AI Article Synopsis

  • Fontan palliation is a significant surgical advancement for patients with functionally univentricular physiology, leading to extended adult survival rates, though some may face challenges due to their anatomy or physiology.
  • The operation relies on passive blood flow to the lungs, which can complicate filling the systemic ventricle and contribute to issues like venous congestion, particularly during physical activity.
  • The review focuses on the importance of pulmonary artery development, biology, and pharmacologic strategies to improve pulmonary vascular function and overall health outcomes for these patients.

Article Abstract

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

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http://dx.doi.org/10.1017/S104795112100192XDOI Listing

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