eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult
Atrial Septal Defect: Treatment
Updated: Jan 21, 2010
Treatment
Medical Therapy
Atrial septal defect (ASD) is a disorder to be addressed surgically or through interventional catheterization. No specific or definitive medical therapy is available. However, patients with significant volume overload or atrial arrhythmias may require specific drug therapy.
Surgical Therapy
Criterion standard
The criterion standard in the treatment of atrial septal defect (ASD) is direct closure of the defect by using an open approach with extracorporeal support. John Gibbon performed the first successful ASD closure by applying this method in 1953. Surgical techniques and equipment have since improved to the point that the mortality rate from this repair approaches zero.
In the usual procedure, a median sternotomy incision is made, and the sternum is split in the midline. Direct arterial and double venous (superior vena cava and inferior vena cava) cannulation are performed. By applying cardiopulmonary bypass, the aorta is clamped, and the heart is arrested with a cardioplegia solution. The caval snares are tightened, and the right atrium is opened. Most secundum defects can be closed by using a direct continuous suture of 3-0 or 4-0 polypropylene (Prolene).
Caution must be taken when large defects are directly closed because this closure can distort the atrium. Large defects that rise superiorly can distort the aortic anulus if closed directly. These ASDs are best closed by using autologous pericardium or synthetic patches made of polyester polymer (Dacron) or polytetrafluoroethylene (PTFE). Care must be taken to completely remove any air or debris from the left atrium and ventricle before cardiopulmonary bypass is discontinued. Temporary pacing wires are left in place on the right ventricle before the chest is closed over the drains.
In an ostium primum defect, surgical closure is more complicated. The patch must be attached to the septum at the juncture of the mitral and tricuspid valves. Mitral valve repair, including closure of the cleft mitral leaflet and, possibly annuloplasty, may be necessary to correct or prevent mitral insufficiency. In rare cases, mitral valve replacement may be required.
In a sinus venosus defect, partial anomalous pulmonary venous return is typical. One or more of the pulmonary veins primarily drains into the right atrium. The ASD must be patched in such a way as to ensure that the anomalous pulmonary venous drainage is diverted into the left atrium. This patching may be simple or complex, depending on where the anomalous drainage enters. Many innovative techniques have been developed to redirect pulmonary venous flow, and the surgeon should be familiar with several approaches. Pulmonary venous return must not be compromised with the redirection because this invariably causes localized pulmonary venous hypertension.
Minimally invasive approaches
In recent years, minimally invasive approaches to the repair of ASD have garnered significant interest. In most cases, the size of the incision is simply decreased with different approaches to cardiopulmonary bypass. Examples include partial or full submammary skin incision, hemisternotomy, and limited thoracotomy. The goal is to improve better cosmetic results because these approaches are not associated with decreased morbidity or mortality.
Percutaneous transcatheter closure
In recent times, secundum ASD have been closed by using a variety of catheter-implanted occlusion devices rather than by direct surgical closure with cardiopulmonary bypass. These devices are placed through a femoral venous approach and are deployed like an umbrella to seal the septal defect. These devices work best for centrally located secundum defects. Although surgical closure is associated with low morbidity and mortality and excellent long-term results, sternotomy and cardiopulmonary bypass are required.
Drs King and Mills performed the first transcatheter closure of a secundum ASD in the mid-1970s. William Rashkind pioneered the development of percutaneous ASD closure technique in late 1970s. Jim Lock developed the clamshell method in 1989. Around the same time, Sideris started clinical trials with buttoned device.
Although many devices have been studied, over the last few years, 4 major devices have become available: CardioSEAL (NMT Medical, Inc; Boston, Mass), Amplatzer septal occluder (ASO) (AGA Medical Corporation; Golden Valley, Minn), HELEX septal occluder (Gore Medical [WL Gore & Associates, Inc]; Flagstaff, Ariz), and Sideris patch (Custom Medical Devices; Amarillo, Tex). The ASO is currently the most widely used device because it is easy to implant and allows closure of large orifices with excellent success rates in most cases. It was first used in humans in 1995. Selection of a particular device is difficult because no randomized trials have been conducted. Furthermore, devices are currently not amenable to percutaneous closure of ostium primum and sinus venosus defects.
With this technique, the static diameter of the defect is first assessed by using TEE, and the stretched diameter is then measured with a sizing balloon to select the proper diameter of the device. The margins of the orifice must be wide enough to accommodate the edges of the closing device. TEE has been the mainstream technique for device sizing, positioning, and deployment, but it can cause discomfort. In addition, airway protection and general anesthesia are required. Intracardiac echocardiography has been used for the same purpose.
Transcatheter closure of ASDs is now established practice at most cardiac centers. It is proven safe in experienced hands, it is cost-effective, and it favorably compares to surgical closure with successful implantation rates of more than 96%. Transcatheter closure has been associated with fewer complications, shortened hospitalization, and reduced need for blood products.
Furthermore, transcatheter closure appears to have additional benefits regarding hemodynamic improvement compared with surgery. In one study, transcatheter closure with ASO improved the left atrial volume index, the left ventricular myocardial performance index, and the right ventricular myocardial performance index.5 The last was unimpressive after surgery, possibly because of cardiopulmonary bypass.
Another group compared atrial function in 45 patients with a mean age of 9 years after surgery and after percutaneous closure by using strain-rate imaging.6 They found that both atrial functions were preserved after transcatheter closure, whereas the same was not seen after surgery. A potential explanation was that an atriotomy scar might have negatively influenced right atrial functio, whereas perioperative hypoxia or intraoperative myocardial damage might have altered the deformation properties of the left atrium.
Postoperative Details
Postoperative management after atrial septal defect (ASD) repair is usually standard. Patients are expected to be awake and often extubated shortly after the operation. Drainage tubes are removed from the chest the first morning after surgery, and, except when rhythm problems occur, the pacing wires are removed shortly thereafter. Most patients can eat and ambulate without difficulty on the first or second postoperative day, and most are discharged by the third or fourth postoperative day. After transcatheter occlusion, patients are generally discharged the next day. Six months of treatment with aspirin with or without clopidogrel is recommended to prevent thrombus formation.
Follow-up
Surgical follow-up care is maintained until the patient's wounds are completely healed and normal activities are resumed. This period rarely exceeds 1-2 months. All complications must be clearly resolved before the patient is discharged from surgical care.
Obtain at least 1 follow-up echocardiogram to confirm complete closure of the ASD. A cardiologist with congenital experience should continue patient care to monitor for recurrence of the shunt and to ensure that the patient has returned to normal activities and cardiac function. For most patients, a yearly appointment after the immediate postoperative period is adequate, in large part to follow and evaluate for arrhythmia complications.
For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Palpitations.
Complications
Surgery may be associated with a long-term risk of atrial fibrillation or flutter. The risk of infective endocarditis exists during the first 6 months after surgery. The following complications are also associated with atrial septal defect (ASD):
- Congestive heart failure
- Arrhythmias
- Pulmonary hypertension
- Cyanosis
- Paradoxical embolization
- Stroke
- Infective endocarditis
The following complications are specifically associated with the use of transcatheter occlusion devices:
- Device embolization and malpositioning: The incidence significantly varies among devices and is related to operator experience and appropriate case selection. With experienced clinicians, the incidence is less than 1%. Device embolization and malpositioning happens as a result of inadequate sizing of the defect or incorrect device placement.
- Postimplantation arrhythmias: The incidence is 1-4% and varies from first- to third-degree AV block and atrial fibrillation. These arrhythmias are usually short-lived and do not require medical treatment. Patients who develop complete heart block are typically hemodynamically stable and do not require pacing. The incidence of arrhythmia appears to be related to the size of the device.
- Thrombus formation: A study of 1000 patients was performed to investigate the incidence of thrombus by performing TEE at 4 weeks and 6 months after the procedure.7 The overall incidence was 1.2%; 70% were found at 4 weeks. The lowest incidence was with ASO. Thromboembolic events were seen in 20% of patients with thrombus. In 80-85%, thrombus resolved with heparin and warfarin. Risk factors for thrombogenesis on the device were the type of device, postprocedural atrial fibrillation, incomplete neoendothelialization of the surface of the device, insufficient antithrombotic treatment, and previously undiagnosed hypercoagulability disorders (including aspirin resistance and persistent interatrial septal aneurysm). For prophylaxis, aspirin given for 6 months is a common practice. When combined with clopidogrel, no thrombus was noted at 4 weeks and 6 months in one study of 37 patients.8
- Cardiac perforation: The incidence is 0.1-0.4% for various devices. Oversizing of the device and deficient anterosuperior rims are risk factors for perforation. Technique-related perforation during the procedure is amenable to intervention. Device-related perforations occur after technically adequate procedure in approximately 70% of patients after hospital discharge. A retrospective review of 24 patients revealed that all presented with chest pain, shortness of breath, hemodynamic collapse, or sudden death. About 76% were female patients, and 70% of the perforations were late. If pericardial effusion is present on predischarge echocardiography, the patient should be hospitalized for 24-48 hours of observation and follow-up echocardiography.9
- Device erosion: Erosion of septal occluder devices occurs in 0.1-0.15% of implants.10 Nearly all erosions occur at the dome of atria near the aortic root. Risk factors are a deficient aortic rim and/or superior rim and use of an oversized device. Aortic–right atrial fistula may be the consequence. Although device erosion is rare, the mortality rate is 10%.
- Increased levels of cardiac troponin I: Transcatheter closure induces minor myocardial lesions, the extent of which depends on the size of the ASO. The patient's age is not a factor.11
- Residual shunts: As many as 20% of patients may have a residual shunt persisting for 24 hours after the procedure; >90% of such residua are small. During follow-up, the incidence approaches 0% at 3 years for most series.12,13
- Other complications include pericardial effusion, transient ischemic attack, and sudden death.
More on Atrial Septal Defect |
| Overview: Atrial Septal Defect |
| Workup: Atrial Septal Defect |
Treatment: Atrial Septal Defect |
| Follow-up: Atrial Septal Defect |
| Multimedia: Atrial Septal Defect |
| References |
| « Previous Page | Next Page » |
References
Li QY, Newbury-Ecob RA, Terrett JA, Wilson DI, Curtis AR, Yi CH. Holt-Oram syndrome is caused by mutations in TBX5, a member of the Brachyury (T) gene family. Nat Genet. Jan 1997;15(1):21-9. [Medline].
Ruiz-Perez VL, Ide SE, Strom TM, Lorenz B, Wilson D, Woods K. Mutations in a new gene in Ellis-van Creveld syndrome and Weyers acrodental dysostosis. Nat Genet. Mar 2000;24(3):283-6. [Medline].
Benson DW, Silberbach GM, Kavanaugh-McHugh A, Cottrill C, Zhang Y, Riggs S. Mutations in the cardiac transcription factor NKX2.5 affect diverse cardiac developmental pathways. J Clin Invest. Dec 1999;104(11):1567-73. [Medline].
[Guideline] Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation. Dec 2 2008;118(23):2395-451. [Medline].
Salehian O, Horlick E, Schwerzmann M, Haberer K, McLaughlin P, Siu SC. Improvements in cardiac form and function after transcatheter closure of secundum atrial septal defects. J Am Coll Cardiol. Feb 15 2005;45(4):499-504. [Medline].
Di Salvo G, Drago M, Pacileo G, Rea A, Carrozza M, Santoro G, et al. Atrial function after surgical and percutaneous closure of atrial septal defect: a strain rate imaging study. J Am Soc Echocardiogr. Sep 2005;18(9):930-3. [Medline].
Krumsdorf U, Ostermayer S, Billinger K, Trepels T, Zadan E, Horvath K. Incidence and clinical course of thrombus formation on atrial septal defect and patient foramen ovale closure devices in 1,000 consecutive patients. J Am Coll Cardiol. Jan 21 2004;43(2):302-9. [Medline].
Brandt RR, Neumann T, Neuzner J, Rau M, Faude I, Hamm CW. Transcatheter closure of atrial septal defect and patent foramen ovale in adult patients using the Amplatzer occlusion device: no evidence for thrombus deposition with antiplatelet agents. J Am Soc Echocardiogr. Oct 2002;15(10 Pt 1):1094-8. [Medline].
Divekar A, Gaamangwe T, Shaikh N, Raabe M, Ducas J. Cardiac perforation after device closure of atrial septal defects with the Amplatzer septal occluder. J Am Coll Cardiol. Apr 19 2005;45(8):1213-8. [Medline].
Amin Z, Hijazi ZM, Bass JL, Cheatham JP, Hellenbrand WE, Kleinman CS. Erosion of Amplatzer septal occluder device after closure of secundum atrial septal defects: review of registry of complications and recommendations to minimize future risk. Catheter Cardiovasc Interv. Dec 2004;63(4):496-502. [Medline].
Tarnok A, Bocsi J, Osmancik P, Hausler HJ, Schneider P, Dahnert I. Cardiac troponin I release after transcatheter atrial septal defect closure depends on occluder size but not on patient's age. Heart. Feb 2005;91(2):219-22. [Medline].
Egred M, Andron M, Albouaini K, Alahmar A, Grainger R, Morrison WL. Percutaneous closure of patent foramen ovale and atrial septal defect: procedure outcome and medium-term follow-up. J Interv Cardiol. Oct 2007;20(5):395-401. [Medline].
Fischer G, Stieh J, Uebing A, Hoffmann U, Morf G, Kramer HH. Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder: a single centre study in 236 consecutive patients. Heart. Feb 2003;89(2):199-204. [Medline].
Anzai H, Child J, Natterson B, Krivokapich J, Fishbein MC, Chan VK. Incidence of thrombus formation on the CardioSEAL and the Amplatzer interatrial closure devices. Am J Cardiol. Feb 15 2004;93(4):426-31. [Medline].
Argenziano M, Oz MC, DeRose Jr JJ, et al. Totally endoscopic atrial septal defect repair with robotic assistance. The Heart Surgery Forum. 2001;5(3):[Full Text].
Bartel T, Konorza T, Arjumand J, et al. Intracardiac echocardiography is superior to conventional monitoring for guiding device closure of interatrial communications. Circulation. Feb 18 2003;107(6):795-7. [Medline].
Bartel T, Konorza T, Neudorf U, Ebralize T, Eggebrecht H, Gutersohn A. Intracardiac echocardiography: an ideal guiding tool for device closure of interatrial communications. Eur J Echocardiogr. Mar 2005;6(2):92-6. [Medline].
Bedford DE. The anatomical types of atrial septal defect. Their incidence and clinical diagnosis. Am J Cardiol. Sep 1960;6:568-74. [Medline].
Benson DW, Sharkey A, Fatkin D, Lang P, Basson CT, McDonough B. Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects. Circulation. May 26 1998;97(20):2043-8. [Medline].
Besterman E. Atrial septal defect with pulmonary hypertension. Br Heart J. Sep 1961;23(5):587-98. [Medline].
Bialkowski J, Karwot B, Szkutnik M, Banaszak P, Kusa J, Skalski J. Closure of atrial septal defects in children: surgery versus Amplatzer device implantation. Tex Heart Inst J. 2004;31(3):220-3. [Medline].
Braunwald E. Atrial septal defect. In: Braunwald E, ed. Heart Disease: A Text of Cardiovascular Medicine. 1992. 4th ed. Philadelphia, Pa: WB Saunders; 1992:906-8.
Carlsson E. Anatomic diagnosis of atrial septal defects. Am J Roentgenol Radium Ther Nucl Med. Jun 1961;85:1063-70. [Medline].
Cherian G, Uthaman CB, Durairaj M, et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. Am Heart J. Jun 1983;105(6):952-7. [Medline].
Chessa M, Carminati M, Butera G, et al. Early and late complications associated with transcatheter occlusion of secundum atrial septal defect. J Am Coll Cardiol. Mar 20 2002;39(6):1061-5. [Medline].
Goldman L, Braunwald E. Primary Cardiology. Philadelphia, Pa: WB Saunders; 1998:394-411.
Holmvang G, Palacios IF, Vlahakes GJ, et al. Imaging and sizing of atrial septal defects by magnetic resonance. Circulation. Dec 15 1995;92(12):3473-80. [Medline].
Isselbacher KJ, Braunwald E, Wilson JD. Atrial septal defect. In: Isselbacher KJ, ed. Harrison's Principles of Internal Medicine. 1994. 13th ed. New York, NY: McGraw-Hill; 1994:1041.
Jost CH, Connolly HM, Danielson GK, et al. Sinus venosus atrial septal defect: long-term postoperative outcome for 115patients. Circulation. Sep 27 2005;112(13):1953-8.
Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. London, England: Churchill Livingstone; 1986:463-97.
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. Aug 24 1995;333(8):469-73. [Medline].
Kronzon I, Tunick PA, Freedberg RS, et al. Transesophageal echocardiography is superior to transthoracic echocardiography in the diagnosis of sinus venosus atrial septal defect. J Am Coll Cardiol. Feb 1991;17(2):537-42. [Medline].
Latson LA. Per-catheter ASD closure. Pediatr Cardiol. Jan-Feb 1998;19(1):86-93; discussion 94. [Medline].
Marelli AJ, Moodie DS, Topol EJ. Adult congenital heart disease. In: Textbook of Cardiovascular Medicine. Philadelphia, Pa: Lippincott-Raven; 1998:775-9.
Masura J, Gavora P, Podnar T. Long-term outcome of transcatheter secundum-type atrial septal defect closure using Amplatzer septal occluders. J Am Coll Cardiol. Feb 15 2005;45(4):505-7.
Moore KL, Persaud TVN. Before We Are Born: Essentials of Embryology and Birth Defects. Philadelphia, Pa: WB Saunders Co; 1998:774-7.
Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. Aug 26 1993;329(9):593-9. [Medline].
O'Laughlin MP. Catheter closure of secundum atrial septal defects. Tex Heart Inst J. 1997;24(4):287-92. [Medline].
Piéchaud JF. Closing down: transcatheter closure of intracardiac defects and vessel embolisations. Heart. Dec 2004;90(12):1505-10. [Medline].
Rao PS. Catheter closure of atrial septal defects. J Invasive Cardiol. Jul 2003;15(7):398-400. [Medline].
Ruge H, Wildhirt SM, Libera P, Vogt M, Holper K, Lange R. Images in cardiovascular medicine. Left atrial thrombus on atrial septal defect closure device as a source of cerebral emboli 3 years after implantation. Circulation. Sep 6 2005;112(10):e130-1. [Medline].
Sealy WC, Farmer JC, Young Jr WG, et al. Atrial dysrhythmia and atrial secundum defects. J Thorac Cardiovasc Surg. Feb 1969;57(2):245-50. [Medline].
Seward JB, Khandheria BK, Edwards WD, et al. Biplanar transesophageal echocardiography: anatomic correlations, image orientation, and clinical applications. Mayo Clin Proc. Sep 1990;65(9):1193-213. [Medline].
Staniloae CS, El-Khally Z, Ibrahim R, et al. Percutaneous closure of secundum atrial septal defect in adults a single center experience with the amplatzer septal occluder. J Invasive Cardiol. Jul 2003;15(7):393-7. [Medline].
Stark J. Secundum atrial septal defect. In: Surgery for Congenital Heart Defects. New York, NY: Grune & Stratton; 1983.
Tardif JC, Schwartz SL, Vannan MA, et al. Clinical usefulness of multiplane transesophageal echocardiography: comparison to biplanar imaging. Am Heart J. Jul 1994;128(1):156-66. [Medline].
Thuny F, Di Salvo G, Belliard O, Avierinos JF, Pergola V, Rosenberg V, et al. Risk of embolism and death in infective endocarditis: prognostic value of echocardiography: a prospective multicenter study. Circulation. Jul 5 2005;112(1):69-75. [Medline].
Tonni G, Ferrari B, Defelice C, Centini G. Neonatal porencephaly in very low birth weight infants: ultrasound timing of asphyxial injury and neurodevelopmental outcome at two years of age. J Matern Fetal Neonatal Med. Dec 2005;18(6):361-5. [Medline].
Vick GW 3rd, Murphy DJ Jr, Ludomirsky A, et al. Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography. J Am Coll Cardiol. Mar 1987;9(3):580-7. [Medline].
Warnes CA. The adult with congenital heart disease: born to be bad?. J Am Coll Cardiol. Jul 5 2005;46(1):1-8. [Medline].
Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. Circulation. Oct 10 2006;114(15):1645-53. [Medline].
Weyman AE, Wann LS, Caldwell RL, et al. Negative contrast echocardiography: a new method for detecting left-to- right shunts. Circulation. Mar 1979;59(3):498-505. [Medline].
Zanchetta M, Onorato E, Rigatelli G, Pedon L, Zennaro M, Carrozza A. Intracardiac echocardiography-guided transcatheter closure of secundum atrial septal defect: a new efficient device selection method. J Am Coll Cardiol. Nov 5 2003;42(9):1677-82. [Medline].
Further Reading
Keywords
atrial septal defect, atrial septum defect, ASD, atrial septal defect repair, atrial septal defect closure, atrial septal defect surgery, atrial septal defect symptoms
Treatment: Atrial Septal Defect