A new diagnostic algorithm for assessment of patients with single ventricle before a Fontan operation

ObjectivesCardiac catheterization has a low diagnostic yield before a Fontan operation, and magnetic resonance imaging and computed tomography are reliable alternatives to invasive angiography. A new diagnostic algorithm to avoid cardiac catheterization in “low-risk” subjects before a Fontan operation is proposed.MethodsThe proposed algorithm would identify “high-risk” subjects on the basis of risk factors on medical history, echocardiography, and noninvasive angiography. The efficacy of this algorithm in screening for subjects deemed to be inoperable after catheterization was evaluated retrospectively in 151 children. For this analysis, results of conventional angiography (assumed to be equivalent to noninvasive angiography) were used.ResultsAccording to the algorithm, 95 (63%) of 151 subjects had no risk factors (“low risk”) whereas 56 (37%) of 151 had 1 risk factor or more (“high risk”). Nine (6%) of 151 subjects were found to be inoperable after catheterization and all 9 were correctly classified as high risk by the algorithm. In the 135 of 151 subjects who underwent a Fontan operation, the algorithm predicted an adverse postoperative outcome with a sensitivity of 51% and specificity of 78%. However, this prediction was not improved by including elevated pulmonary artery pressure or ventricular filling pressure as additional risk factors.ConclusionsThe proposed algorithm effectively screened for subjects who were deemed unsuitable for a Fontan procedure. In addition, omitting preoperative invasive hemodynamic assessment did not impair prediction of adverse postoperative outcomes. Prospective evaluation of such a noninvasive diagnostic strategy before the Fontan operation is warranted

Late Status of Fontan Patients With Persistent Surgical Fenestration

ObjectivesThis study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.BackgroundFenestrations are frequently performed during Fontan procedures, but late consequences are not well described.MethodsPatient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.9 years) enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. The status of the fenestration and the association of a currently patent fenestration with health status and measures of ventricular performance were investigated.ResultsFenestration was performed in 361 patients (67%), and frequency differed by year and center (p < 0.001 for each). After adjustment for center, age at Fontan, year of Fontan, and prior superior cavopulmonary surgery, the fenestrated group had shorter length of Fontan hospital stay. At the time of cross-sectional testing 8 ± 3 years after Fontan, the fenestration remained open in 19% of subjects. Among those with confirmed fenestration closure, 59% were by catheter intervention and 1% by surgical intervention, and 40% had apparent spontaneous closure. Compared with those without evidence of a fenestration, subjects with a current fenestration were taking more medications (p = 0.02) and had lower resting oxygen saturation (median 89% vs. 95%, p < 0.001). Functional health status, exercise performance, echocardiographic variables, prevalence of post-Fontan stroke or thrombosis, and growth did not differ by current fenestration status.ConclusionsSurgical fenestration is associated with well-demonstrated early post-operative benefits. This cross-sectional study found few associations between a persistent fenestration and deleterious later outcomes

Heart transplantation in children with congenital heart disease

ObjectivesThe aim of this study was to describe heart transplantation in children with congenital heart disease and to compare the results with those in children undergoing transplantation for other cardiac diseases.BackgroundReports describe decreased survival after heart transplantation in children with congenital heart disease compared with those with cardiomyopathy. However, transplantation is increasingly being considered in the surgical management of children with complex congenital heart disease. Present-day results from this group require reassessment.MethodsThe diagnoses, previous operations and indications for transplantation were characterized in children with congenital heart disease. Pretransplant course, graft ischemia time, posttransplant survival and outcome (rejection frequency, infection rate, length of hospital stay) were compared with those in children undergoing transplantation for other reasons (n = 47).ResultsThirty-seven children (mean [±SD] age 9 ± 6 years) with congenital heart disease underwent transplantation; 86% had undergone one or more previous operations. Repair of extracardiac defects at transplantation was necessary in 23 patients. Causes of death after transplantation were donor failure in two patients, surgical bleeding in two, pulmonary hemorrhage in one, infection in four, rejection in three and graft atherosclerosis in one. No difference in 1- and 5-year survival rates (70% vs. 77% and 64% vs. 65%, respectively), rejection frequency or length of hospital stay was seen between children with and without congenital heart disease. Cardiopulmonary bypass and donor ischemia time were significantly longer in patients with congenital heart disease. Serious infections were more common in children with than without congenital heart disease (13 of 37 vs. 6 of 47, respectively, p = 0.01).ConclusionsDespite the more complex cardiac surgery required at implantation and longer donor ischemic time, heart transplantation can be performed in children with complex congenital heart disease with success similar to that in patients with other cardiac diseases