Embryological determinants of right ventricular morphogenesis in pulmonary atresia: The role of bipartite and tripartite configurations

ABSTRACT

Pulmonary atresia (PA) is a complex congenital heart defect with marked morphological and clinical heterogeneity. Central to this variability is the embryological development of the right ventricle (RV) and its subcomponents. This review explores the embryological basis of RV development, emphasizing the bipartite and tripartite configurations and their clinical relevance in PA. Recent advances in human embryology, including high-resolution reconstructions from the Human Developmental Biology Resource and the work of Hikspoors et al. , provide an updated framework for understanding RV morphogenesis. We highlight how the disruption of ventricular partitioning, mural hypertrophy, and proximal and distal outflow malalignment contribute to the phenotypic spectrum of PA. Finally, we examine the impact of RV morphology on surgical strategy and long-term prognosis. Understanding PA as a failure of stage-specific RV morphogenesis provides a developmental basis for its clinical and surgical diversity.