Indications for closure and medical management of atrial septal defects in adults
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
Atrial septal defect (ASD) is the most common congenital lesion in adults after bicuspid aortic valve. Although the defect is often asymptomatic until adulthood, potential complications of an undetected ASD include atrial arrhythmias, paradoxical embolization, cerebral abscess, right ventricular failure, and pulmonary hypertension that can become irreversible and lead to right-to-left shunting (Eisenmenger syndrome).
This topic will review indications for closure and medical management of ASDs in adults.
Surgical and percutaneous closure of ASDs, pathophysiology, anatomy, natural history, and clinical features of ASDs in adults, the identification and assessment of ASDs, and issues related to ASDs in children are discussed separately. (See "Surgical and percutaneous closure of atrial septal defects in adults" and "Clinical manifestations and diagnosis of atrial septal defects in adults" and "Identification and assessment of atrial septal defects in adults" and "Classification of atrial septal defects (ASDs), and clinical features and diagnosis of isolated ASDs in children" and "Management and outcome of isolated atrial septal defects in children".)
INDICATIONS FOR DEFECT CLOSURE
Overview — The main indication for atrial septal defect (ASD) closure is right ventricular enlargement with or without symptoms (eg, exercise intolerance, fatigue, dyspnea, heart failure, paradoxical emboli, arrhythmias) . ASD closure is also reasonable in patients with paradoxical embolism or documented orthodeoxia-platypnea. Primum ASD closure is also indicated at the time of cleft mitral valve repair. (See "Clinical manifestations and diagnosis of atrial septal defects in adults" and "Management and outcome of atrioventricular (AV) canal defects", section on 'Surgical interventions'.)
Right ventricular volume overload — Closure (surgical or percutaneous) of an ASD is indicated in patients with associated right ventricular enlargement and no evidence of pulmonary arterial hypertension (pulmonary vascular resistance <5 Wood units) with or without symptoms [1-3]. (See "Identification and assessment of atrial septal defects in adults", section on 'Evaluation for RV overload'.)
- Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
- Silversides CK, Dore A, Poirier N, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: shunt lesions. Can J Cardiol 2010; 26:e70.
- Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31:2915.
- Fuse S, Tomita H, Hatakeyama K, et al. Effect of size of a secundum atrial septal defect on shunt volume. Am J Cardiol 2001; 88:1447.
- Vick GW III. Defects of the atrial septum Including atrioventricular septal defects. In: Science and Practice of Pediatric Cardiology, 2nd ed, Garson A Jr, Bricker JT, Fisher DJ, Neish SR (Eds), Williams & Wilkins, Baltimore 1998. p.1141.
- Attie F, Rosas M, Granados N, et al. Surgical treatment for secundum atrial septal defects in patients >40 years old. A randomized clinical trial. J Am Coll Cardiol 2001; 38:2035.
- Gatzoulis MA, Freeman MA, Siu SC, et al. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med 1999; 340:839.
- Konstantinides S, Geibel A, Olschewski M, et al. A comparison of surgical and medical therapy for atrial septal defect in adults. N Engl J Med 1995; 333:469.
- John Sutton MG, Tajik AJ, McGoon DC. Atrial septal defect in patients ages 60 years or older: operative results and long-term postoperative follow-up. Circulation 1981; 64:402.
- Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
- Engelfriet P, Boersma E, Oechslin E, et al. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur Heart J 2005; 26:2325.
- Zuber M, Gautschi N, Oechslin E, et al. Outcome of pregnancy in women with congenital shunt lesions. Heart 1999; 81:271.
- Perloff, JK. The Clinical Recognition of Congenital Heart Disease, 6th ed, Elsevier/Saunders, Philadelphia 2012.
- McFaul PB, Dornan JC, Lamki H, Boyle D. Pregnancy complicated by maternal heart disease. A review of 519 women. Br J Obstet Gynaecol 1988; 95:861.
- Perloff JK. Pregnancy and cardiovascular disease. In: Heart Disease, Braunwald E (Ed), WB Saunders, Philadelphia 1988.
- Harvey JR, Teague SM, Anderson JL, et al. Clinically silent atrial septal defects with evidence for cerebral embolization. Ann Intern Med 1986; 105:695.
- Pitkin RM, Perloff JK, Koos BJ, Beall MH. Pregnancy and congenital heart disease. Ann Intern Med 1990; 112:445.
- Loscalzo J. Paradoxical embolism: clinical presentation, diagnostic strategies, and therapeutic options. Am Heart J 1986; 112:141.
- Perloff JK, Child JS. Congenital Heart Disease in Adults, 2nd ed, WB Saunders, Philadelphia 1998.
- Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med 1990; 323:1645.
- Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49:2303.
- Posch MG, Perrot A, Berger F, Ozcelik C. Molecular genetics of congenital atrial septal defects. Clin Res Cardiol 2010; 99:137.
- Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
- Liberthson, R. Arrhythmias in the athlete with congenital heart disease — Guidelines for participation. From North American Society of Pacing and Electrophysiology Expert Consensus Conference on Arrhythmias and the Athlete, May 6, 1998.
- McMahon CJ, Feltes TF, Fraley JK, et al. Natural history of growth of secundum atrial septal defects and implications for transcatheter closure. Heart 2002; 87:256.
- INDICATIONS FOR DEFECT CLOSURE
- Right ventricular volume overload
- - Qp/Qs ratio
- - In selected patients with pulmonary hypertension
- Paradoxical embolization
- Supporting data
- MEDICAL MANAGEMENT WITH OR WITHOUT REPAIR
- Atrial arrhythmias
- Pulmonary hypertension
- Endocarditis prophylaxis
- - Unrepaired ASD
- - Repaired ASD
- - Genetic factors
- Participation in sports
- ISSUES WITH UNREPAIRED ASDS
- Scuba diving
- High altitude exposure
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS