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The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation

Published online by Cambridge University Press:  11 August 2021

Ahmed Krimly ,
C. Charles Jain ,
Alexander Egbe Open the ORCID record for Alexander Egbe [Opens in a new window] ,
Ahmed Alzahrani ,
Khalid Al Najashi ,
Dimpna Albert-Brotons Open the ORCID record for Dimpna Albert-Brotons [Opens in a new window]  and
Gruschen R. Veldtman Open the ORCID record for Gruschen R. Veldtman [Opens in a new window]
Ahmed Krimly
Affiliation:
King Faisal Cardiac Center, National Guard Health Affairs, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia
C. Charles Jain
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA
Alexander Egbe
Affiliation:
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA
Ahmed Alzahrani
Affiliation:
Department of Pediatric Cardiology, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia
Khalid Al Najashi
Affiliation:
Department of Pediatric Cardiology, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia
Dimpna Albert-Brotons
Affiliation:
Heart Centre, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
Gruschen R. Veldtman*
Affiliation:
Scottish Adult Congenital Cardiac Service, Golden Jubilee Hospital, Glasgow, Scotland, UK
*
Author for correspondence: Dr Gruschen R. Veldtman, Scottish Adult Congenital Cardiac Service Golden Jubilee Hospital, Glasgow Agamemnon Street, G81 4DY, Glasgow, UK. Tel: +44(0)1419515390; Fax: +44(0)1419515948. E-mail: Gruschen@me.com
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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.

Keywords

Fontan operationsingle ventricle physiologypulmonary arteryvascular biologyFontanpulmonary vascular resistancepulmonary vasodilatorstricuspid atresiaFontan procedureheart defectslong-term outcomesuniventricular heartsingle-centre experience
Type
Review
Information
Cardiology in the Young , Volume 31 , Issue 8 , August 2021 , pp. 1241 - 1250
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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