In situ pericardial extracardiac lateral tunnel Fontan operation: fifteen-year experience.

BACKGROUND: The study purpose is to evaluate the long-term outcome of the in situ pericardial extracardiac lateral tunnel Fontan operation. METHODS: From June 1994 to August 2009, 160 patients with single ventricle (boys, n = 96, 60%, median age = 39 months, mean weight 15.5 kg) underwent the pedicled pericardial extracardiac lateral tunnel operation. Patients' charts were reviewed for perioperative and long-term follow-up data, outcome, and mortality. The potential growth of these tunnels was evaluated. RESULTS: The main diagnoses included tricuspid atresia (n = 44, 27%); double-outlet right ventricle (n = 29, 18%), and hypoplastic left heart syndrome (n = 26, 16%). The mean follow-up was 6.5 ± 3.7 years (range: 0.1-15 years). There were 2 (1.3%) operative and 6 (3.7%) late deaths. Actuarial survival at 14 years was 93%. Early complications included prolonged effusions (n = 35, 22%), chylothorax (n = 5, 3.1%), readmissions (n = 35, 22%), cerebrovascular accidents (n = 8, 5%), contralateral phrenic nerve palsy (n = 1, 0.8%), and transient arrhythmias (n = 5, 3.1%). No pacemaker was needed. Late complications included tunnel stenosis (n = 3, 1.8%) managed with balloon dilatation and stenting in 2 patients and surgical revision in 1; tunnel thrombosis (n = 2, 1.2%) causing death in both patients; and protein losing-enteropathy (n = 4, 2.5%). Follow-up echocardiography of 10 patients showed laminar flow, no turbulence/gradient at the inferior vena cava and mid-tunnel levels. The diameter indexed to body surface area showed growth, reduction, or no change depending on flow demands. CONCLUSIONS: The construction of the extracardiac lateral tunnel Fontan conduit using viable pedicled pericardium is a relatively simple, durable, and safe operation. Long-term follow-up confirms low morbidity and mortality. Fenestration is unnecessary in most patients. This viable tunnel adapts to physiologic flow demands.

Scoring system to determine need for balloon atrial septostomy for restrictive interatrial communication in infants with hypoplastic left heart syndrome.

BACKGROUND: Restrictive interatrial communication (IAC) causes morbidity and mortality in infants with hypoplastic left heart syndrome awaiting cardiac transplantation. We sought to create a scoring system, based on echocardiographic and clinical findings, to serve as a guide for determining the need for balloon atrial septostomy (BAS). METHODS: We retrospectively reviewed echocardiograms of 44 infants with hypoplastic left heart syndrome. Infants were studied from the time of admission to the final end-point of transplantation, Norwood procedure, or death. Seventeen infants underwent BAS for clinical indications of oxygen saturation <80% in room air. Data collected included age at BAS, maximum velocity (V(max)), and IAC diameter throughout the clinical course. We assigned higher IAC scores to smaller IAC diameter, greater V(max) through the IAC, and lower oxygen saturation value. The minimum score was 3, and the maximum score was 9. RESULTS: Only 10% of infants with a score <6 at presentation required BAS, whereas 67% of those with scores > or =6 required BAS. Higher IAC scores at presentation were associated with earlier need for BAS (p = 0.04). CONCLUSIONS: The IAC scoring system can serve as a reliable clinical guide for identifying infants with hypoplastic left heart syndrome who are likely to require BAS for relief of critically restrictive IAC while awaiting cardiac transplantation.

Moderate acute rejection detected during annual catheterization in pediatric heart transplant recipients.

BACKGROUND: Acute rejection commonly occurs within the first year after heart transplantation, and then decreases in frequency with time. Recently, the long-term utility of endomyocardial biopsy during routine annual catheterization has been questioned. The purpose of this study was to retrospectively review the prevalence of biopsy-proven rejection during routine annual catheterization in our patient population, determine whether biopsies late after transplant are useful, and identify factors that correlate with late unsuspected rejection. METHODS: Biopsy results from the annual catheterization were evaluated from 1986 to August 2000. The prevalence of moderate rejection was evaluated and compared with the patient's immunosuppressive regimen; the prevalence of late rejection; and how late rejection correlated with recipient age, number of first-year rejections and presence of sub-therapeutic cyclosporine. RESULTS: A total of 1108 biopsies were performed in 269 children with a mean follow-up of 5 +/- 3 years (median 5 years, range 1 to 11 years). Three-drug immunosuppressive therapy, including steroids, was used in 93 patients. There was a persistent 8% to 10% prevalence of moderate rejection at up to 10 years post-transplantation. Moderate rejection was more likely in patients: (1). on 3-drug immunosuppressive therapy; (2). with a recipient age >1 year; and (3). with a relatively lower cyclosporine level. CONCLUSIONS: These data suggest that continued surveillance of pediatric transplant patients for acute rejection is indicated for long-term follow-up.

Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy.

Surgical mortality is high in children with visceral heterotaxy (VH), particularly if atrioventricular valve insufficiency, ventricular dysfunction, or aortic atresia is present. This study reviews the outcome of cardiac transplantation (CT) in infants and children with VH and congenital heart disease who are at high risk for standard palliative or corrective surgery. We reviewed CT outcomes in 29 children with VH, congenital heart disease, atrioventricular valve insufficiency, ventricular dysfunction, and/or aortic atresia. Median age at CT was 3.1 years. Cardiac surgery had been performed in 20 patients (69%) before CT. Follow-up since CT has been 8.5 +/- 2.2 years. Outcomes were compared with 45 children who underwent transplantation for dilated cardiomyopathy. Actuarial graft survival in the VH group at 30 days and 1, 5, and 10 years was 100%, 86%, 68%, and 50%, respectively, compared with 100%, 96%, 83%, and 68% in children who underwent transplantation for dilated cardiomyopathy (p = 0.12). Splenic status, cardiac position, age at CT, number of prior cardiac surgeries, or systemic venous anomalies were not predictors of mortality after CT. Cardiopulmonary bypass and graft ischemic times were longer in the VH group; time on the ventilator after CT, length of hospitalization, and rejection, infection, post-transplant lymphoproliferative disease, and transplant coronary artery disease rates were equal. Thus, CT is a viable alternative therapy for high-risk patients with VH, possibly offering improved survival over standard surgical management.