Franklin, Rodney C.G.; (1997) The fate, survival and suitability for definitive surgery of infants with double inlet ventricle and tricuspid atresia. Doctoral thesis (Ph.D.), University College London (United Kingdom).

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Abstract

Definitive surgery by Fontan-type surgery is regarded as optimal long term treatment for children with functionally a univentricular heart, such as double inlet ventricle and tricuspid atresia. Most patients with such complex congenital heart disease require palliative surgery to survive beyond infancy with suitable anatomy and physiology for definitive surgery. The influences on these parameters of different management strategies undertaken in infancy are crucial, but have not been studied in an unselected population in the Fontan era. This thesis addresses these issues and evaluates the effectiveness of management strategies in achieving suitability for a successful definitive repair. A retrospective study was undertaken by examining patient records of 428 unselected consecutive infants (1972-87): 191 with double inlet ventricle and 237 with tricuspid atresia. The previously unknown full morphological spectrum of the two lesions when presenting in infancy are detailed. Time related survival for the patient sub-groups using different management strategies were determined and compared. Risk factor analyses gave relative risks for survival and suitability for definitive surgery. These analyses found that the more complex the lesion, the worse the outcome, particularly for those with systemic outflow obstruction. Based on these past patients, the methodology uniquely enabled the estimation of future survival and/or suitability for definitive repair, as determined by a new individual patient's morphological and physiological sub-group at presentation. Patients with aortic arch obstruction inevitably developed subaortic stenosis after conventional palliation. Justifying the use of higher risk primary palliation, whilst isolated banding of the pulmonary trunk provided good palliation for those with the same morphological substrate without an arch lesion. The results and extensive literature review support undertaking definitive surgery during early childhood, to balance the risks of young age against the development of adverse risk factors to a successful repair. Conclusions and recommendations for the future management of these children are made.

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