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Isolated atrial septal defects (ASDs) in children: Management and outcome

Isolated atrial septal defects (ASDs) in children: Management and outcome
Literature review current through: Jan 2024.
This topic last updated: Aug 01, 2022.

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 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

These absolute measurements are not exact, and the relative size of the defect (related to overall heart size) may be more clinically relevant. For example, a 6-mm ASD would be insignificant in an adult but would be of moderate size in a newborn.

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 repair 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, echocardiographic evidence of substantial right heart enlargement and qualitative color and spectral Doppler findings of a large amount of left-to-right shunting through the ASD can be sufficient to justify ASD closure. In addition, the following findings support ASD closure, though these are not sufficient in isolation:

Evidence of right ventricular hypertrophy on electrocardiogram

Auscultatory finding of a tricuspid flow rumble by an experienced clinician

Increased heart size and pulmonary vascularity on chest radiograph

Spontaneous closure — The majority of 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'.)

Persistent defect — For children who have persistent small defects without any symptoms, we do not suggest closure. High-quality evidence supporting a benefit of ASD closure in this setting is generally lacking. The risk of paradoxical embolism through a small ASD or valve-competent patent foramen ovale (PFO) is uncertain. The benefit of ASD closure in reducing the risk of embolism must be weighed against the short- and long-term risks of the procedure for closure. Procedure risks are changing as closure techniques continue to improve with decreasing morbidity. Closure is recommended by many clinicians in older adult patients with a small ASD or PFO who have experienced embolic strokes or transient ischemic attacks [11,12]. This issue is discussed in a separate topic review. (See "Stroke associated with patent foramen ovale (PFO): Evaluation".)

For patients with a persistent moderate or larger defect, left-to-right shunting usually increases with age if the defect is not corrected. In these patients, we recommend closure if there is significant shunting, as discussed below (see 'Criteria and timing for closure' below). The indications for closure of asymptomatic ASDs that are not associated with significant shunting are less clear. Evolving experience with transcatheter ASD closure may make a subset of such patients suitable candidates for closure. Since an increase in shunt volume can occur with moderate-sized ASDs [13], periodic follow-up of patients with such defects is clearly indicated. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Persistent moderate to large ASDs' and 'Monitoring' below.)

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.

Monitoring — The apparent incidence of ASD may be increasing because of increased usage of echocardiography in the neonatal period. Many secundum ASDs or incompetent foramen ovales identified on echocardiograms obtained for nonspecific indications in early infancy will close spontaneously during later infancy or early childhood. The monitoring of these patients, as well as those patients with defects other than secundum who are diagnosed prenatally, is dependent on the size and type of their 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. 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.

It is possible that future longitudinal population studies will reveal follow-up to be worthwhile in certain specific anatomical subtypes of PFO, such as those associated with a substantial aneurysm of the septum primum or a large Eustachian valve. However, definitive information in this regard is not yet available, because of selection bias of the extant reports.

Follow-up for trivial ASDs – Similar to PFO, children with trivial secundum ASDs (<3 mm) do not require specific follow-up.

Follow-up for clinically significant ASDs – The child in whom a clinically significant ASD is detected should be followed regularly by a pediatric cardiologist. Clinically significant ASDs include secundum ASDs with a maximal diameter of ≥3 mm since experience has shown that secundum ASDs of this size can increase substantially with patient growth. 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. (See 'Persistent defect' above.)

Criteria and timing for closure — We recommended ASD closure for children who have right heart enlargement, pulmonary overcirculation, and evidence of substantial left-to-right shunting through an ASD (ie, ratio of pulmonary-to-systemic flow [Qp/Qs] >2:1 or other evidence of large amount of left-to-right shunting, as described above). (See 'Measurement of shunt ratio' above.)

These findings 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'.)

As previously discussed, 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. (See 'Spontaneous closure' above.)

There is no definitive evidence that ASD closure is beneficial for patients with a persistent ASD without a significant left-to-right shunt and without symptoms directly attributable to the ASD (including paradoxical embolism or platypnea-orthodeoxia, which is shortness of breath and hypoxemia that occurs when sitting or standing and that is relieved when lying down). Most pathologic consequences of ASD, such as atrial arrhythmias and (rarely) pulmonary hypertension, are secondary to right heart volume overload or pulmonary overcirculation. No studies have shown that prospective closure of asymptomatic small ASDs prevents paradoxical embolization, and the risk of endocarditis with isolated small ASDs appears to be negligible. Since all closure techniques have risk, closure of asymptomatic small ASDs does not appear to be warranted at this time.

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 intervention

Our approach – The choice of intervention is dependent on the type of the ASD and if there are any other cardiac defects.

Transcatheter closure is the preferred closure intervention for patients with secundum ASDs that have favorable anatomy, including both of the following criteria (see 'Transcutaneous closure' below):

-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 that meet these criteria, we suggest transcatheter closure rather than surgical closure. Transcatheter closure should not be used in patients with sinus venosus or primum or coronary sinus ASD or in infants with large defects and heart failure.

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 family/caregiver prefers 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 [14]. There may be a slightly increased risk of needing reintervention with transcatheter closure.

Outcomes with the two approaches were described in a multicenter nonrandomized 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 [15]. 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) [14]. 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).

Young infants – Although successful transcatheter closure can be performed in young infants, the complication rate appears to be higher in infants and young children compared with older patients in the three- to five-year age range who weigh at least 15 kg. Furthermore, spontaneous closure of even large ASDs can occur in infants and young children. As a result, the benefits of closure in infants and young children (weight <15 kg) must be weighed against the increased risk of complication and possibility of spontaneous closure.

Post-procedure care — Clinical evaluation subsequent to closure should assess for signs of embolic events, arrhythmia, and chest pain. Echocardiographic surveillance is essential to detect complications including residual shunting, pericardial effusion, ventricular function, and pulmonary artery pressure, and, in patients who underwent transcatheter device closure, device migration and thrombosis. Electrocardiographic monitoring is also performed to detect any evidence of atrial arrhythmia.

Following transcatheter ASD closure – Patients are typically treated with aspirin, clopidogrel, or another anticoagulant agent subsequent to transcatheter ASD defect closure for six months to reduce the chance of thrombus formation on the ASD device before the device has become endothelialized.

In addition, temporary limitations on highly vigorous physical activity during the weeks immediately subsequent to ASD device implantation are usually advised. Specific recommendations regarding these temporary exercise limitations vary among interventional cardiologists.

Following surgical ASD closure – In the immediate postoperative period, patients are monitored for complications including pericardial and pleural effusions and post-pericardiotomy syndrome. Fevers, chest pain, abdominal pain, and vomiting in postoperative ASD patients suggest the possible presence of clinically significant effusions and/or post-pericardiotomy syndrome.

CLOSURE PROCEDURES

Transcutaneous 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 small to moderate secundum ASDs that have appropriate anatomic characteristics [16]. The applicability of transcatheter closure in an individual patient is highly dependent on specific secundum ASD anatomy and institutional expertise. Larger secundum defects are generally closed surgically, although defects over 30 mm have been successfully closed by transcatheter technique. Transcatheter closure also 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) [17]. However, criteria vary among centers, based on the individual institution's level of expertise. Transcatheter closure should not be used in patients with sinus venosus or primum or coronary sinus ASD or in infants with large defects and heart failure.

In the United States, devices that have been approved by the US Food and Drug Administration for transcatheter closure of secundum ASDs include [18,19]:

The Amplatzer Septal Occluder (figure 2).

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.

Echocardiographic monitoring — The use of transesophageal or intracardiac echocardiography (ICE) may facilitate the deployment of transcatheter ASD closure devices. Measurement of the size and location of the ASD by transesophageal echocardiography (TEE) can help the clinician select the appropriate device. In addition, TEE can be used to guide the procedure in real time, an approach that may minimize fluoroscopy time [20]. Two- or even three-dimensional TEE is particularly helpful when multiple devices are used [17].

The development of ICE has provided a modality for evaluating cardiac anatomy that may be particularly well suited for use in the management of ASDs [21-25]. ICE can provide guidance for device placement and facilitates proper device selection by providing an accurate assessment of the size and shape (typically, elliptical) of the defect [23].

ICE has the following advantages compared with TEE: It does not require endotracheal intubation or general anesthesia [22,25], it permits continuous monitoring during the procedure, and it may shorten the duration of the procedure [25].

Complications — Periprocedural complications associated with transcatheter ASD closure include device embolization or malposition and arrhythmias (usually atrial) [15,26-31]. In a prospective multicenter postapproval study in 1000 patients (mean age 21±22 years) who underwent secundum ASD 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 and 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 [30].

In a systematic review and meta-analysis of 13 observational studies, the overall rate of complications following transcatheter ASD closure was 6.6 percent and the rate of major complications was 1.9 percent [14]. 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.

Cardiac perforation, which can present as hemopericardium, pericardial effusion, cardiovascular collapse, or sudden death, is an uncommon complication of the Amplatzer Septal Occluder [32]. This complication has been reported as early as the first day and as late as 15 years after the procedure [33-35]. Erosion leading to fatal tamponade has been very rarely reported with other devices as well [36,37].

Late complications are unusual. In a report of 417 patients, there was one case of peripheral embolization at one year and one case of sudden cardiac death at 1.5 years [27]. The available evidence and experience with transcatheter ASD closure suggest that major late problems are quite rare; however, a definitive assessment of long-term risk cannot be made, because device technology and implantation technique continue to change and because follow-up times are relatively limited [38].

Outcome — Short-term outcomes (up to two years) of transcatheter closure using the different available devices have been excellent, with reported procedural success rates of 88 to 98 percent [15,26,29,30,39-41]. There are fewer data on long-term outcomes, but the available data suggest that these devices achieve lasting successful ASD closure.

Amplatzer device – In a prospective multicenter postapproval study in 1000 patients (mean age 21±22 years) who underwent secundum ASD with the Amplatzer device 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 [30].

Long-term outcomes with the Amplatzer device were described in a review of 151 children and young adults (mean age 11.9 years) who were followed for a median of 78 months after successful closure [42]. There were no deaths or significant complications, and all defects were completely closed at three years and remained closed thereafter.

Although clinical indications for ASD closure are less commonly encountered in infancy, the Amplatzer device has also been successfully used in patients who weigh <8 kg, including patients with multiple lesions [43,44]. Adverse events are generally minor and include transient arrhythmias and need for blood transfusions. Major reported complications are rare and include thrombosis of the inferior vena cava due to vascular intimal injury and inadequate systemic flow as well as pulmonary congestion resulting in device failure in a patient with Shone complex.

Gore Cardiform Septal and ASD Occluders – In a multicenter retrospective analysis of secundum ASD closure with the Gore Septal Occluder in 173 consecutive pediatric patients (mean age six years), no serious adverse events occurred over a median follow-up of 20 months [45]. Five percent of patients had small residual shunts that did not require reintervention. A previous multicenter study reported similar results [46,47].

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 [48,49].

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 [39]. Device wire frame fracture occurred in 8 percent of deployments, but only one patient required treatment [39].

Surgery — Patients with sinus venosus defects, coronary sinus defects, primum ASDs, and complex congenital lesions including partial anomalous pulmonary venous connection are not candidates for transcatheter closure and 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.

Surgical repair requires cardiopulmonary bypass. The traditional surgical approach for ASD repair has been through a median sternotomy [50]. 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 [51-53]:

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.

Outcome — Outcomes following surgical closure of ASD in childhood are excellent, with almost no difference in long-term mortality compared with age-matched controls [52,54]. Long-term morbidities such as stroke, heart failure, arrhythmia, heart block, and pulmonary hypertension are also rare [31,54,55]. In one study, symptomatic supraventricular tachyarrhythmias occurred in 6 percent of patients at 15 years after surgery and in an additional 2 percent in the next decade, a lower rate than in patients with untreated ASDs or after surgical correction in adulthood [55].

LONG-TERM MANAGEMENT — Long-term cardiology follow-up is indicated following surgical or transcatheter ASD closure if there are other cardiac lesions, if there were preoperative or postoperative atrial arrhythmias, if the patient had pulmonary artery hypertension preoperatively, or if the ASD was repaired during adulthood.

Patients who have had ASDs surgically repaired during childhood and who have uncomplicated early postoperative courses generally are free of late complications and activity restrictions. Patients with surgically repaired ASDs are at increased risk of developing late atrial arrhythmias compared with the general population (see 'Outcome' above). However, routine surveillance is generally not necessary, since the incidence is relatively low overall and the nature of these atrial arrhythmias is typically benign. In our practice, we inform patients, families, and referring clinicians of the low but real potential for development of typically benign late atrial arrhythmias, including atrial fibrillation, and instruct patients to follow up if symptoms develop.

Long-term outcomes following transcatheter ASD closure are less certain, and we generally follow these children longitudinally.

Sports participation — The 2015 scientific statement of the American Heart Association and American College of Cardiology provides competitive athletic participation guidelines for patients with congenital heart disease, including ASD [56]:

Patients with untreated ASDs and normal pulmonary pressure can participate in all competitive sports

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

Patients with repaired ASD can participate in all competitive sports three to six months after closure if they have normal pulmonary pressure, normal ventricular function, and no arrhythmias

Physical activity and exercise in patients with congenital heart disease are discussed in detail separately. (See "Physical activity and exercise in patients with congenital heart disease".)

Endocarditis prophylaxis — The 2007 guidelines of the American Heart Association recommend no antibiotic prophylaxis in children with an isolated ASD, except in the following circumstances:

In children with a repaired ASD that required the use of prosthetic material or device, prophylactic antibiotics are recommended for dental and respiratory tract procedures during the first six months after the repair

In children with repaired ASD with a residual defect at the site or adjacent to the site of a prosthetic device, antibiotic prophylaxis is recommended for dental and respiratory tract procedures

Endocarditis prophylaxis is discussed in greater detail separately. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

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. Our general approach is as follows (see 'Management approach' above):

Indications for closure – For children with an ASD who have right heart enlargement, pulmonary overcirculation, and evidence of substantial left-to-right shunting, we recommend closure (Grade 1B). These findings are typically associated with moderate to large ASDs, and such defects are unlikely to close spontaneously. ASD closure 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. Patients who are asymptomatic without evidence of significant left-to-right shunting can be monitored longitudinally. (See 'Criteria and timing for closure' above.)

Preprocedure evaluation – Prior to closure, patients should have a comprehensive echocardiographic evaluation to accurately measure the size of the defect, assess the degree of left-to-right shunting, identify other cardiac defects, and assess for pulmonary hypertension. These factors help inform the choice of intervention. (See 'Preprocedure evaluation' 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 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 is associated with excellent short- and midterm outcomes. This approach has largely replaced surgery in many centers for patients who meet the anatomic requirements. Observational data suggest that transcatheter closure has a similar likelihood of success, with lower complication rates and shorter duration of hospital stay compared with surgery. (See 'Transcutaneous closure' above.)

-Patients with sinus venosus defects, coronary sinus defects, primum ASDs, and complex congenital lesions 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. The long-term outcome is excellent, with almost no difference in long-term mortality compared with age-matched controls. (See 'Surgery' above.)

Complications – Complications differ between the two interventions:

Transcatheter closure – Periprocedural complications associated with percutaneous 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 wound infection. Long-term morbidities such as stroke, heart failure, arrhythmia, and pulmonary hypertension are rare. (See 'Surgery' above.)

Long-term follow-up – Long-term cardiology follow-up is indicated following surgical or transcatheter ASD closure if there are other cardiac lesions, if there were preoperative or postoperative atrial arrhythmias, if the patient had pulmonary artery hypertension preoperatively, or if the ASD was repaired during adulthood. Patients who have had ASDs surgically repaired during childhood and who have uncomplicated early postoperative courses generally remain free of late complications. Long-term outcomes following transcatheter ASD closure are less certain, and we generally follow these children longitudinally. Most children who have undergone uncomplicated ASD closure can participate in sports without restrictions. Antibiotic prophylaxis is not required beyond the first six months after ASD closure unless there is a residual defect adjacent to the device or patch. (See 'Long-term management' above and "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

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Topic 5779 Version 28.0

References

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