Hypoplastic left heart syndrome (HLHS) is a congenital anomaly that poses a serious threat to life immediately after birth. Without prompt intervention through a series of life-saving surgeries, infants with HLHS typically do not survive. In HLHS, the left ventricle fails to develop, which may be due to underlying issues like mitral or aortic valve stenosis or aortic hypoplasia. As a result, the right ventricle is forced to take on the entire job of pumping blood throughout the body. Despite the severity of this condition, there is still much we don’t fully understand about how HLHS affects heart function. One of the major challenges has been the lack of reliable large animal models that could allow us to study the condition in more detail.
One key aspect of heart health that has been under-explored in HLHS is coronary perfusion, the process by which blood vessels supply oxygen and nutrients to the heart muscle. Just as a lack of blood flow causes damage during a heart attack, chronic under-perfusion can lead to long-term damage in the heart tissue. This is particularly critical in HLHS, where the right ventricle is under extreme stress. In this study, we show for the first time how coronary capillary perfusion and growth are altered in a fetal univentricular heart model. Our findings shed light on how altered fetal coronary circulation may contribute to the unique physiology of infants with HLHS, and could help guide future strategies to improve heart function in these patients.
Find this paper in the Journal of Physiology and Pubmed.