The Fontan Operation: The Pursuit of Associated Lesions and Cumulative Trauma

Pediatric Cardiology, Apr 2011

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 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.

A PDF file should load here. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a PDF plug-in installed and enabled in your browser.

Alternatively, you can download the file locally and open with any standalone PDF reader:

https://link.springer.com/content/pdf/10.1007%2Fs00246-011-9973-0.pdf

The Fontan Operation: The Pursuit of Associated Lesions and Cumulative Trauma

Robert L. Hannan 0 1 2 Jennifer A. Zabinsky 0 1 2 Jane L. Salvaggio 0 1 2 Anthony F. Rossi 0 1 2 Danyal M. Khan 0 1 2 Francisco A. Alonso 0 1 2 Jorge W. Ojito 0 1 2 David G. Nykanen 0 1 2 Evan M. Zahn 0 1 2 Redmond P. Burke 0 1 2 0 D. G. Nykanen Department of Pediatric Cardiology, Arnold Palmer Hospital for Children , Orlando, FL, USA 1 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 2 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 - 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)


This is a preview of a remote PDF: https://link.springer.com/content/pdf/10.1007%2Fs00246-011-9973-0.pdf

Robert L. Hannan, Jennifer A. Zabinsky, Jane L. Salvaggio, Anthony F. Rossi, Danyal M. Khan, Francisco A. Alonso, Jorge W. Ojito, David G. Nykanen, Evan M. Zahn, Redmond P. Burke. The Fontan Operation: The Pursuit of Associated Lesions and Cumulative Trauma, Pediatric Cardiology, 2011, pp. 778-784, Volume 32, Issue 6, DOI: 10.1007/s00246-011-9973-0