Title: ESC Guidelines for the Management of Grown-up Congenital Heart Disease (new version 2010): The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC)
Date Posted: September 13, 2010
Authors: Baumgartner H, Bonhoeffer P, De Groot NM, et al.
Citation: Eur Heart J 2010;Aug 27:[Epub ahead of print].
Perspective:
The following are 10 points to remember from the updated European Society of Cardiology guidelines for the management of adult congenital heart disease:
1. Cardiac magnetic resonance imaging is an important technique for the evaluation of adults with congenital heart disease. It is particularly important for quantification of right ventricular size and function; quantification of pulmonary insufficiency; evaluation of extracardiac structures including the pulmonary arteries, veins, and the aorta; and detection of myocardial fibrosis and scar.
2. Device closure is the preferred method for closure of most atrial septal defects, and is indicated if signs of right ventricular volume overload are present. Atrial septal defect closure should be avoided in patients with Eisenmenger physiology.
3. Patients with ventricular septal defects should be monitored for long-term complications including endocarditis, development of double-chambered right ventricle, subaortic stenosis, and aortic insufficiency due to leaflet prolapse (particularly in patients with supracristal ventricular septal defects).
4. Percutaneous closure of patent ductus arteriosus is appropriate when left ventricular volume overload is present. Closure of a “silent ductus” is not indicated. Closure of patent ductus arteriosus is not appropriate in the setting of Eisenmenger physiology.
5. Patients with repaired coarctation of the aorta are at risk for multiple long-term complications including arterial hypertension, recurrent coarctation, aneurysms of the ascending aorta or coarctation repair site, premature coronary artery disease, and berry aneurysms of the circle of Willis.
6. Indications for intervention in adults with coarctation of the aorta include a noninvasive pressure difference ≥20 mm Hg between the upper and lower extremities with upper extremity hypertension, pathologic blood pressure response to exercise, and significant left ventricular hypertrophy.
7. All patients with tetralogy of Fallot should be seen at a specialized adult congenital heart program. The presence or absence of pulmonary insufficiency must be determined. Pulmonary valve replacement (PVR) should be performed in symptomatic patients with severe pulmonary insufficiency for stenosis (Class I, level of evidence C). PVR should be performed in asymptomatic patients with declining exercise capacity, progressive right ventricular dilatation, progressive tricuspid regurgitation, right ventricular outflow tract obstruction with right ventricular systolic pressure greater than 80 mm Hg, and sustained atrial or ventricular arrhythmias (Class IIa, level of evidence C).
8. All patients with single-ventricle physiology following the Fontan procedure should be seen at an adult congenital center. Long-term complications include progressive heart failure, cyanosis, development of atrial arrhythmias, chronic venous insufficiency, and protein losing enteropathy.
9. Patients with a Rastelli procedure, pulmonary atresia, common arterial trunk, and previous Ross operation will have had a conduit placed between the right ventricle and pulmonary arteries. Such patients are now candidates for percutaneous pulmonary valve replacement when conduit insufficiency or stenosis develops.
10. Bosentan should be initiated in patients with Eisenmenger syndrome who are World Health Organization-Functional Class III. Patients with Eisenmenger require meticulous follow-up and avoidance of high-risk states including pregnancy, iron deficiency anemia, dehydration, vaccine preventable infectious disease, smoking, transvenous pacemaker and defibrillator leads, strenuous exercise, and acute heat exposure as in hot tubs or saunas.
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