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The Fontan Operation: The Pursuit of Associated Lesions and Cumulative Trauma
Robert L. Hannan
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Jennifer A. Zabinsky
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Jane L. Salvaggio
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Anthony F. Rossi
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Danyal M. Khan
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Francisco A. Alonso
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Jorge W. Ojito
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David G. Nykanen
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Evan M. Zahn
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Redmond P. Burke
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D. G. Nykanen Department of Pediatric Cardiology, Arnold Palmer Hospital for Children
, Orlando,
FL, USA
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A. F. Rossi D. M. Khan E. M. Zahn Department of Cardiology, Congenital Heart Institute at Miami Children's Hospital
, 3100 SW 62nd Avenue, Miami,
FL 33155, USA
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R. L. Hannan (&) J. A. Zabinsky J. L. Salvaggio F. A. Alonso J. W. Ojito R. P. Burke Department of Cardiovascular Surgery, Congenital Heart Institute at Miami Children's Hospital
, 3100 SW 62nd Avenue, Miami,
FL 33155, USA
Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/ 137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit
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of residual lesions results in a significant number of
additional interventional catheterizations and operative
procedures but might have an important influence on long-term
survival after the Fontan procedure.
Approaches to single ventricle palliation have evolved
since the introduction of the Fontan operation in 1971 [4].
This evolution encompasses multiple technical and
anatomic challenges. The technical approach to the Fontan
operation itself has evolved into two major approaches: the
lateral tunnel technique [3] and the extracardiac technique
[9, 12]. Bidirectional cavopulmonary shunt as an interim
staging procedure [7] between initial palliation and Fontan
is now widely used. Fenestration in the Fontan pathway [1]
might reduce the risk of Fontan operation in some patients.
In addition to the technical evolution of the Fontan
procedure, the patients undergoing Fontan operation have
changed. Improved results in single ventricle palliation of
all types and especially of hypoplastic left heart syndrome
have changed the anatomic diagnosis of patients
undergoing Fontan procedure, including the increasing proportion
of patients with systemic right ventricles. The 10 Choussat
criteria [2] are rarely present in a modern Fontan patient.
In addition to technical changes in the operation and a
broadening of patients being offered the Fontan procedure,
other important evolutions in cardiac care have occurred. A
significant one is the improved ability to intervene on
intracardiac and extracardiac lesions in the cardiac
catheterization laboratory [11].
Our programmatic approach to the Fontan operation has
evolved since 1995. We now perform almost exclusively
extracardiac Fontan procedures using expanded
polytetrafluoroethylene grafts from the inferior vena cava to the
pulmonary arterysuperior vena caval anastomosis.
Fenestration is performed on a selective basis. Bidirectional
cavopulmonary shunts are performed on all patients who
are initially palliated as babies; some older children who do
not require initial palliation might undergo primary Fontan.
Since 2001, we have programmatically avoided deep
hypothermic circulatory arrest at all stages of single
ventricle palliation [5]. Both bidirectional cavopulmonary
anastomosis and Fontan operations are performed with the
heart warm and beating if possible. We aggressively
address associated lesions on a presumptive basis with
treatment either in the cardiac catheterization laboratory or
the operating room.
We retrospectively reviewed our single-institution
Fontan experience from November 1995 to January 2008 to
evaluate the evolution in our approach to the Fontan
operation, the outcome of our approach, and the intensity
of effort and trauma to patients required to achieve these
results.
Materials and Methods
The study was approved by the Western Institutional
Review Board on February 22, 2008, and individual
consent was waived. A retrospective study of 137 consecutive
patients who underwent a Fontan procedure at Miami
Childrens Hospital from November 1995 to January 2008
was conducted. All medical recor (...truncated)