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.

中文翻译：

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功能性单心室生理和 Fontan 循环患者的肺血管床

Fontan 姑息治疗代表了在治疗出生时具有功能性单心室生理学的个体中最显着的外科手术进展之一。该手术可确保除少数解剖学和/或生理学状况不佳的人外，其他所有人都能存活。生理学固有的是被动跨肺血流，它会在休息和运动期间产生对全身心室充分充盈的脆弱性。同样，肺部被动流动的上游影响是静脉充血和静脉高压，尤其是在体力活动期间。肺血管床已成为 Fontan 循环行为和临床结果阶段的决定性特征。因此，其药理调节和解剖康复似乎是重要的战略治疗目标。