Functionally Univentricular Heart and the Fontan Operation: Lessons Learned About Patterns of Practice and Outcomes From the Congenital Heart Surgery Databases of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons
Johns Hopkins Children's Heart Surgery, All Children's Hospital and Florida Hospital for Children, Saint Petersburg, Tampa, and Orlando, FL, USA.World Journal for Pediatric and Congenital Hearth Surgery 10/2013; 4(4):349-55. DOI: 10.1177/2150135113494228
Background: “The term “functionally univentricular heart” describes a spectrum of congenital cardiovascular malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation and another to the pulmonary circulation.” The purpose of this article is to review patterns of practice and outcomes in the Congenital Heart Surgery Databases (CHSDBs) of the European Association for Cardio-Thoracic Surgery (EACTS) and the Society of Thoracic Surgeons (STS) in patients with functionally univentricular hearts undergoing the Fontan operation.
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ABSTRACT: The term “univentricular”, although frequently used, is confusing due to the fact that its literal meaning does not coincide at all with its real meaning in clinical practice. This lack of precision is the source of frequent misunderstandings, especially among those who may be less familiar with congenital heart disease. The matter is made worse by the fact that the same term is used when referring to three different concepts —univentricular heart, univentricular physiology, and univentricular path or protocol. For these reasons, it is worth clarifying, in a precise and simple fashion, the true meaning of this term. This is the objective of this article.Cirugia Cardiovascular 08/2014; DOI:10.1016/j.circv.2014.06.006
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ABSTRACT: Although significant progress has been made in the care of patients with paediatric and congenital cardiac disease, optimization of outcomes remains a constant goal. This review article will discuss the latest advances in the science of assessing the outcomes and improving the quality of the congenital and paediatric cardiac care, and will also review some of the latest associated research. Important advances continue to be made in each of the following domains: standardized nomenclature; established uniform core dataset; evaluation of case complexity; verification of the completeness and accuracy of the data; collaboration between subspecialties; strategies for longitudinal follow-up; and incorporating quality improvement. In January 2015, the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) will begin voluntary public reporting of programmatic congenital cardiac surgical outcomes using a new risk model that includes both procedural risk (as defined by the procedure itself and STAT Categories) and a number of patient-specific characteristics including age, weight, prior cardiothoracic operation, prematurity, chromosomal abnormalities, syndromes, noncardiac congenital anatomic abnormalities and preoperative factors. Clinical databases have been linked with administrative database to answer questions neither dataset can answer independently, providing new information about long-term mortality, rates of rehospitalization, long-term morbidity, comparative effectiveness of various treatments, and the cost of healthcare. Multiple research initiatives have recently been published using STS-CHSD. The science of assessing the outcomes and improving the quality of congenital and paediatric cardiac care continues to evolve. Recent advances will facilitate the continued evolution of a meaningful method of multiinstitutional outcomes analysis for congenital and paediatric cardiac surgery.Current Opinion in Cardiology 01/2015; 30(1):100-111. DOI:10.1097/HCO.0000000000000133 · 2.70 Impact Factor
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ABSTRACT: To evaluate the utility of magnetic resonance elastography (MRE) in screening patients for hepatic fibrosis, cirrhosis, and hepatocellular carcinoma after the Fontan operation. Hepatic MRE was performed in conjunction with cardiac magnetic resonance imaging in patients who had undergone a Fontan operation between 2010 and 2014. Liver stiffness was calculated using previously reported techniques. Comparisons to available clinical, laboratory, imaging, and histopathologic data were made. Overall, 50 patients at a median age of 25 years (range, 21-33 years) who had undergone a Fontan operation were evaluated. The median interval between Fontan operation and MRE was 22 years (range, 16-26 years). The mean liver stiffness values were increased: 5.5±1.4 kPa relative to normal participants. Liver stiffness directly correlated with liver biopsy-derived total fibrosis score, time since operation, mean Fontan pressure, γ-glutamyltransferase level, Model for End-Stage Liver Disease score, creatinine level, and pulmonary vascular resistance index. Liver stiffness was inversely correlated with cardiac index. All 3 participants with hepatic nodules exhibiting decreased contrast uptake on delayed postcontrast imaging and increased nodule stiffness had biopsy-proven hepatocellular carcinoma. The association between hepatic stiffness and fibrosis scores, Model for End-Stage Liver Disease scores, and γ-glutamyltransferase level suggests that MRE may be useful in detecting (and possibly quantifying) hepatic cirrhosis in patients after the Fontan operation. The correlation between stiffness and post-Fontan time interval, mean Fontan pressure, pulmonary vascular resistance index, and reduced cardiac index suggests a role for long-term hepatic congestion in creating these hepatic abnormalities. Magnetic resonance elastography was useful in detecting abnormal nodules ultimately diagnosed as hepatocellular carcinoma. The relationship between stiffness with advanced fibrosis and hepatocellular carcinoma provides a strong argument for additional study and broader application of MRE in these patients. Copyright © 2015 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.Mayo Clinic Proceedings 06/2015; 90(7). DOI:10.1016/j.mayocp.2015.04.020 · 6.26 Impact Factor
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