The first-ever published indicators to assess the quality of outpatient management for adult congenital heart disease (ACHD) patients have been identified for six ACHD conditions: secundum atrial septal defects, aortic coarctation, Eisenmenger syndrome, Fontan repair for a single ventricle, dextro-transposition of the great arteries and tetralogy of Fallot.

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The quality indicators, published Oct. 9 in the Journal of the American College of Cardiology (JACC) , were developed by experts in the management of ACHD after review of both medical literature and practice guidelines from the U.S., Canada and Europe. A total of 55 indicators were accepted – eight for atrial septal defects, nine for aortic coarctation, 12 for Eisenmenger syndrome, nine for the Fontan procedure, nine for dextro-transposition of the great arteries and eight for tetralogy of Fallot. These indicators include actions such as an annual visit with a specialist in the treatment of ACHD patients, annual measurement of oxygen saturation, catheterization performed by a specialist and a transthoracic echocardiogram interpreted by a cardiologist with imaging experience in congenital heart defects.

"The development of these quality indicators constitutes a pivotal point that will allow us to begin to measure quality of care being delivered," the authors said. Moving forward, they note that "In future studies, this first iteration of a quality assessment tool will be tested, updated, refined and expanded as more data become available. With this project we have advanced the process of improving the quality of care for the growing group of ACHD patients."

Meanwhile, a separate article also published in JACC  addresses tetralogy of Fallot, and summarizes the latest information from experts concerning key clinical issues for treatment and long-term management. Specifically, the authors discuss the genetics of tetralogy of Fallot, imaging, arrhythmias and sudden cardiac death associated with the condition, surgical therapy, catheter-based therapy and pulmonary valve replacement.

According to the authors, tetralogy of Fallot has a genetic familial recurrence rate of three percent, and "genetic data can be used to risk stratify patients regarding cardiac and non-cardiac manifestations of disease." For young patients, cardiac magnetic resonance imaging represents the gold-standard assessment tool, whereas an echocardiogram is the primary imaging tool. Recent advances in echocardiography "have improved the assessment of global and regional right ventricle performance," and the authors add that the techniques currently under development "could provide reliable information for clinical decision-making."

Other observations of note include atrial re-entrant tachycardia develops in more than 30 percent of patients and high-grade ventricular arrhythmias develop in about 10 percent of patients. In addition, the incidence of sudden cardiac death is "estimated at 0.2 percent per year of follow-up." The authors also note that defibrillator placement is often recommended for high-risk patients, however, definitive information about optimal surgical treatment strategies is lacking. In terms of transcatheter intervention, the authors suggest that postoperative lesions are frequently amenable to transcatheter intervention to avoid reoperation and that transcatheter valve insertion may be used to treat pulmonary regurgitation in selected patients.

According to Juan Villafane, MD, FACC, professor of pediatric cardiology at the University of Kentucky, immediate past governor of the ACC Kentucky Chapter and lead author of the article, "Tetralogy of Fallot is the most common cyanotic congenital heart defect. The clinician is faced with multiple potential complications even in the asymptomatic post-operative patient. There is emerging science relating to these complications that will help improve the management, morbidity and mortality of these patients who are faced with multiple surgeries, right heart failure, arrhythmias and even sudden cardiac death."

Keywords: Tetralogy of Fallot, Defibrillators, Follow-Up Studies, Heart Defects, Congenital, Fontan Procedure, Heart Septal Defects, Atrial, Magnetic Resonance Imaging, Tachycardia, Incidence, Reoperation, Eisenmenger Complex, Pulmonary Valve Insufficiency, Quality Indicators, Health Care, Heart Failure, Catheterization, Oxygen, Death, Sudden, Cardiac, Echocardiography

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