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ItemOutcomes of Surgery for Congenital Anomalies of the Pulmonary CirculationYong, Shiung-En Matthew ( 2020)Congenital anomalies of the pulmonary veins and arteries are rare congenital cardiac defects. Without surgery, mortality during infancy is high with few children surviving to adulthood. Prognosis after surgery is improving with many children now surviving the initial operative period. Our understanding of the outcomes after surgery is evolving as emerging information from longitudinal studies have shed light on longer term survival of these patients. There is now a growing population of these patients surviving into adulthood. This project aimed at expanding our understanding of the prognosis and long-term outcomes of children who underwent surgery for partial and total anomalous pulmonary venous drainage (PAPVD and TAPVD). It has explored the entire spectrum of these patients and shows that excellent long-term survival and freedom from reoperation can be achieved in these patients into early adulthood. To better understand the long-term disease perceptions and impact, we conducted quality of life assessments on adult PAPVD and TAPVD patients. These patients were found to have similar quality of life outcomes compared to age-matched Australian population data. Furthermore, we examined the outcomes of surgery amongst TAPVD patients with a univentricular circulation. This group of patients have been rarely reported in the literature due to the extremely poor prognosis and limited experience even amongst the largest paediatric centres. We showed that these patients have a poor prognosis with high mortality. Future research into improving outcomes amongst these patients are needed. With the aid of a bi-national registry, we have reported a large cohort of TAPVD patients with a univentricular circulation who have reached Fontan completion. This has enabled better understanding of the composition, outcomes and risk factors for mortality of the population. Finally, to complete our study we addressed the long-term outcomes of the rare pulmonary artery sling condition. These patients often present in respiratory failure due to concomitant airway compromise and the need for airway surgery is associated with a higher mortality and morbidity. As part of a multi-centre study, we found that the overall results have improved in patients requiring concomitant airway surgery. Importantly, we found that at late follow-up, many of these patients had evidence of obstructive airway defects on lung function testing. Though currently asymptomatic, the long-term impact on functional status and respiratory function is unknown. As the early survival outcomes of these patients improve, our focus is now shifting towards understanding the long-term prognosis, functional impacts and quality of life. Enhanced knowledge in these areas will allow us to better inform families and develop strategies to, ultimately, improve the lives of the children and young adults with these diseases.
ItemOutcomes of the arterial switch operationFricke, Tyson Alexander ( 2020)The last few decades have seen great advances in the world of paediatric cardiac surgery. In particular, the arterial switch operation (ASO) is one of the specialty’s most impressive achievements. It is the procedure of choice for most children born with transposition of the great arteries (TGA). This project aimed to expand our understanding of the long-term outcomes and prognosis of patients who have had an ASO for TGA. The ultimate goal of this work was to not only increase clinicians’ knowledge of the long-term consequences of the ASO but also to allow cardiologists, cardiac surgeons and paediatricians to give the parents of patients, and patients themselves, more information about their future cardiac health. We aimed to achieve these goals by retrospectively reviewing patients who underwent an ASO at The Royal Children’s Hospital in Melbourne. This unit has performed the ASO for more than 30 years with a very low hospital mortality from the beginning. This allows for review of a large population of surviving patients from which long-term outcomes can be investigated, and makes possible the analysis of rarer subgroups of patients undergoing the ASO. We showed that the long-term outcomes of patients undergoing the ASO are generally good extending into young adulthood. However, reintervention is common and the incidence of clinically significant neo-aortic regurgitation or the need for neo-aortic root reoperation remains an issue for survivors with more than 25 years of follow-up. In regards to the subgroups examined, we showed that patients with intramural coronaries can be operated with excellent long-term outcomes using a simple, reproducible technique. Children who are less than 2.0 kilograms at time of ASO represent one of the highest mortality risk subgroups. Female adult survivors can undergo pregnancy with a low risk of maternal cardiac complications. Patients with associated aortic arch obstruction have a higher rate of reintervention for right sided obstruction and have a low rate of recurrent arch obstruction when avoiding the use of patch material. We also demonstrated that quality of life in adult ASO survivors was comparable to the general population using a validated quality of life questionnaire.