The right ventricle: the Achilles heel in congenital heart disease?

Abstract

The life expectancy of people with a congenital heart defect (CHD) has improved significantly over the past decades. As a result, focus is shifting to long-term outcomes, including residual lesions as a result of surgery or heart catheterization. Common problems include narrowing of the pulmonary artery or leakage of the pulmonary valve, which can lead to right ventricular (RV) pressure or volume overload. The extent to which the RV can adapt to this is crucial for patients’ symptoms and survival. When the RV fails to adapt properly, it can result in permanent damage. Because effective medications for RV failure are limited, early surgical or catheter-based interventions are often required. Timely identification of this maladaptation is therefore crucial to prevent irreversible deterioration. This dissertation seeks to enhance our understanding of RV adaptation and function in patients with CHD. The results show that conventional imaging is not always sensitive enough to detect early RV dysfunction. Since indications for interventions are currently based mainly on robust parameters and values that are load-dependent, there is a risk that early damage is overlooked. Pressure-volume loop analysis provide a more advanced understanding of how the RV responds to pressure and volume overload, offering valuable insights in addition to conventional imaging. Future research should build on these techniques, along with stress testing, to better characterize RV adaptation and determine the optimal timing for intervention.