Multimodality hemodynamic evaluation for optimizing management of anomalous origin of the right pulmonary artery from the aorta in an adolescent

Abstract

The hemodynamics of anomalous origin of the pulmonary artery (PA) from the aorta is challenging. Different sources of blood supply to the lungs lead to a unique state of differential flow, pressure, and pulmonary vascular resistance in each lung. The decision for surgical reimplantation of the anomalous PA during infancy is easy. The assessment of operability beyond infancy, however, is perplexing. In this report, we describe stepwise multimodal hemodynamic evaluation and successful surgical management in a 15-year-old boy with an isolated anomalous origin of the right PA from the aorta. We also report 5-year hemodynamic data confirming sustained benefit over the long term, thus providing much-needed clinical validation of often cited Poiseuille’s and Ohm’s laws.

Anomalous origin of branch pulmonary artery from the aorta: Current challenges in the management

“…[ 7 ] In doubtful cases, this can be done to assess the flows with immediate pressure measurement in the cardiac catheterization lab to derive PVR in each lung bed. [ 8 ] It may be assumed that the surgical re-implantation would then reduce the overall PVR, based on Ohm’s law (Total PVR = 1/[1/PVR Right + 1/PVR Left ]). [ 8 ] Due to the complexity of shifting these sick patients between the cardiac catheterization and MRI suite to obtain near-simultaneous measurements, such an exercise is not routinely carried out.…”