Optimal initial palliation for patients with functionally univentricular hearts.

This review will outline the optimal, initial palliation for children who are born with a functionally univentricular heart. Optimizing the initial palliation is of critical importance in this patient population to prevent potential problems such as systemic outflow and pulmonary vein obstruction that may complicate further surgical intervention. The palliative techniques that are discussed include pulmonary artery banding, modified Blalock-Taussig shunt, Damus-Kaye-Stansel procedure, modified Norwood, hybrid, and early bidirectional Glenn.

Extracardiac versus intra-atrial lateral tunnel fontan: extracardiac is better.

This article was prepared to summarize the points made in a debate that the first author (C.L.B.

Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.

Background: Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution.

Methods: Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008.

Tracheal reconstruction in children with unilateral lung agenesis or severe hypoplasia.

Background: Infants with congenital tracheal stenosis may also have unilateral lung agenesis or severe lung hypoplasia. The purpose of this review is to evaluate our results with these patients and compare their presentations and outcomes to those of tracheal stenosis patients with two lungs.

Methods: Our database was queried for patients undergoing tracheal stenosis repair since 1982.

Nomenclature and databases for the surgical treatment of congenital cardiac disease--an updated primer and an analysis of opportunities for improvement.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up.

Arrhythmic complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A detailed hierarchal nomenclature of arrhythmias is offered with definition of its applications to diagnosis and complications. The conceptual and organizational approach to discussion of arrhythmias employs the following sequence: location--mechanism--aetiology--duration. The classification of arrhythmias is heuristically divided into an anatomical hierarchy: atrial, junctional, ventricular, or atrioventricular.

Late reoperations for Fontan patients: state of the art invited review.

Conversion of the atriopulmonary Fontan to a total cavopulmonary extracardiac connection with concomitant arrhythmia surgery and pacemaker placement is a safe and efficacious procedure for this patient population. From 1994 to 2007 a total of 118 patients have undergone this procedure with one (0.8%) early and nine (7.

Maze procedure in single ventricle patients.

As of September 2007, 120 patients have undergone a Fontan conversion procedure at Children's Memorial Hospital (Chicago, IL). One of the primary indications for surgery in these patients has been arrhythmias, either atrial reentry tachycardia or atrial fibrillation. The surgical treatment of those two lesions has been with the modified right atrial maze and the Cox-maze III.

What is the best technique for repair of complete atrioventricular canal?

Surgical management of pediatric patients with the diagnosis of complete atrioventricular canal (CAVC) is accomplished by using one of three procedures: (1) the classic single-patch, (2) two-patch, or (3) modified single-patch technique. Of these, the modified single-patch is currently the best technique available for repair of CAVC. The goal of this review is to describe our experience with the modified single-patch technique and explain how it evolved into our procedure of choice for repair of CAVC in our pediatric population.

Evolving anatomic and electrophysiologic considerations associated with Fontan conversion.

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them.

Atrioventricular valve procedures with repeat fontan operations: influence of valve pathology, ventricular function, and arrhythmias on outcome.

Background: The purpose of this study is to analyze atrioventricular valve procedures when performed in association with repeat Fontan operations and to determine the influence of atrioventricular valvar pathology, ventricular function, and arrhythmias on outcome.

Methods: Between December 1994 and August 2004, 80 patients had repeat Fontan operations that included arrhythmia surgery (78 of 80), venous pathway revision (78 of 80), atrioventricular valve repair-replacement (15 of 80), and other associated procedures. Mean ages were the following: at operation, 20.