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In this descriptive analysis, data in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database pertaining to patients who underwent reoperative cardiac surgery were analyzed. Practice patterns and outcomes are described. Reoperative cardiac surgery for congenital heart disease is common, with one third of index operations in the database occurring subsequent to prior cardiothoracic operation(s) performed on cardiopulmonary bypass. This analysis suggests that a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, but that patients with greater number of previous operations appear to be at higher risk., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Management of the ventricular septal defect at the time of the double switch operation can be challenging and deserves careful attention to the anatomic details of ventricular septal defect location, alignment with the appropriate subtended ventricles and their respective atrioventricular valves, and the disposition of the specialized conduction tissue. These anatomic details can differ considerably from hearts with concordant atrioventricular alignments, and further variations of right-left geometry and septal alignment can be exhibited. A case is described that underscores some of the particular challenges associated with management of the ventricular septal defect during double switch operation for hearts with discordant atrioventricular connections and either transposition of the great arteries or double outlet right ventricle., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The question posed in the title of this article is: "Congenital Heart Surgery Databases Around the World: Do We Need a Global Database?" The answer to this question is "Yes and No"! Yes--we need to create a global database to track the outcomes of patients with pediatric and congenital heart disease. No--we do not need to create a new "global database." Instead, we need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This review article will achieve the following objectives: (A) Consider the current state of analysis of outcomes of treatments for patients with congenitally malformed hearts. (B) Present some principles that might make it possible to achieve life-long longitudinal monitoring and follow-up. (C) Describe the rationale for the creation of a Global Federated Multispecialty Congenital Heart Disease Database. (D) Propose a methodology for the creation of a Global Federated Multispecialty Congenital Heart Disease Database that is based on linking together currently existing databases without creating a new database. To perform meaningful multi-institutional analyses, any database must incorporate the following six essential elements: (1) Use of a common language and nomenclature. (2) Use of a database with an established uniform core dataset for collection of information. (3) Incorporation of a mechanism to evaluate the complexity of cases. (4) Implementation of a mechanism to assure and verify the completeness and accuracy of the data collected. (5) Collaboration between medical and surgical subspecialties. (6) Standardization of protocols for life-long longitudinal follow-up. Analysis of outcomes must move beyond recording 30-day or hospital mortality, and encompass longer-term follow-up, including cardiac and non-cardiac morbidities, and importantly, those morbidities impacting health-related quality of life. Methodologies must be implemented in our databases to allow uniform, protocol-driven, and meaningful long-term follow-up. We need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This "Global Federated Multispecialty Congenital Heart Disease Database" will not be a new database, but will be a platform that effortlessly links multiple databases and maintains the integrity of these extant databases. Description of outcomes requires true multi-disciplinary involvement, and should include surgeons, cardiologists, anesthesiologists, intensivists, perfusionists, neurologists, educators, primary care physicians, nurses, and physical therapists. Outcomes should determine primary therapy, and as such must be monitored life-long. The relatively small numbers of patients with congenitally malformed hearts requires multi-institutional cooperation to accomplish these goals. The creation of a Global Federated Multispecialty Congenital Heart Disease Database that links extant databases from pediatric cardiology, pediatric cardiac surgery, pediatric cardiac anesthesia, and pediatric critical care will create a platform for improving patient care, research, and teaching related to patients with congenital and pediatric cardiac disease., (Copyright (c) 2010. Published by Elsevier Inc.)

The status of pulmonary circulation is of utmost importance to the success of the modified Fontan operation. Pulmonary artery distortion, hypoplasia of the total pulmonary vascular bed, and elevated pulmonary vascular resistance are all risk factors for adverse outcome after modified Fontan operations. In cases of irreparable acquired atresia of one or the other branch pulmonary artery, clinicians are forced to contemplate the possibility of total cavopulmonary connection to one lung. The combined experience of the authors with 12 cases suggests that the likelihood of operative survival following Fontan's operation to one lung is predicted based on the usual hemodynamic parameters: pulmonary artery pressure and flow, ventricular end diastolic pressure, transpulmonary gradient, and pulmonary vascular resistance. In this series, there were no operative mortalities among patients undergoing Fontan's operation to one lung, all of whom meet the usual criteria for hemodynamic acceptability. There may, however, be a higher incidence of protein-losing enteropathy than in Fontan patients with normal pulmonary vascular beds. All possible means of resuscitating the lost elements of the pulmonary vascular bed and re-establishing pulmonary artery continuity should be attempted to minimize pulmonary vascular capacitance of patients undergoing Fontan's operation. It is clear, however, that the presence of only one pulmonary artery does not in and of itself preclude satisfactory outcome.

In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume load associated with parallel pulmonary and systemic circulations. The hemi-Fontan operation was conceived as a means of accomplishing early reduction of the volume work of the single ventricle, in anticipation of eventual completion of a modified Fontan procedure. The hemi-Fontan procedure includes association of the superior vena(e) cava(e) (SVC) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the SVC into the right atrium, and elimination of other sources of pulmonary blood flow. Because it is preparatory to an eventual completion Fontan procedure, concomitant procedures are conveniently performed in conjunction with the hemi-Fontan. These may include atrial septectomy, relief of aortic arch obstruction, proximal main pulmonary artery to ascending aortic anastomosis, repair or revision of anomalous pulmonary venous connection, atrioventricular valvuloplasty, or other procedures., (Copyright 2003 Elsevier, Inc. All rights reserved.)

Enormous progress has been made in the last decade toward decreasing the risk of the Fontan operation and optimizing early and intermediate outcomes. Heterotaxy syndrome patients, with their extensive constellation of anatomic abnormalities, constitute a challenging group to manage surgically. Palliative procedures early in life are directed at creation of a satisfactory balance between pulmonary and systemic blood flow and at the same time assurance of unobstructed pulmonary venous return. For heterotaxy patients who survive beyond the newborn period, and are considered to have nonseptatable hearts, early reduction of the volume load of the functional single ventricle is extremely important. Early conversion from parallel pulmonary and systemic circulations to a superior cavopulmonary anastomosis (bidirectional Glenn or hemi-Fontan) is the preferred strategy. Traditionally, those patients with heterotaxy who survive initial surgical management have been considered a very high-risk population with respect to an eventual Fontan procedure. In heterotaxy patients it is particularly important to understand the unique and variable anatomy of the sinus node and conduction system, the potential for occult pulmonary venous obstruction, the tendency for development of atrioventricular valve regurgitation in volume loaded ventricles, and the potential for recurrent or persistent cyanosis because of intrahepatic shunting or pulmonary arteriovenous malformations., (Copyright 2002 by W.B. Saunders Company)