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Challenges in Congenital Cardiology

Challenges in Congenital Cardiology

Perhaps the greatest challenge in the field of congenital heart disease (CHD) has arisen, rather counterintuitively, as a result of greatly improved care.  The dramatic improvements in surgical outcomes for CHD have revolutionised the field, leading to greatly improved survival and quality of life for children and young adults with CHD but also a cohort of patients sailing rapidly into uncharted waters. Enabling these patients to live long, symptom-free lives poses a number of challenges.

As patients live longer with complex cardiac abnormalities, even after surgical correction, their hearts are under significant physiological stress when compared to the general population. As such it is unsurprising that these patients are at risk of developing cardiac complications such as heart failure. Take, for example, a patient who has previously had a repair for Tetralogy of Fallot; although the repair is likely to have corrected the underlying right ventricular outflow tract obstruction, there is a significant risk of subsequent moderate to severe pulmonary regurgitation. In this case the right ventricular is exposed to volume overload for an extended period of time, leading to right heart failure. 

Managing heart failure in this context is not a straightforward task and the standard approaches, which have been shown to reduce mortality in the context of left ventricular failure in a non-CHD patient (e.g. beta blockers and ACE inhibitors), are not effective. Although the reasons for this discrepancy are not yet entirely clear; it may be related to the differing underlying causes in combination with intrinsic differences between the left and right ventricles. Developing effective strategies to better manage these patients appears to remain some way off. 

The potential issues linked to patients with CHD surviving for long periods of time are not limited to potential future medical complications but also to the way in which care for these patients is provided. As it stands, children with CHD are under the care of paediatric cardiologists who provide long term specialised care. Due to the increased survival following surgery for CHD, including for complex conditions, the demands on these services will increase as a result of both an increase in the numbers of patients requiring long term follow up and an increase in complexity of the patients being cared for. In addition, as patients with CHD survive for longer they become increasingly likely to acquire other medical problems, thus further complicating the management of their conditions and potentially resulting in situations of which the physician has only limited experience.

In order to manage this increased demandm it is likely that either general physicians, including paediatricians and GPs, will be asked to become more involved in the care of these patients or the number of CHD specialists will need to increase. For the former, maintaining the same standard of care as is currently achieved would require significant specialised training for all general paediatricians. This would in theory be possible but could be challenging given that paediatrics is already a relatively understaffed specialty, therefore expecting the general paediatricians to take on additional responsibilities may not be a practical solution in the current climate. The latter option would maintain the standard of care however it would be expensive to train and employ more staff.

It is indisputable that congenital cardiology is facing some major challenges as the patient mix changes due to improvements in surgery, becoming increasingly older with long-term complications from CHD. These challenges do not necessarily require a complete change in strategy but do require some alterations to optimise future care, in particular, planning staffing and services to meet the future needs of this patient cohort and educating general physicians in order to enable to improve the care of CHD patients across the board, including in the community. Undoubtedly future clinical trials and scientific research are required to understand the underlying pathogenesis of these complications in the context of CHD and develop effective treatments for the complications of CHD, such as right ventricular failure. The ideal outcome would be the development of treatments which enable patients to life a full life with minimal symptoms despite CHD. There is no harm in aiming high after all. 

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