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

Abstract

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.

Longitudinal development of obesity in the post-Fontan population

“…Hypoplastic left heart syndrome and tricuspid atresia were the most common anatomic lesions necessitating Fontan palliation, similar to other Fontan cohorts studied. 7 Table 2 summarizes mean BMI, height and weight Z-scores at each time point for the group as a whole and also broken down by gender and race. The percentage of patients in each BMI category at the time of Fontan and for the ensuing 5 years post surgery is shown in Figure 1.…”

State of the art of the Fontan strategy for treatment of univentricular heart disease

“…Twenty-year freedom of re-operation following TCPC procedures in recent eras ranges from 86 to 92% 56 . In older cohorts, higher re-operation rates have been reported 24 , 57 , 58 . The most common surgical re-intervention procedures, in order of incidence, are pacemaker implantation in 9 to 23% of patients 23 , 24 , Fontan revision or conversion in 3 to 18% of patients 9 , 24 , 59 , and atrioventricular (AV) valve repair in 1 to 14% of patients 24 , 59 , 60 .…”

“…Hemodynamically significant obstruction in the Fontan pathway may occur, most commonly in the left pulmonary artery 57 , 65 . In the absence of a prepulmonary pump, this can severely affect the Fontan circulation, and obstructions are routinely dilated or stented.…”

Late catheter interventions in hypoplastic left heart syndrome

“…It has been reported to occur in 6.6-14.6% of all patients who underwent a Fontan operation. [2][3][4] Clinical manifestations of obstruction can include ascites, pleural effusion, protein-losing enteropathy, desaturation, and low cardiac output.…”

“…5 The decrease in cross-sectional area of the conduit has been reported to be 14-17%, presumably because of neointimal growth within the conduit in the early post-operative period, although this did not result in significant haemodynamic changes. 2,3 Late obstruction can occur in 3% of patients, most commonly at the anastomosis of the inferior caval vein to the conduit 2 (Fig 1). This condition can be treated with balloon angioplasty and stent placement, which can obviate the need for surgical intervention and has been shown to relieve ascites, protein-losing enteropathy, and improve cardiac output.…”