WRITTEN BY HITESHI PATEL

At present, around 85% of children with congenital heart disease reach adulthood thanks to various drugs, surgeries, and heart transplants. Depending on the case, however, overcoming the disease itself is not the hardest part of the process. It is extremely likely the patient’s cardiopulmonary function will decrease from their previous procedures; this can lead to advanced heart failure. Many researchers are currently exploring this weakening and how to minimize it, however, a definitive solution does not seem likely in the near future. 

The case is slightly different for adults that acquire heart disease and progress to heart failure, as there are options available for treatment. For children, on the other hand, anatomic and physiological factors greatly restrict the number of available treatments. For instance, mechanical circulatory support or cardiac transplants are not feasible treatments for heart failure, as they will likely be too intense for a child’s body to handle. This can then lead to organ rejection or failure of other bodily systems such as ventricular dysfunction or pulmonary hypertension, which should be avoided. Hence, physicians are now resorting to heart failure therapies as a solution to this issue. 

In order to consider these therapies, registries like the “United Network for Organ Sharing Status 4 for ACHD in New Allocation System” are now used as databases for determining things like risk factors for certain therapies and strategies for end-organ function. Often, patients will start with invasive procedures to correct their hemodynamics, or blood flow, which maintains adequate tissue perfusion and alerts before an impending cardiovascular crisis. However, this is a short-term solution to sustain the patient while therapies or surgeries are performed as more permanent solutions. If the condition continues to worsen, another transplant can be considered, although it is rarely performed. 

These new procedures and therapies have been implemented to standardize heart failure treatment and help those patients where traditional treatments are not possible. With future research in the coming years, these registries will be updated to provide an even greater standard of care for all patients. 

Givertz, M. M., DeFilippis, E. M., Pinney, S. P., Woods, R. K., & Valente, A. M. (2019). Advanced Heart Failure Therapies for Adults With Congenital Heart Disease: JACC State-of-the-Art Review. Journal of the American College of Cardiology, 74(18), 2295–2312. doi: https://doi.org/10.1016/j.jacc.2019.09.004