1. Reverse order of staging in single ventricle palliation
out of necessity: Southern Glenn followed a
Northern Fontan
A. Dodge-Khatami, MD, PhD
Chief of Pediatric and Congenital Heart Surgery
Children’s Heart Center
Professor of Surgery, University of Mississippi Medical Center
Jackson, MS, USA
2. Introduction: from animal models to surgical norms
• 1950-58: Dr. Carlon/Italy, Dr. Meshalkin/Russia, Dr.
Glenn/Yale had no canine survivors of the IVC-PA
connection but success using the SVC. >>
historical preference for standard palliation in
single ventricle physiology to include a bi-
directional Glenn with the SVC-PA anastomosis.
• however, with unfavorable upper body systemic
venous anatomy (SVC thrombosis/stenosis),
performing single ventricle staging in the usual
manner with an initial superior vena cava-PA
connection may be precluded or hazardous.
• if a standard SVC-PA Glenn is deemed a poor
option, the suboptimal alternative is to leave the
patient with shunt-dependent/banded physiology.
• we report a case of reverse staging out of
necessity, namely performing a primary inferior
cavo-pulmonary (IVC-PA) connection, followed by
completion Fontan from above, after adequate SVC
growth.
3. Case Presentation
• in a 6-month old infant with a very small SVC and
thrombosed innominate vein, a primary extracardiac
(Ø 14mm) inferior cavo-pulmonary connection
(“Southern” Glenn) was performed.
• early post-operative extubation, standard low-dose iv.
heparin (10U/kg/h) was transitioned to Aspirin.
• uneventful ICU stay (3 days), removal of chest drains
and discharge to home on post-operative day 8.
• 3 month angiogram and CT scan: open IVC-PA
connection, no veno-venous collaterals, and no
hepatic venous congestion: victory?
• unknown outcome: ”Longer follow-up is warranted …
whether it leads to an unplanned inter-stage
reoperation, will be an ideal set-up for future Fontan
completion with the SVC, or results in a final palliative
stage not needing any further intervention…”
Dodge-Khatami et al. When the bidirectional Glenn is an unfavorable
option: primary extracardiac inferior cavopulmonary connection as an
alternative palliation. Cardiol Young 2016;26:1247-9
4. Case Presentation
• sixteen months later, increasing cyanosis led to the
discovery of a veno-venous collateral that was
coiled
• an SVC that had grown to larger-than-normal size.
• pre-Fontan angiogram showing the primary IVC-PA
connection (“Southern Glenn”).
• despite the coiled collateral, persisting cyanosis led
us to plan a fenestrated completion Fontan:
– mean PA 15mmHg
– PVR 1.47 Wood/m2.
5. Surgery
• at 22 months of age, completion Fontan = standard
“Northern” bi-directional cavo-pulmonary
connection with the superior vena cava, and
extracardiac fenestration, was performed.
• intra-operative picture prior to cannulation showing
the larger-than-normal superior vena cava
(remember the reverse IVC-PA staging was done
owing to an initially unusable small SVC)
• the cephalad opening into the PA which will
accommodate the SVC anastomosis.
• uneventful hospital course, 10-day intensive care
stay requiring iNO, and was discharged to home on
day 17, with 1-liter supplemental oxygen and
empirical sildenafil.
• current 8 months post-operative follow-up: he (2 ½
years old) is thriving at home, fully saturated (with
spontaneous fenestration closure) allowing
discontinuation from oxygen.
6. Discussion
• reverse geographical staging, namely initial partial right heart
bypass from below (South), followed by complete right heart bypass
from above (North), is feasible in humans, despite it never working
in a canine/animal model.
• if faced with unfavorable anatomical conditions for a bi-directional
Glenn, reversing the staging order from North to South, namely
performing a primary IVC-PA connection, allows earlier ventricular
unloading rather than indefinitely leaving a volume-loaded heart
with shunt-dependent physiology, or a pressure-loaded heart with a
PA band.
• in our patient, following primary IVC-PA connection, altered
systemic venous flow patterns or decompressing veno-venous
collaterals led to impressive growth of a previously diminutive SVC,
allowing completion Fontan with the SVC at 22 months of age, and a
good clinical outcome.
• in complex shunt-dependent or banded single ventricle patients,
increased experience with reversed staging out of necessity may
encourage future protocol flexibility (2nd
patient planned for
completion Northern Fontan).
7. Ponderings on Innovation / Courage
• 1958 - Glenn: no canine survivors of the IVC-PA
connection but success with the SVC, leading to
systematic application of the SVC-PA palliation in
humans as we know it today.
• 1968 - Fontan/Kreutzer: no canine survivors of
total right heart bypass; the concept was still
attempted out of desperation in humans,
succeeded, and is now the routine surgical norm.
• animal models don’t necessarily apply to
human physiology, or vice versa…
• 1954 - in the context of 7/14 deaths for VSD
closure using cross-circulation, and then the first
AVSD and TOF repair, “Cardiologist Helen Taussig,
of blue-baby operation fame, also condemned
Lillehei. Learning of his one success with
tetralogy of Fallot, Taussig said, Too bad, now
he’ll continue.”
King of Hearts: the true story of the
Maverick who pioneered open heart
surgery. G. Wayne Miller
