Outcomes of Surgery for Congenital Anomalies of the Pulmonary Circulation
AuthorYong, Shiung-En Matthew
Document TypePhD thesis
Access StatusOpen Access
© 2020 Shiung-En Matthew Yong
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.
KeywordsCongenital heart disease; Anomalies of the pulmonary veins and arteries; Long-term outcomes of surgery
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