine best treatment option for aortic stenosis.

Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis.

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 post-operative 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.