Version July 2025
INTRODUCTION —
Congenital defects of the atrial septum are common, accounting for approximately 7 percent of congenital heart disorders [1]. They can occur in several different anatomic portions of the atrial septum, and the location of the defect generally reflects the abnormality of embryogenesis that led to the anomaly (figure 1). An atrial septal defect (ASD) can be isolated or can be associated with other congenital cardiac abnormalities. The sizes of ASDs are variable, and their functional consequences are related to the anatomic location of the defect, its size, and the presence or absence of other cardiac anomalies, as well as systolic and diastolic function of the ventricles.
This topic will review the management and outcomes of children with an isolated ASD, focusing on management of secundum ASDs, which are the most common type of isolated ASD. The classification of ASDs and the pathophysiology, clinical features, and diagnosis of isolated ASD in children are discussed separately. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis".)
GENERAL CONSIDERATIONS —
Important considerations in the management of a child with an isolated ASD include:
●Size, degree of left-to-right shunting, and persistence of the ASD
●Likelihood of spontaneous closure
●When closure is indicated, deciding between transcatheter and surgical closure
Atrial septal defect size — For the purpose of the discussion in this topic review, we generally define the size of isolated ASDs as follows:
●Trivial – <3 mm in diameter
●Small – 3 to <6 mm in diameter
●Moderate – 6 to 8 mm in diameter
●Large – >8 mm in diameter
While these absolute size criteria provide useful guidelines for categorizing the size of the defect, they have important limitations and should be interpreted in the context of the patient's size. The relative size of the defect (relative to overall heart size) is more clinically relevant. For example, a 2.5-mm ASD is generally trivial in a five year old but may be clinically significant in a small preterm infant. In addition, the echocardiographic technique of measuring the size of the defect has some inherent limitations. Acoustic windows are variable, young patients may be uncooperative, and there can be substantial interobserver and intraobserver variability. ASDs are often elliptical in shape rather than circular and thus measuring the diameter depends on where the measurement is made. Morphology of the septum is also an important consideration. The flap valve of the fossa (septum primum) may or may not be visible, and this should be a factor in distinguishing between a very small secundum ASD and a patent foramen ovale (PFO). If an aneurysm of the septum primum is present, this can further complicate measuring the size of the defect.
Measurement of shunt ratio — The main indication for ASD closure has been the presence of a left-to-right shunt sizable enough to result in clinically significant right heart overload. This typically occurs in patients with moderate to large ASDs. The usual clinical standard for ASD closure is a measured shunt ratio of pulmonary-to-systemic flow (Qp/Qs) >2:1, though high-quality evidence supporting this threshold is generally lacking [2]. Other experts in the field have advocated lower thresholds of 1.7:1 and 1.5:1 [3-5].
Cardiac catheterization is considered the gold standard for measuring Qp/Qs, but noninvasive techniques are accepted alternatives in experienced centers. Phase contrast magnetic resonance imaging (MRI) in experienced hands appears to be at least as good as the standard Fick technique for measuring shunt flow with blood samples obtained at catheterization [6,7]. Pulsed Doppler echocardiography is now routinely used in sites that have established an acceptable correlation between echocardiographic and catheterization laboratory or MRI estimates of shunt flow. Because echocardiography and MRI are noninvasive, these techniques easily permit serial monitoring. The technical aspects of measuring Qp/Qs are discussed separately. (See "Clinical manifestations and diagnosis of atrial septal defects in adults", section on 'Approach to diagnosis and evaluation'.)
If quantitative measures of Qp/Qs are not available, other echocardiographic findings that are sufficient to justify ASD closure include:
●Right atrial and/or ventricular enlargement
●Qualitative color and spectral Doppler findings of a large amount of left-to-right shunting through the ASD.
Other findings that suggest substantial left-to-right shunting include the following, though in isolation, none of these are sufficient criteria for intervention:
●Right ventricular hypertrophy on electrocardiogram
●Audible tricuspid flow rumble by an experienced clinician
●Cardiomegaly and increased pulmonary vascular markings on chest radiograph
Spontaneous closure — Most isolated small secundum ASDs close spontaneously by two years and some as late as by five years of age [8-10]. Thus, in the absence of associated symptoms, early closure is not indicated for these defects. Secundum defects of moderate and large size, and types of ASDs other than secundum, are unlikely to close spontaneously. However, because there is a possibility, albeit small, of spontaneous closure during the first two years of life, intervention to close even moderate and large isolated secundum ASDs is generally deferred until after the age of two years in asymptomatic patients. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Spontaneous closure'.)
MANAGEMENT APPROACH —
Key aspects of managing children with isolated ASDs include deciding whether ASD closure is warranted, determining the choice and timing of intervention, and providing post-procedure follow-up (algorithm 1). The guidance in the following sections is generally consistent with guidance from the American College of Cardiology [11].
Monitoring — Secundum ASDs or patent foramen ovales (PFOs) that are diagnosed in infancy or early childhood often come to light because echocardiography was performed to evaluate a heart murmur or other nonspecific indication. In most cases, the defect will close spontaneously during the first few years of life. Monitoring of these patients and patients with defects other than secundum ASDs who are diagnosed prenatally depends upon the size and type of the defect. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Presentation' and "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Diagnosis'.)
●Patent foramen ovale (PFO) versus ASD – PFO and small ASDs constitute a spectrum, and there is not general consensus on how best to differentiate between them.
•Many cardiologists base the distinction between the two entities on the presence or absence of a tissue flap partially occluding the opening of the atrial septum. Openings with a tissue flap are defined as PFO, and those without a tissue flap are defined as secundum ASD. The absence of a tissue flap is usually associated with a "T artifact," a hyperintense blunted edge at the margin of the defect in the atrial septum on echocardiography.
•Other cardiologists use defect size or position in the atrial septum to differentiate between ASD and PFO. Caution should be employed when using absolute defect diameter for classification in premature infants because the physiologic effect of defect size varies with patient size.
Because of the lack of uniformity in subjective interpretations of echocardiograms, and because pediatric cardiology practice standards differ, primary care clinicians should seek pediatric cardiology advice regarding the necessity for follow-up when receiving an echocardiographic report identifying an ASD or a borderline classification such as "PFO/ASD."
●Follow-up for PFO – If a patient has an isolated defect characterized as PFO on the echocardiographic report, follow-up is generally not necessary. Based on the available evidence, medical follow-up of asymptomatic patients with PFO does not appear to have any benefit [11,12]. Many PFOs spontaneously close during childhood and adolescence, and most of the PFOs that persist into adulthood never cause problems. Requiring routine follow-up for such children may cause undue anxiety regarding rare complications that can be associated with PFO later in adulthood (eg, paradoxical embolus and decompression illness). Furthermore, given the high prevalence of PFO, medical follow-up of all PFOs would be burdensome on a population basis.
●Follow-up for trivial secundum ASDs – Similar to PFO, children with secundum ASDs that are characterized as "trivial" in the echocardiogram report do not require specific follow-up.
●Follow-up for clinically significant ASDs – If a clinically significant ASD is detected on echocardiogram, the child should be followed regularly by a pediatric cardiologist [11,12]. Clinically significant ASDs include small, moderate, and large secundum ASDs as well as primum ASDs and other less common types of ASDs (sinus venosus or coronary sinus defects). Experience has shown that secundum ASDs ≥3 mm can increase substantially with patient growth [13-15].
The history and physical examination during follow-up evaluations focuses on detecting any signs or symptoms of increased shunting. Signs suggestive of increased shunting include failure to gain weight appropriately, increased frequency of respiratory infections, and, rarely, signs of heart failure such as hepatomegaly or auscultatory evidence of pulmonary congestion. Echocardiography is indicated when there is concern that the shunt may be increasing or to confirm spontaneous closure.
Indications for ASD closure — There are two main indications for performing an ASD closure procedure. The first of these indications is by far the more common scenario in pediatric patients.
●Substantial left-to-right shunting – We recommended ASD closure for children who have signs of substantial left-to-right shunting through an ASD, including any of the following:
•Right atrial and/or ventricular enlargement on echocardiogram
•Signs or symptoms of pulmonary overcirculation (pulmonary edema, heart failure)
•High ratio of pulmonary-to-systemic blood flow (Qp/Qs) – The usual threshold is Qp/Qs >2:1, though some experts use lower thresholds, as discussed above (see 'Measurement of shunt ratio' above)
Findings of significant left-to-right shunting are typically associated with moderate to large ASDs. Such defects are unlikely to close spontaneously, and observational data suggest that left-to-right shunting may increase with age, resulting in heart failure, exercise intolerance, and increased risk of arrhythmia and pulmonary hypertension. This approach is also supported by indirect evidence from a randomized trial in adults with ASD demonstrating lower risk of death or major cardiovascular events in patients managed with ASD closure compared with medical management [3]. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Natural history' and "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Outcomes of surgical closure'.)
●History of embolic stroke – Young patients with ASDs who have experienced an embolic stroke with no other identified cause are generally considered candidates for ASD closure [11]. Paradoxical embolism is uncommon in pediatric patients. It occurs more commonly in adults, as discussed separately. (See "Atrial septal abnormalities (PFO, ASD, and ASA) and risk of cerebral emboli in adults" and "Stroke associated with patent foramen ovale (PFO): Management", section on 'Patient selection for PFO closure'.)
For patients with moderate-sized ASDs who are asymptomatic and lack evidence of significant shunting, the indications for closure of are less clear. Some cardiologists manage such patients expectantly with ongoing monitoring, whereas others may offer transcatheter closure to select patients in this category. The optimal approach is uncertain. If a noninterventional approach is selected, periodic follow-up is warranted since shunt volume can increase with age [16].
For asymptomatic patients with persistent small ASDs, we suggest ongoing monitoring rather than performing a closure procedure (see 'Monitoring' above). There is no definitive evidence that ASD closure is beneficial for patients with small ASDs in the absence of significant left-to-right shunting or complications directly attributable to the ASD (including paradoxical embolism or platypnea-orthodeoxia syndrome, which is a rare disorder manifested by shortness of breath and hypoxemia that occurs when sitting or standing and that is relieved when lying down). (See "Patent foramen ovale", section on 'Platypnea-orthodeoxia syndrome'.)
No studies have shown that preemptive closure of small ASDs in an asymptomatic patient prevents paradoxical embolization, and the risk of endocarditis with isolated small ASDs appears to be negligible. Most other pathologic consequences of ASD, such as atrial arrhythmias and (rarely) pulmonary hypertension, are secondary to right heart volume overload or pulmonary overcirculation. Since all closure techniques have risk, closure of asymptomatic small ASDs in childhood is not warranted. ASD closure may be warranted later in life, particularly in adult patients with a small ASD or PFO who have experienced embolic strokes or transient ischemic attacks. These issues are discussed separately. (See "Management of atrial septal defects in adults", section on 'ASD closure' and "Stroke associated with patent foramen ovale (PFO): Management".)
Timing of ASD closure — ASD closure is generally deferred until after age two years in asymptomatic patients because there is a possibility, albeit small, of spontaneous closure during the first two years of life, as discussed above. (See 'Spontaneous closure' above.)
In addition, deferring the closure procedure until after the age of two years allows time for the child to grow, which will make them a better candidate for transcatheter ASD closure should a closure procedure be necessary. Although successful transcatheter closure can be performed in small infants and children weighing <15 kg, the complication rate is higher in this patient group compared with older/larger patients [17-19]. Thus, the benefits of closure in infants and young children (weighing <15 kg) usually do not outweigh the increased risk of complications and possibility of spontaneous closure. (See 'Transcatheter closure' below.)
Occasionally, an infant or young child may present with signs of heart failure due to a very large ASD. In these patients, early surgical closure is generally the preferred approach. (See 'Indications for surgical closure' below and 'Surgery' below.)
Preprocedure evaluation — Prior to selecting an intervention, a comprehensive echocardiographic evaluation is performed. Important aspects of the evaluation include:
●Accurately measuring the size of the defect and degree of left-to-right shunting
●Identifying other cardiac defects
●Assessing for pulmonary hypertension
The preprocedural echocardiogram helps to determine the optimal choice of intervention. For example, severe fixed pulmonary hypertension is a contraindication for ASD closure, though this is a rare finding in children with ASDs.
Echocardiographic evaluation of ASDs is discussed in greater detail separately. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Echocardiography' and "Clinical manifestations and diagnosis of atrial septal defects in adults", section on 'Echocardiography'.)
Choice of closure intervention — The choice of intervention (transcatheter versus surgical closure) depends on the type of the ASD, whether there are any other cardiac defects, and the expertise at the center.
Transcatheter closure (preferred for most patients) — For most patients with isolated secundum ASDs with a clinical indication for closure, transcatheter device closure is the preferred intervention, provided that the ASD anatomy is favorable. (See 'Transcatheter closure' below.)
Favorable anatomy for transcatheter closure of a secundum ASD requires both of the following:
●The ASD is not excessively large (ie, <30 mm in diameter), and
●There is a rim of tissue around the defect sufficient for effective closure without obstruction or impingement on adjacent cardiac structures (at least 5 mm)
For patients with secundum ASDs meeting these criteria, we suggest transcatheter closure rather than surgical closure. However, criteria vary among centers, based on the institution's level of expertise.
Larger secundum defects are generally closed surgically, although successful transcatheter closure of defects >30 mm has been described. Transcatheter closure requires an adequate rim of tissue around the defect (at least 5 mm) to adequately anchor the device and prevent obstruction of the coronary sinus, right pulmonary veins, vena cavae, or atrioventricular valves. The insertion of more than one device to close multiple ASDs is safe and effective if the patient is large enough (ideally >20 kg) and the distance between the defects is adequate (at least 7 mm) [20].
Transcatheter closure is rarely used in pediatric patients with sinus venosus, primum, or coronary sinus ASDs, or in infants with large defects and heart failure. Successful transcatheter closure of sinus venosus ASDs has been accomplished in adult patients in centers with specialized expertise using covered intravascular stents and preprocedural planning with cross-sectional imaging and three-dimensional modeling [21,22]. However, pediatric experience with transcatheter closure of sinus venous ASDs is lacking. Experience using covered stents for coronary sinus ASD closure in pediatric patients is limited to a single case report [23].
Indications for surgical closure — Surgical closure is reserved for patients with any of the following (see 'Surgery' below):
●Secundum ASD with unfavorable anatomy (ie, not meeting the criteria above)
●Sinus venosus defects, coronary sinus defects, primum ASDs, and complex congenital lesions
●Infants with heart failure due to a large ASD
●Patient or parents/caregivers prefer surgical closure
Comparative data — Observational data suggest that procedural success rates are similarly high for both transcatheter and surgical closure, but complication rates and length of hospital stay are lower with the transcatheter approach [24]. There may be a slightly increased risk of needing reintervention with transcatheter closure.
Outcomes with the two approaches were described in a multicenter prospective observational study of 442 children and young adults who underwent transcatheter closure with the Amplatzer device (median age 9.8 years) and 154 patients who underwent surgery (median age 4.1 years); the choice of procedure was based on the family's preference [25]. There were no deaths in either group. The procedural success rate was slightly lower with transcatheter closure compared with surgery (96 versus 100 percent), but the complication rate was lower (7 versus 24 percent) and hospital stay was shorter (1 versus 3.4 days). At 12-month follow-up, five patients (1.5 percent) in the device-closure group had a residual shunt, one of whom required a second procedure to correct the residual shunt.
In a systematic review and meta-analysis of 13 observational studies (including >3000 adult and pediatric patients) comparing short-term outcomes with transcatheter (n = 1812) or surgical (n = 1270) ASD closure, mortality was low in both groups (no deaths in the transcatheter group and one death in the surgical group) [24]. Length of hospital stay was 2.6 days shorter in the transcatheter group. Early complication rates were lower in the transcatheter group compared with the surgical group (7 versus 31 percent). The types of major complications differed in the two groups. The most common major early complication in the surgical group was bleeding requiring blood transfusion, which occurred in 7 percent, whereas blood transfusion was rarely required in the transcatheter group (<1 percent of patients). Redo surgery was performed in nine surgical patients (five for patch dehiscence, and four for major bleeding). The most frequent major early complication in the transcatheter group was device embolization or malposition needing surgery or further transcatheter therapy, which occurred in 1.6 percent. Other major complications included perforation of the heart due to erosion by the device (three patients), pericardial effusion requiring surgical drainage (two patients), and femoral venous or arterial injury requiring vascular surgery (four patients).
CLOSURE PROCEDURES —
The following sections describe procedural details of transcatheter and surgical ASD closure. Indications for ASD closure and the choice of procedure are discussed above (algorithm 1). (See 'Indications for ASD closure' above and 'Choice of closure intervention' above.)
Transcatheter closure — Transcatheter ASD closure avoids cardiopulmonary bypass, sternotomy or thoracotomy, and atriotomy and is associated with excellent outcomes. As a result, this approach has largely replaced surgery in many centers for the closure of secundum ASDs that have appropriate anatomic characteristics [26]. (See 'Transcatheter closure (preferred for most patients)' above.)
Devices — In the United States, devices that have been approved by the US Food and Drug Administration for transcatheter closure of secundum ASDs include [27,28]:
●The Amplatzer Septal Occluder (figure 2). The Amplatzer Cribiform Multifenestrated Septal Occluder was developed for treating multifenestrated ASDs [21,29].
●The Gore Cardioform Septal Occluder (for defects up to 17 mm) (picture 1) and ASD Occluder (for defects 8 to 35 mm). The predecessor device (Gore HELEX) is no longer available.
●The Cardio-Seal Septal Occlusion System and its next-generation version (Starflex), both of which are no longer available.
Intraprocedural echocardiography — Transesophageal echocardiography (TEE) or intracardiac echocardiography (ICE) can be used during the procedure to assist in selecting an appropriate ASD closure device and to facilitate the deployment of the device.
TEE can be used to accurately measure the size and location of the ASD. In addition, TEE can be used to guide the procedure in real time, an approach that may minimize fluoroscopy time [30]. Two- or even three-dimensional TEE is particularly helpful when multiple devices are used [20].
ICE is a modality that is particularly well suited for evaluating ASD anatomy and assisting in ASD device placement [31-35]. ICE facilitates proper device selection by providing an accurate assessment of the size and shape (typically, elliptical) of the defect [33].
ICE has the following advantages compared with TEE:
●It does not require endotracheal intubation or general anesthesia [32,35]
●It permits continuous monitoring during the procedure
●It is associated with shorter procedure duration [35]
Procedural success rates — Reported procedural success rates of transcatheter closure using the different available devices range from 88 to 98 percent [25,36-41].
●Amplatzer Septal Occluder – In a prospective multicenter postapproval study of 1000 patients (mean age 21 years) who underwent secundum ASD closure with the Amplatzer Septal Occluder at 50 sites in the United States with follow-up for two years, the procedural closure rate was 97.9 percent, which was unchanged at two years post-procedure [41].
Similarly, a multicenter study from France including >1300 attempted transcatheter ASD closure procedures using the Amplatzer Septal Occluder reported an overall procedural success rate of 95.3 percent with no deaths or cardiac erosion over a medium follow-up period of 3.5 years [17].
Although clinical indications for ASD closure are uncommon in infancy, the Amplatzer device has been successfully used in patients weighing <8 kg [18,19]. Adverse events are more common in smaller patients and may include transient arrhythmias and need for blood transfusion. Major complications are rare and include thrombosis of the inferior vena cava due to vascular intimal injury, systemic blood flow impairment, and pulmonary edema.
●Gore Cardiform Septal and ASD Occluders – In a multicenter retrospective study of 173 consecutive pediatric patients (mean age six years) who successfully underwent secundum ASD closure with the Gore Septal Occluder, 95.4 percent had confirmed complete closure of the ASD at median follow-up of 20 months [42]. The remaining 4.6 percent of patients had small residual shunts that did not require reintervention. No serious adverse events were reported. A previous multicenter study reported similar results [43,44].
In a prospective multicenter study evaluating the Gore Cardiform ASD occluder in 125 patients with ASDs not suitable for device closure with the Gore Septal Occluder (due to ASD diameter ≥18 mm, deficient retroaortic rim, or both), technical success was achieved in 96 percent, although three devices were removed. Of the 112 patients who returned for six-month follow-up, all demonstrated successful ASD closure [45,46].
Data from single-arm clinical trials demonstrated that the predecessor of the Gore Septal and ASD Occluders (HELEX) appeared to be best suited for small to moderate secundum ASDs (stretch diameter <20 mm) with reported success rates of approximately 98 percent at one-year follow-up [36]. Device wire frame fracture occurred in 8 percent of deployments, but only one patient required treatment [36]. The HELEX device is no longer available.
Complications — Periprocedural complications associated with transcatheter ASD closure include device embolization or malposition and arrhythmias (usually atrial) [25,37,38,41,47-49]. In a prospective multicenter postapproval study in 1000 patients (mean age 21 years) who underwent secundum ASD closure with the Amplatzer device at 50 sites in the United States with follow-up for two years, complications occurred in 6.6 percent of patients [41]. The most frequently reported complications included pericardial effusion (3.1 percent), migraine headaches (1.9 percent), atrial arrhythmia (1.2 percent), other dysrhythmia (0.2 percent), device embolization (0.6 percent), cardiac erosion (0.3 percent), and thrombus (0.1 percent). There were no deaths related to the procedure or the device.
In a meta-analysis of 13 observational studies, the overall rate of complications (including major and nonmajor complications) following transcatheter ASD closure was 6.6 percent; major complications occurred in 1.9 percent of cases [24]. The most frequent major early complication was device embolization or malposition needing surgery or further transcatheter therapy, which occurred in 1.6 percent. Other rare major complications included perforation of the heart due to erosion by the device, pericardial effusion requiring surgical drainage, and femoral venous or arterial injury requiring vascular surgery.
In one multicenter study including 1326 attempted transcatheter ASD closure procedures using the Amplatzer Septal Occluder, 1.8 percent of patients experienced major periprocedural complications, which included device migration, pericardial effusion, conduction abnormality (ie, arrhythmia or heart block), transient hemolysis, and air embolism [17]. Risk factors for complications included weight ≤15 kg and ASD diameter ≥20 mm.
Cardiac perforation, which can present as hemopericardium, pericardial effusion, cardiovascular collapse, or sudden death, is an uncommon complication of the Amplatzer Septal Occluder [50]. This complication has been reported as early as the first day and as late as 15 years after the procedure [51-53]. Erosion leading to fatal tamponade has been very rarely reported with other devices as well [54,55].
Late complications are uncommon. In a report of 417 patients who underwent transcatheter ASD closure, there was one case of peripheral embolization at one year and one case of sudden cardiac death at 1.5 years [47]. Late device thrombosis has been reported, although it appears to be rare [56].
Surgery — Pediatric patients with sinus venosus defects, coronary sinus defects, primum ASDs, and complex congenital lesions including partial anomalous pulmonary venous connection are generally not candidates for transcatheter closure and typically require surgical correction. In addition, infants with large secundum defects who present with heart failure generally cannot be adequately treated with transcatheter closure and typically undergo surgical correction. (See 'Indications for surgical closure' above.)
Procedure — Surgical repair requires cardiopulmonary bypass. The traditional surgical approach for ASD repair has been through a median sternotomy [57]. In some centers, surgeons are now employing minimally invasive approaches that substantially reduce incision size. However, these approaches are not necessarily appropriate for all ASDs. The applicability of a minimally invasive surgical approach will depend on specific patient ASD anatomy and institutional surgical expertise. (See "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Minimally invasive surgery'.)
Surgical closure is usually performed using a pericardial or Dacron patch. Small defects are often closed by direct suturing. However, suture closure of large defects can lead to distortion of the atrium. Repair of sinus venosus defects requires placement of a patch to direct the right pulmonary venous return into the left atrium and the systemic venous return into the right atrium.
Intraoperative TEE can assess the adequacy of repair and detect residual flow across the surgical site (movie 1). TEE can also detect tricuspid or mitral regurgitation, either of which may occur if tension from the repair on the valvular annulus distorts the geometry of leaflet coaptation.
Complications — Postoperative complications occur in 25 to 30 percent of patients and include [58-60]:
●Pericardial effusion
●Pleural effusions
●Arrhythmias
●Bleeding
●Pneumothorax
●Wound infection
Complications are usually transient and often do not require intervention. They may occur because of the standard surgical technique, which requires sternotomy or thoracotomy, atriotomy, and cardiopulmonary bypass, with its potentially deleterious consequences.
POST-PROCEDURE CARE AND FOLLOW-UP —
Following the closure procedure, patients are monitored for potential complications (eg, arrhythmia, thromboembolic events, pericardial effusion, residual shunting, device migration). Patients routinely undergo echocardiography and electrocardiography (ECG) to monitor for these complications.
Following transcatheter ASD closure
●Prior to discharge – An ECG, chest radiograph, and echocardiogram are usually performed immediately after the procedure before the child is discharged home.
●Antiplatelet therapy – For most patients who undergo transcatheter device closure of an ASD, we suggest antiplatelet therapy (with aspirin, clopidogrel, or another antiplatelet agent) for six months to reduce the chance of thrombus formation before the device becomes endothelialized. The choice of agent and duration of thromboprophylaxis varies by institution.
●Activity restrictions – Following the ASD device closure procedure, most cardiologists advise temporary limitations on vigorous physical activity for at least several weeks to reduce the risk of device migration. Specific recommendations regarding these temporary activity limitations vary among interventional cardiologists.
●Follow-up visits – Patients typically have initial follow-up at one month and then at 6 to 12 months after the procedure [11]. Follow-up visits should include an interval history, physical examination, ECG, and echocardiogram [12]. The frequency of subsequent follow-up depends on whether there is residual atrial level shunting, as discussed below. (See 'Cardiology follow-up' below.)
Following surgical ASD closure
●During the hospitalization – Patients who undergo surgical ASD closure are typically hospitalized for three to four days postoperatively to monitor for complications including arrhythmias, pericardial and pleural effusions, and residual shunting (see 'Complications' above). An echocardiogram, ECG, and chest radiograph are performed in the immediate postoperative period to assess for these complications. Fevers, chest pain, abdominal pain, and vomiting in the postoperative period after surgical ASD closure suggest the possible presence of clinically significant effusions and/or post-pericardiotomy syndrome. (See "Post-cardiac injury syndromes".)
●After discharge – Patients typically have initial follow-up at two weeks to one month after discharge. Patients are advised to avoid strenuous activity (sports, heavy lifting, swimming) for at least two weeks. The timing and frequency of subsequent follow-up depends on whether there is residual shunting or other complications, as discussed below. (See 'Cardiology follow-up' below.)
LONG-TERM MANAGEMENT
Cardiology follow-up — Long-term cardiology follow-up is advised for most patients who have undergone transcatheter or surgical ASD closure, particularly if there are other cardiac lesions or if there were preoperative or postoperative complications (eg, atrial arrhythmias, pulmonary artery hypertension).
●Patients who undergo transcatheter ASD closure – For patients who undergo successful transcatheter device closure of the ASD, the risk of long-term complications appears to be very low (see 'Long-term outcome' below). However, there are few data on outcomes beyond 10 to 15 years after transcatheter ASD closure, and thus some uncertainty remains as to whether these patients are at risk for long-term complications. Given the uncertainty, we generally follow these patients longitudinally. The frequency of follow-up depends on whether there is residual atrial level shunting. If no residual atrial level shunting was seen on echocardiogram at 6 to 12 months post-procedure, subsequent follow-up visits occur every two to five years [11]. Patients who have residual shunting are typically seen every one to two years. Follow-up visits usually include an interval history, physical examination, electrocardiogram (ECG), and echocardiogram [12].
●Patients who undergo surgical ASD closure – Most patients who have had ASDs surgically repaired during childhood and who have uncomplicated early postoperative courses remain free of late complications. There is an increased risk of developing late atrial arrhythmias compared with the general population, though the overall risk is low. (See 'Long-term outcome' below.)
The frequency of follow-up for patients with surgically repaired ASDs depends on whether there is residual shunting or other complications. For patients with an uncomplicated course, the long-term follow-up schedule is the same as for patients who underwent transcatheter closure (ie, every two to five years if there is no residual atrial level shunting on echocardiogram; every one to two years if the echocardiogram shows residual shunting) [11]. Follow-up visits usually include an interval history, physical examination, ECG, and echocardiogram [12].
Though patients with surgically repaired ASDs are at risk of developing late atrial arrhythmia, routine surveillance for arrythmia with ambulatory ECG monitoring is generally not necessary. This is because the incidence of arrhythmia is low overall, and the nature of these atrial arrhythmias is typically benign. In our practice, we monitor patients with periodic ECGs and we inform patients, families, and referring clinicians of the low but real potential for development of typically benign late atrial arrhythmias, including atrial fibrillation. We instruct patients to follow up if concerning symptoms develop (eg, palpitations, reduced exercise capacity).
Physical activity and sports participation — Temporary activity restrictions are advised for the first several weeks post-procedure, as discussed above. (See 'Post-procedure care and follow-up' above.)
After this recovery period, activity can be increased gradually as tolerated. Long-term restrictions from sports participation are not necessary for children who have undergone uncomplicated ASD closure.
Guidelines for competitive athletic participation in patients with ASDs from the American Heart Association and American College of Cardiology include the following [61]:
●Patients with repaired ASD can participate in any competitive sport three to six months after closure if they have normal pulmonary artery pressure, normal ventricular function, and no arrhythmias.
●Patients with untreated ASDs and normal pulmonary artery pressure can participate in any competitive sport.
●Patients with untreated ASDs and pulmonary hypertension may participate in low-intensity (class IA) sports (figure 3).
●Patients with untreated ASDs and associated pulmonary vascular obstructive disease with cyanosis and large right-to-left shunt should be restricted from participation in all competitive sports.
Additional details regarding physical activity and exercise in patients with congenital heart disease are provided separately. (See "Physical activity and exercise in patients with congenital heart disease".)
Endocarditis prophylaxis — To reduce the risk of infective endocarditis (IE), antibiotic prophylaxis is provided prior to relevant dental, oral, or invasive airway procedures during the first six months after the ASD closure procedure and beyond that if there is residual shunting adjacent to the site of a prosthetic device or material. Children with unrepaired isolated ASDs do not require IE prophylaxis. The approach to determining the need for prophylaxis is summarized in the figure (algorithm 2) and discussed in detail separately. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)
LONG-TERM OUTCOME
●Outcomes after transcatheter ASD closure – The available data suggest that transcatheter ASD closure achieves lasting successful ASD closure [17,62]. However, there are few data on outcomes beyond 10 to 15 years after transcatheter ASD closure since these devices were not available until the late 1990s to early 2000s. Thus, some uncertainty remains as to whether these patients are at risk for long-term complications.
Long-term outcomes following transcatheter ASD closure were described in a study of 1158 patients who underwent successful transcatheter ASD closure in childhood and were followed for a median of 3.5 years (range 6 months to 18 years) [17]. There were no deaths and most patients (96 percent) were symptom-free at last follow-up. Among the small subset of patients reporting symptoms, the most common symptoms were headaches and recurrent atypical chest pain. The main long-term cardiac complication observed in this cohort was cardiac arrhythmia, which occurred in eight patients (<1 percent). There were 69 patients who became pregnant during follow-up (at median of 10 years after the ASD closure procedure). All pregnancies were uneventful with no thromboembolic complications observed during pregnancy or after delivery.
In another study of 151 children and young adults (mean age 12 years) who were followed for a median of 6.5 years after successful transcatheter ASD closure, there were no deaths or significant complications, and all defects were completely closed at three years and remained closed thereafter [62].
●Outcomes after surgical ASD closure – Outcomes following surgical ASD closure in childhood are excellent, with almost no difference in long-term survival compared with age-matched healthy controls [59,63]. Long-term morbidities such as stroke, heart failure, arrhythmia, heart block, and pulmonary hypertension are rare [49,63,64]. In a study of 135 patients with ASD who underwent surgical closure in childhood and were followed for a mean of 26 years, no patient experienced cardiovascular death, heart failure, pulmonary hypertension, or stroke during follow-up [64]. Most patients (88 percent) described their health as good or very good. Symptomatic supraventricular tachyarrhythmias occurred in 8 percent of patients, a lower rate than is seen in adult patients with untreated ASDs or after surgical correction in adulthood. (See "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Effect of defect closure on risk of atrial arrhythmias'.)
INFORMATION FOR PATIENTS —
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword[s] of interest.)
●Basics topics (see "Patient education: Atrial septal defects in children (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Trivial versus clinically significant ASD – Many secundum atrial septal defects (ASDs) and patent foramen ovales (PFOs) identified on echocardiograms obtained for nonspecific indications in early infancy close spontaneously during later infancy or early childhood. If a child has an isolated defect characterized as "PFO" on the echocardiographic report or a trivial secundum ASD, follow-up is generally not necessary. Children with clinically significant ASDs (ie, secundum ASDs ≥3 mm or defects other than secundum such as sinus venosus or primum or coronary sinus ASDs) should be followed by a pediatric cardiologist every one to two years. (See 'Monitoring' above.)
●Management approach – The management of isolated ASDs in children is dependent upon the likelihood of spontaneous closure and, in patients with persistent ASDs, the size of the defect and the degree of shunting (algorithm 1). Our general approach is as follows (see 'Management approach' above):
•Indications for closure (see 'Indications for ASD closure' above)
-For children who have signs of substantial left-to-right shunting through an ASD, including right atrial or ventricular enlargement, signs or symptoms of pulmonary overcirculation (pulmonary edema, heart failure), and/or a high ratio of pulmonary-to-systemic blood flow (Qp/Qs), we recommend closure (Grade 1B). These findings are typically associated with moderate to large ASDs, and such defects are unlikely to close spontaneously.
-We also suggest ASD closure in young patients with ASDs who have experienced an embolic stroke with no other cause identified (Grade 2C). This is an uncommon scenario in pediatric patients. It occurs more commonly in adults. (See "Atrial septal abnormalities (PFO, ASD, and ASA) and risk of cerebral emboli in adults".)
-Patients who are asymptomatic without evidence of significant left-to-right shunting can be monitored longitudinally. (See 'Monitoring' above.)
•Timing – For children who have an indication for ASD closure, the intervention is generally deferred until after the age of two years in asymptomatic patients because there is a possibility, albeit small, of spontaneous closure during the first two years of life. (See 'Timing of ASD closure' above.)
•Transcatheter versus surgical closure – For children who have an indication for ASD closure, our approach to deciding between transcatheter and surgical closure is as follows (see 'Choice of closure intervention' above):
-For most children with isolated secundum ASDs, we suggest transcatheter device closure rather than surgical closure if there are appropriate anatomic characteristics (ie, not excessively large and with a sufficient rim of tissue to anchor the device) (Grade 2C). Transcatheter closure avoids cardiopulmonary bypass, sternotomy, and atriotomy and observational data suggest it has a similarly high success rate with lower complication rates and shorter hospital stay compared with surgery. This approach has largely replaced surgery in many centers for patients who meet the anatomic requirements. (See 'Choice of closure intervention' above and 'Transcatheter closure' above.)
-Sinus venosus defects, coronary sinus defects, primum ASDs, and complex congenital lesions generally require surgical correction. In addition, infants with large secundum defects who present with heart failure generally cannot be adequately treated with transcatheter closure, and they typically undergo surgical correction. Surgery is performed under cardiopulmonary bypass, usually through a median sternotomy, and a pericardial or Dacron patch is used to close the defect. (See 'Indications for surgical closure' above and 'Surgery' above.)
•Complications – Complications differ between the two interventions:
-Transcatheter closure – Periprocedural complications associated with transcatheter ASD closure include device embolization or malposition and arrhythmias. (See 'Complications' above.)
-Surgical closure – Postoperative complications occur in 25 to 30 percent of patients and include pericardial effusion, pleural effusions, arrhythmias, bleeding, pneumothorax, and infection. Long-term morbidities such as stroke, heart failure, arrhythmia, and pulmonary hypertension are rare. (See 'Complications' above.)
●Long-term follow-up
•Cardiology follow-up – Long-term cardiology follow-up is advised for most patients who have undergone transcatheter or surgical ASD closure. The frequency of follow-up depends on whether there is residual atrial level shunting or other complications. (See 'Cardiology follow-up' above.)
•Physical activity and sports participation – Temporary activity restrictions are advised for the first several weeks post-procedure. After this recovery period, activity can be increased gradually as tolerated. Long-term restrictions from sports participation are not necessary for children who have undergone uncomplicated ASD closure. (See 'Physical activity and sports participation' above.)
•Antibiotic prophylaxis – To reduce the risk of infective endocarditis (IE), antibiotic prophylaxis is provided prior to relevant dental, oral, or invasive airway procedures during the first six months after the ASD closure procedure and beyond that if there is residual shunting adjacent to the site of a prosthetic device or material (algorithm 2). Children with unrepaired isolated ASDs do not require IE prophylaxis. This issue is discussed in detail separately. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)
●Outcome – Long-term outcomes following transcatheter or surgical ASD closure in childhood are generally excellent, with almost no difference in long-term survival compared with age-matched healthy controls. Long-term morbidities such as stroke, heart failure, arrhythmia, and pulmonary hypertension are rare. Patients with surgically repaired ASDs are at risk of developing late atrial arrhythmias, though the absolute risk is low. (See 'Long-term outcome' above.)
