Sudden cardiac death and late arrhythmias after the Fontan operation.

Objectives: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.

Background: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.

Methods: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution.

Heart transplantation after Fontan: Results from a surgical Fontan cohort.

We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.

Liver Disease in Patients After the Fontan Operation.

We reviewed records of all patients with an initial Fontan operation or revision from 1973 to 2012 at our institution (n = 1,138); 195 patients had postoperative liver data available. Cirrhosis was identified by histopathology or characteristic findings on imaging with an associated diagnosis of cirrhosis by a hepatologist. Of 195 patients with biopsy or imaging, 10-, 20-, and 30-year freedom from cirrhosis was 99%, 94%, and 57%, respectively.

Ventricular fibrillation after elective surgery in an adolescent with long QT syndrome.

Congenital long QT syndrome (LQTS) is a potentially lethal but highly treatable channelopathy. Along with multiple risk reduction measures, a recommendation for left sympathetic cardiac denervation therapy and/or implantable cardioverter defibrillator is made for higher risk patients. Despite its relatively common incidence in paediatric patients, there are no formal recommendations regarding perioperative management and discharge criteria for LQTS patients undergoing ambulatory surgery.

40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients.

Background: There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation.

Objectives: This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic.

Methods: Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed.

Contraception Practices and Pregnancy Outcome in Patients after Fontan Operation.

Objective: The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation.

Design: We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database.

Automated external defibrillator rescues among children with diagnosed and treated long QT syndrome.

Background: Long QT syndrome (LQTS) is a potentially lethal yet highly treatable cardiac channelopathy. A comprehensive LQTS-directed treatment program often includes an automated external defibrillator (AED).

Objective: The purpose of this study was to determine the incidence of AED rescues among children evaluated, risk-stratified, and treated in an LQTS specialty center.

Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience.

Objective: Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly.

Empiric treatment of protracted idiopathic purpura fulminans in an infant: a case report and review of the literature.

Introduction: Idiopathic purpura fulminans is a cutaneous thrombotic disorder usually caused by autoimmune-mediated protein C or S deficiency. This disorder typically presents with purpura and petechiae that eventually slowly or rapidly coalesce into extensive, necrotic eschars on the extremities. We present the first known case of idiopathic purpura fulminans consistent with prior clinical presentations in the setting of a prothrombotic genetic mutation, but without hallmark biochemical evidence of protein C or protein S deficiency.

Sudden infant death syndrome-associated mutations in the sodium channel beta subunits.

Background: Approximately 10% of sudden infant death syndrome (SIDS) cases may stem from potentially lethal cardiac channelopathies, with approximately half of channelopathic SIDS involving the Na(V)1.5 cardiac sodium channel. Recently, Na(V) beta subunits have been implicated in various cardiac arrhythmias.