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Primary Fontan, 1½ Ventricular Repair, and Fontan Conversion Operations in Adults

  • Constantine Mavroudis
  • Joseph A. Dearani
Chapter

Abstract

The Fontan operation has undergone many revisions since its introduction by Francis Fontan and Guillermo Kreutzer in 1968. Gone are the venous pathway valves, the right atrial connections to small subpulmonic ventricles, and atriopulmonary connections. The standard therapy for the functionally single-ventricle heart is determined by three distinct time periods. The first is the neonatal management of pulmonary artery flow (by systemic to pulmonary artery shunt, pulmonary artery band, or observation). At approximately 6 months of age, the second stage takes place: systemic to pulmonary artery shunt takedown or pulmonary artery band takedown and a bidirectional Glenn shunt that prepares the single ventricle by volume unloading, resulting in favorable ventricular remodeling. By 2–3 years of age, the Fontan operation is usually performed. Since the introduction of the atrial fenestration, some surgeons have used this obligatory right-to-left shunt in all cases; some surgeons have used it selectively, and others have not used it at all. Most surgeons now use the fenestration selectively, especially in cases with borderline ventricular function, small pulmonary arteries, abundant aortopulmonary artery collaterals, and single-lung physiology.

Keywords

Adult congenital heart surgery Fontan in adults 1½ ventricular repair Fontan conversion Fenestration Arrhythmia Maze procedure Bjork Modification 

Abbreviations

Ao

Aorta

APL

Atrial pacemaker lead

ASD

Atrial septal defect

ccTGA

Congenitally corrected transposition of the great arteries

CS

Coronary sinus

IVC

Inferior vena cava also known as inferior caval vein (ICV)

LAA

Left atrial appendage

LLPV

Left lower pulmonary vein

LPA

Left pulmonary artery

LSVC

Left superior vena cava or left superior caval vein

LUPV

Left upper pulmonary vein

LV

Left ventricle

MV

Mitral valve

PA

Pulmonary artery

PTFE

Polytetrafluoroethylene

RA

Right atrium

RLPV

Right lower pulmonary vein

RPA

Right pulmonary artery

RSVC

Right superior vena cava or right superior caval vein

RUPV

Right upper pulmonary vein

RV

Right ventricle

SVC

Superior vena cava also known as superior caval vein (SCV)

TEE

Transesophageal echocardiography

VSD

Ventricular septal defect

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Constantine Mavroudis
    • 1
  • Joseph A. Dearani
    • 2
  1. 1.Professor of SurgeryJohns Hopkins University School of Medicine, Johns Hopkins All Children’s HospitalSt. PetersburgUSA
  2. 2.Professor of Surgery, Department of Cardiac SurgeryMayo ClinicRochesterUSA

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