Pediatric Atrial Septal Defects Treatment & Management
- Author: Michael R Carr, MD; Chief Editor: P Syamasundar Rao, MD more...
Medical therapy is of no benefit in children with asymptomatic atrial septal defects (ASDs). With the exception of ostium secundum types, atrial septal defects are structural defects that do not spontaneously close. Surgical closure is ultimately required for most atrial septal defects, other than ostium secundum atrial septal defects. Occasionally, small primum ASDs may not require closure, but due to their association with mitral valve abnormalities, they may be closed at the time of mitral valve repair, if such a repair is indicated. An ostium secundum atrial septal defect that measures 6 mm in diameter or smaller in the patient's first year of life is likely to spontaneously close; however, such closure is substantially slower than that of a typical small, muscular ventricular septal defect.
Infants who are severely affected with an atrial septal defect and who develop congestive heart failure (CHF) may be treated as any other child with CHF from a left-to-right shunt. This treatment, which includes diuretics, afterload reduction, and less commonly, digoxin, is covered in other articles. As noted previously, it is of particular importance to rule out other etiologies of symptoms in these infants, especially those with secundum ASDs, due to the typical paucity of symptoms associated with this lesion in infancy and early childhood.
Arrhythmias associated with atrial septal defect are similarly uncommon in childhood become increasingly common with age. In fact, the development of atrial fibrillation may trigger CHF in adults with atrial septal defect who are younger than 40 years. Arrhythmias may result from atrial distention, and these individuals may require antiarrhythmic therapy until the atrial septal defect is repaired. In some cases, arrhythmias may persist after repair due to the chronicity of the right atrial dilation.
Some children with an atrial septal defect present with recurrent respiratory tract infections, which may be poorly tolerated. If immunologically normal, these children are not at a higher risk for infection in general, but the chronically increased pulmonary bloodflow, when combined with the inflammatory changes associated with lower respiratory tract infections, can lead to prolonged symptoms and altered cardiopulmonary interactions.
Bacterial endocarditis prophylaxis is not necessary for the typical patient with an isolated atrial septal defect, regardless of the type of atrial septal defect. Prophylaxis is recommended prior to surgical repair if the atrial septal defect is part of complex cyanotic congenital heart disease (CCHD). Endocarditis prophylaxis is recommended for 6 months following either surgical or percutaneous repair of atrial septal defects and, in some instances, may be recommended for longer in patients with residual mitral valve abnormalities after surgical repair of primum atrial septal defects. Additionally, endocarditis prophylaxis is recommended indefinitely for any persistent residual shunting detected by echocardiography after surgical or device closure. For more information see Antibiotic Prophylactic Regimens for Endocarditis.
Definitive therapy for an atrial septal defect has historically been limited to surgical closure. However, with the advent of transcatheter techniques, many children undergo successful treatment in the cardiac catheterization laboratory.
The most common surgical approach to the defect is primary repair with suture closure or with patch repair (generally with glutaraldehyde treated autologous pericardium, Gore Tex patch or fabric made of polyester fiber [Dacron]).
Not all children with an atrial septal defect are candidates for surgery, which is only indicated for those children with clinically significant left-to-right shunting. In general, a pulmonary-to-systemic flow ratio of 1.5:1 or more is considered the principal indication for surgical repair. Shunting less than this in children with small defects and in those with existing pulmonary hypertension may be observed. Because cardiac catheterization is rarely necessary, echocardiographic evidence of right atrial and right ventricular enlargement is usually considered evidence of a clinically significant left-to-right shunt and an indication for surgical closure of the atrial septal defect.
Surgery is ideally performed in children aged 2-4 years and has a very low mortality rate. However, surgery may be performed earlier than this if the child has evidence of CHF. Surgery can be deferred until later in childhood if there is a specific family preference without adding any substantial risk by delaying intervention.
Newer, minimally invasive surgical techniques have been developed. These improve cosmetic appearances and decrease hospital stays. These techniques are ideally suited for simple closure of a secundum atrial septal defect.
The surgical mortality rate is low in patients with uncomplicated atrial septal defects. In an experienced pediatric center, the mortality rate should be less than 1%.
Postoperative morbidity in individuals with atrial septal defects is almost exclusively due to accumulation of pericardial fluid (postpericardiotomy syndrome), which occurs in approximately one third of patients. On occasion, tamponade occurs and requires pericardiocentesis. Pericardial effusion should be suspected in any pediatric patient who undergoes postsurgical repair of an atrial septal defect and who presents with chest pain, fever, shortness of breath, or general malaise. In young children, symptoms may be nonspecific and include irritability and decreased appetite.
Transcatheter approaches to atrial septal defect closure are well accepted in the pediatric population. Secundum atrial septal defects are currently the only subtype of atrial septal defect that are amenable to this approach. The preference for timing of catheter-based closure is institution/interventionalist specific, but generally around age 4-6 years with a known, hemodyanmically significant defect. Catheter-based intervention has been successful in smaller children.
Such techniques require individuals with considerable expertise in the field of interventional pediatric cardiology and cooperation between the interventionalist and the noninvasive imaging specialists.
Benefits of the transcatheter approach include its minimal invasiveness, the lack of median sternotomy, the avoidance of cardiopulmonary bypass, and the relatively quick recovery time. Potential drawbacks and concerns include residual shunting around the device, embolization during placement requiring surgical intervention, lack of adequate septal rims to properly seat the device and the need for specific technical expertise and equipment. Long-term safety concerns are noted because device placement in smaller children is still relatively new. There has been some recent concern regarding device erosion of surrounding tissue, which is not limited to the pediatric popluation. Further follow-up should provide additional information regarding optimal patient selection, as well as longer-term prognosis of the catheter-based approach. Overall however, the medium- to long-term outcomes of ASD closure, either surgically or percutaneously, appear very good.
Diagnosis of an atrial septal defect in a newborn or an older child should prompt consultation with a pediatric cardiologist.
Almost all newborns have a small left-to-right shunt at the foramen ovale, as detected during echocardiography in the neonatal period. In the absence of a murmur or other signs of a true atrial septal defect on subsequent well-child care visits, consultation with a cardiologist is probably not necessary, although review of the initial echocardiogram report is prudent to verify the description of the defect.
Children with atrial septal defects generally have no restrictions on their activity. Children with compensated CHF with an atrial septal defect are candidates for surgical or catheter-based intervention and should be able to resume normal activity after the defect is corrected.
Van Praagh R, Corsini I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13 postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart J. 1969 Sep. 78(3):379-405. [Medline].
Weinberg PM, Chin AJ, Murphy JD, et al. Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery. Am J Cardiol. 1986 Dec 1. 58(13):1228-32. [Medline].
Pierpont ME, Basson CT, Benson DW Jr, Gelb BD, Giglia TM, Goldmuntz E, et al. Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007 Jun 12. 115(23):3015-38. [Medline].
[Guideline] Jenkins KJ, Correa A, Feinstein JA, et al. Noninherited risk factors and congenital cardiovascular defects: current knowledge: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007 Jun 12. 115(23):2995-3014. [Medline].
Basson CT, Bachinsky DR, Lin RC, et al. Mutations in human TBX5 [corrected] cause limb and cardiac malformation in Holt-Oram syndrome. Nat Genet. 1997 Jan. 15(1):30-5. [Medline].
Reamon-Buettner SM, Borlak J. NKX2-5: an update on this hypermutable homeodomain protein and its role in human congenital heart disease (CHD). Hum Mutat. 2010 Nov. 31(11):1185-94. [Medline].
Hirayama-Yamada K, Kamisago M, Akimoto K, et al. Phenotypes with GATA4 or NKX2.5 mutations in familial atrial septal defect. Am J Med Genet A. 2005. 135(1):47-52. [Medline].
Posch MG, Waldmuller S, Müller M, Scheffold T, Fournier D, Andrade-Navarro MA, et al. Cardiac alpha-myosin (MYH6) is the predominant sarcomeric disease gene for familial atrial septal defects. PLoS One. 2011. 6(12):e28872. [Medline]. [Full Text].
Chin AJ, Weinberg PM, Barber G. Subcostal two-dimensional echocardiographic identification of anomalous attachment of septum primum in patients with left atrioventricular valve underdevelopment. J Am Coll Cardiol. 1990 Mar 1. 15(3):678-81. [Medline].
Prompona M, Muehling O, Naebauer M, Schoenberg SO, Reiser M, Huber A. MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects. Int J Cardiovasc Imaging. 2011 Mar. 27(3):403-12. [Medline].
Hoey ET, Gopalan D, Ganesh V, Agrawal SK, Screaton NJ. Atrial septal defects: magnetic resonance and computed tomography appearances. J Med Imaging Radiat Oncol. 2009 Jun. 53(3):261-70. [Medline].
Johri AM, Rojas CA, El-Sherief A, Witzke CF, Chitty DW, Palacios IF, et al. Imaging of atrial septal defects: echocardiography and CT correlation. Heart. 2011 Sep. 97(17):1441-53. [Medline].
Ko SF, Liang CD, Yip HK, Huang CC, Ng SH, Huang CF. Amplatzer septal occluder closure of atrial septal defect: evaluation of transthoracic echocardiography, cardiac CT, and transesophageal echocardiography. AJR Am J Roentgenol. 2009 Dec. 193(6):1522-9. [Medline].
Vida VL, Padalino MA, Boccuzzo G, Veshti AA, Speggiorin S, Falasco G. Minimally invasive operation for congenital heart disease: a sex-differentiated approach. J Thorac Cardiovasc Surg. 2009 Oct. 138(4):933-6. [Medline].
Moore J, Hegde S, El-Said H, Beekman R 3rd, Benson L, Bergersen L, et al. Transcatheter device closure of atrial septal defects: a safety review. JACC Cardiovasc Interv. 2013 May. 6(5):433-42. [Medline].
Kutty S, Hazeem AA, Brown K, Danford CJ, Worley SE, Delaney JW, et al. Long-term (5- to 20-year) outcomes after transcatheter or surgical treatment of hemodynamically significant isolated secundum atrial septal defect. Am J Cardiol. 2012 May 1. 109(9):1348-52. [Medline].
Fukazawa M, Fukushige J, Ueda K. Atrial septal defects in neonates with reference to spontaneous closure. Am Heart J. 1988 Jul. 116(1 Pt 1):123-7. [Medline].
Hanslik A, Pospisil U, Salzer-Muhar U, Greber-Platzer S, Male C. Predictors of spontaneous closure of isolated secundum atrial septal defect in children: a longitudinal study. Pediatrics. 2006 Oct. 118(4):1560-5. [Medline].
Jonas RA. Atrial septal defect. Comprehensive Surgical Management of Congenital Heart Disease. 2004. 225-41.
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 Dec 13. 323(24):1645-50. [Medline].
Alexi-Meskishvili VV, Konstantinov IE. Surgery for atrial septal defect: from the first experiments to clinical practice. Ann Thorac Surg. 2003 Jul. 76(1):322-7. [Medline].
Bierman FZ, Williams RG. Subxiphoid two-dimensional imaging of the interatrial septum in infants and neonates with congenital heart disease. Circulation. 1979 Jul. 60(1):80-90. [Medline].
Bruneau BG. The developmental genetics of congenital heart disease. Nature. Febuary 2008. 451(7178):943-8. [Medline].
Butera G, Carminati M, Chessa M, et al. Percutaneous versus surgical closure of secundum atrial septal defect: comparison of early results and complications. Am Heart J. 2006. 151(1):228-34. [Medline].
Chin AJ, Murphy JD. Identification of coronary sinus septal defect (unroofed coronary sinus) by color Doppler echocardiography. Am Heart J. 1992 Dec. 124(6):1655-7. [Medline].
Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am. 1999 Apr. 46(2):355-68, x. [Medline].
Friedman WF. Congenital heart disease in infancy and childhood. Braunwald ed. Heart Disease: A Textbook of Cardiovascular Medicine. 5th ed. 1997. 896-910.
Helgason H, Jonsdottir G. Spontaneous closure of atrial septal defects. Pediatr Cardiol. 1999. 20(3):195-9. [Medline].
Mahoney LT, Truesdell SC, Krzmarzick TR, Lauer RM. Atrial septal defects that present in infancy. Am J Dis Child. 1986 Nov. 140(11):1115-8. [Medline].
Mas MS, Bricker JT. Clinical physiology of left-to-right shunts. The Science and Practice of Pediatric Cardiology. 1990. Vol 2: 999-1001.
Patel HT, Hijazi ZM. Pediatric catheter interventions: a year in review 2004-2005. Curr Opin Pediatr. 2005. 17(5):568-73. [Medline].
Porter CJ, Feldt RH, Edwards WD, et al. Atrial septal defects. Heart Disease in Infants, Children, and Adolescents. 6th ed. 2001. Vol 1: 603-17.
Radzik D, Davignon A, van Doesburg N, et al. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol. 1993 Sep. 22(3):851-3. [Medline].
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 Oct 9. 116(15):1736-54. [Medline].
Yeager SB, Chin AJ, Sanders SP. Subxiphoid two-dimensional echocardiographic diagnosis of coronary sinus septal defects. Am J Cardiol. 1984 Sep 1. 54(6):686-7. [Medline].