MEDICAL SCIENCES
The adult congenital heart disease population continues to grow thanks to the success of their management in the paediatric age. This manuscript will briefly review the special characteristic of this patient population when they progress into end stage disease and require treatment in the form of heart transplantation. Juan M Ortega-Legaspi, Cardiologist, Penn Medicine
Cardiac Transplantation in the Adult Congenital Heart Disease Population H
eart transplantation has become an effective therapy for patients with end stage heart failure. The success rate has now been translated to a median survival of close to eleven years. Considering patients with end stage heart failure have a mortality that is close to 100 per cent at one year, this is a fabulous achievement from science and medicine. It must also be noted that heart transplantation has had reasonable stable volumes for many years with less procedures than needed. It has only had a steady slight increase from 1993 to 2004 reaching an all-time high in 2015 with just over five thousand operations done worldwide. Not only does the heart failure population continue to grow overall, making heart transplant an even more precious resource, but there is also a patient population that has increased at an even higher proportion. This population is that of the patients with adult congenital
40
A SI A N H O S P I T A L & H EA LT HCA R E M A N AGE M E N T
heart disease who currently only represent approximately 3 per cent of the heart transplant operations done. Nevertheless, while that 3 per cent seems small, it represents a 40 per cent increase compared to the previous decade. This population is therefore likely to need wider representation in the near future. Congenital heart disease is the most common cause of birth defects. The fact that this patient population is increasing is testament to the success of the surgical and medical management of the paediatric population. In the not too distant past, the more complex patients within this group of disorders used to end their lives in childhood. Thanks to breakthrough science, technology and medical improvements, the expected survival of these patients has improved dramatically. This has led to formal adult congenital heart disease programs that now care for these patients as they grow older.
IS S UE - 54, 2021
As these patients grow older, they continue to require intense care. While it is true that the simpler and more common lesions such as an atrial septal defect rarely progress to end stage heart failure, the more complex cases such as those with tetralogy of Fallot, systemic right ventricle or the myriad of conditions that end up in single ventricle physiology do tend to continue to decline. Very notably, the main causes of death of the complex congenital heart disease population are by far due to cardiac reasons. Furthermore, within those potential cardiac causes of death which can include sudden cardiac death, ischemic heart disease or peri-procedural complications, the main cause is not the aforementioned but, indeed, end stage heart failure. If one keeps in mind that continued decline and refractory class IV symptoms are an indication for advanced therapies, such as cardiac transplantation, one can only expect that the number
