EditorialYoung and Free: Over 25 Years of Seminal Contributions to Complex Congenital Heart Disease From Australia & New Zealand
Introduction
In the second year of this journal, Timothy Cartmill (Figure 1a) provided a succinct history of the development of paediatric cardiac surgery, which had benefitted greatly from the contribution of local figures like Sir Brian Barratt-Boyes [1]. By the 1970s, three of the premier units in the world for the care of complex congenital heart disease (CHD) had been established in Australia and New Zealand (ANZ): in Auckland, Sydney and Melbourne. The tradition of multidisciplinary care established by these units – between dedicated surgeons, cardiologists, radiologists and anaesthetists – pervaded the region through generations of their trainees. The 1980s and 90s saw mortality for repair of most lesions in infancy and childhood fall to 2% overall [2]. Echocardiography and prenatal ultrasound enabled foetal detection and early accurate diagnosis, and the ready availability of prostaglandin ensured survival to transfer to tertiary referral centres. Once lesions were repaired, the technological developments available in intensive care allowed effective multi-organ support for even the smallest bodies. Cartmill concluded that, “It remains to understand the pathology more clearly, to develop the techniques … and to refine preoperative and postoperative care.” [1]
Teams in Australia and New Zealand have led too many clinical and research developments in the field of congenital heart disease in the last 25 years to list comprehensively. In this editorial, we chronicle some of the most influential and innovative work from the region. These achievements have led to a better understanding of the pathology and natural history after repair, more sophisticated techniques and better quality of care for the most high-risk groups of patients. Our region has become renowned around the world for its multi-disciplinary and multi-institutional collaborations, which are resulting in small improvements in already excellent outcomes.
Section snippets
The Causes and Inheritance of Congenital Heart Disease
Most congenital heart disease is known to develop through a complex interplay between genes and the environment [3]. Through international collaboration between basic scientists, clinicians and genetic counsellors, the team at The Children's Hospital at Westmead and the Victor Chang Research Institute in Sydney have made strides in defining the causes and inheritance of congenital heart disease. Basic science research has contributed to our understanding of normal cardiac development, and the
Nunn's Repair of Atrioventricular Septal Defect
Repair of atrioventricular septal defect (AVSD) has historically been complex and difficult to teach. In 1995, Graham Nunn described a simplified technique which allowed reproducible repair using a single patch, with applicability to most forms of AVSD. This technique has now become one of two standard approaches around the world for repairing AVSD, and is often referred to as the “Australian Technique” [20] (Figure 4).
Aortic Valve Repair
Myriad techniques exist for the treatment of neonatal and paediatric aortic
New Zealand's Commitment to Excellence in Clinical Care
Barratt-Boyes’ (Figure 1c) and Alan Kerr’s high standard of clinical achievement and technical development continues to be realised by the Green Lane team at Starship Children's Hospital in New Zealand. This is demonstrated in their world-leading published outcomes after the Norwood [26], arterial switch operation [27], Fontan conversion [28], [29], transplantation [30] and mitral valve repair [31]. Consistently, results in multi-institutional studies stand out as being better than the norm,
Disclosures
Yves d’Udekem is a consultant for companies MSD and Actelion.
Acknowledgements
The authors would like to thank Associate Professor Robert G Weintraub for his assistance in the drafting of this manuscript. Dr Yves d’Udekem is a Clinician Practitioner Fellow of the National Health and Medical Research Council of Australia (1082186).
References (42)
- et al.
Mutations in cardiac T-box factor gene TBX20 are associated with diverse cardiac pathologies, including defects of septation and valvulogenesis and cardiomyopathy
Am J Hum Genet
(2007) - et al.
Contribution of global rare copy-number variants to the risk of sporadic congenital heart disease
Am J Hum Genet
(2012) - et al.
Targeted next-generation sequencing identifies pathogenic variants in familial congenital heart disease
Journal of the American College of Cardiology
(2014) - et al.
Low risk of pulmonary valve implantation after a policy of transatrial repair of tetralogy of Fallot delayed beyond the neonatal period: the Melbourne experience over 25 years
Journal of the American College of Cardiology
(2014) - et al.
Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience
Ann Thorac Surg
(2012) - et al.
Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits
J Thorac Cardiovasc Surg
(2005) - et al.
Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance
Journal of the American College of Cardiology
(2012) - et al.
Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization
Journal of the American College of Cardiology
(2013) - et al.
Ventricular assist devices in pediatric cardiac surgery
Ats
(1995) - et al.
Centrifugal pump left heart assist in pediatric cardiac operations. Indication, technique, and results
J Thorac Cardiovasc Surg
(1991)