Adult congenital heart disease: Past, present and future

Abstract

The challenge is great; the rewards are enormous. For me there have been few things in life which have been more satisfying than to face a small child, struggling for his very existence, to perform some corrective surgical maneuver, and later to see the youngster, thriving and healthy, starting out in life, sound in body and mind.Robert Edward Gross 'father of cardiac surgery' | INTRODUC TI ONThe diagnosis and management of congenital heart disease (CHD), the most common inborn and global defect, has been a tremendous success story of modern medicine. Back in the 1950s, survival of children born with CHD was only approximately 15%. Thanks to remarkable advances in paediatric cardiology, cardiac surgery and catheter interventions, including radical and innovative procedures such as atrial/arterial switch for transposition of great arteries (TGA), the Fontan operation for "single ventricle", percutaneous pulmonary valve implantation and others, more than 90% of children survive now well into adulthood ( Figure 1). 1 Residual and progressive haemodynamic lesions, exercise intolerance, arrhythmias, heart failure (HF) and premature death, however, afflict many patients. 2 Beyond CHD, adult patients face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non-cardiac pathology, necessitating integrated care and most if not all medical disciplines. Furthermore, there is a pressing need to understand better the late pathophysiology of CHD and provide evidence regarding drug therapy, devices and transplantation. 3 We are truly faced with a "tsunami" in terms of adult CHD numbers, disease heterogeneity and complexity of work and intervention/s needed. We must work together, beyond age and geographic boundaries to provide

Cardiac catheter intervention complexity and safety outcomes in adult congenital heart disease

“…Cardiac catheterisation for CHD is one of the key areas where major advances have taken place. Evolving from being a diagnostic tool to becoming a therapeutic option for many conditions previously requiring cardiac surgery,1 catheter interventions are now an integral and indispensable component of the treatment of paediatric and adult CHD, both native and postsurgical.…”

Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations

“…We acknowledge that we will not receive all the hearts from adults dying suddenly with congenital heart disease from around the United Kingdom. This is primarily because some may not be autopsied, as the cause of death is simply designated as “congenital heart disease.” Despite this, our analysis highlights that a specific cause of death may be identified through autopsy, helping the families, as well as, the cardiologists and surgeons who manage the increasing population of adults with congenitally malformed hearts (Brida and Gatzoulis, ).…”

Adult congenital heart care in the COVID-19 era, and beyond: A call for action

“…We have argued for such an advanced model for some time. [ 22 ] The Covid pandemic has led, of necessity, to the destruction of the previous, outdated model, consisting of bringing patients to outpatient clinics at pre-set intervals, submitting them to prognostic investigations, trying to co-ordinate the results of these, then reaching consensus on best action plans, communicating these plans with the patient before eventually effecting them, clearly a slow and frustrating route for the patients and for us, the providers. We are right now faced with a unique opportunity to build something much better and more 21 st Century.…”