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Management of atrial septal defects in adults

Management of atrial septal defects in adults
Literature review current through: Jan 2024.
This topic last updated: Feb 03, 2023.

INTRODUCTION — Atrial septal defect (ASD) is the most common congenital lesion in adults after bicuspid aortic valve. Although the defect often causes no symptoms until adulthood, potential complications of an undetected ASD include atrial arrhythmias, paradoxical embolization, cerebral abscess, right ventricular (RV) volume overload with late RV failure, and pulmonary hypertension that can become irreversible and lead to right-to-left shunting (Eisenmenger syndrome).

This topic will review indications for closure and medical management of ASDs in adults. Surgical and percutaneous closure of ASDs; pathophysiology, anatomy, natural history, and clinical features of ASDs in adults; the identification and assessment of ASDs; and issues related to ASDs in children are discussed separately. (See "Surgical and percutaneous closure of atrial septal defects in adults" and "Clinical manifestations and diagnosis of atrial septal defects in adults" and "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis" and "Isolated atrial septal defects (ASDs) in children: Management and outcome".)

GENERAL MANAGEMENT — The following issues may arise in patients with unrepaired or repaired ASDs.

Atrial arrhythmias — Patients with ASDs are at increased risk of atrial fibrillation (AF) and atrial flutter, particularly if defect closure occurs after age 25. AF should be managed according to standard recommendations. An attempt at cardioversion is generally recommended. Arrhythmia interventions can be considered in conjunction with defect closure when appropriate. (See "Atrial fibrillation: Overview and management of new-onset atrial fibrillation" and "Atrial fibrillation in adults: Use of oral anticoagulants".)

Pulmonary hypertension — Management of patients with congenital heart disease (CHD) (including ASD) with secondary pulmonary hypertension (PH) and Eisenmenger syndrome is discussed separately. There are a number of conditions or procedures that are associated with increased risk in patients with PH-CHD, including volume depletion, exercise, endocardial pacing, pregnancy, and traveling to or living at high altitude; patients with Eisenmenger syndrome are at particularly high risk for complications related to these conditions and also face complications related to cyanosis and pulmonary artery thrombosis. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

Selected patients with PH secondary to CHD are candidates for advanced therapy for pulmonary arterial hypertension, which may improve symptoms, and in some cases, may enable the patient to meet criteria for ASD closure, with advanced therapy continued after ASD closure. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis" and 'Pulmonary hypertension' below.)

Endocarditis prophylaxis — Patients with ASDs should receive education regarding the importance of good oral hygiene and prompt medical attention for infections to reduce the risk of infective endocarditis (IE).

Antimicrobial prophylaxis for the prevention of IE is recommended during the six months after ASD closure (percutaneous or surgical) when a relevant procedure likely to result in bacteremia with a microorganism that has the potential ability to cause endocarditis is performed [1]. Antimicrobial prophylaxis is also recommended in patients with an ASD who have a prior history of IE or a residual shunt adjacent to a prosthetic patch or prosthetic device. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

We agree with the 2007 American Heart Association (AHA) guidelines on endocarditis prophylaxis, which state that an isolated or repaired ASD with no residual shunt is associated with negligible risk of endocarditis and that antibiotic prophylaxis is not warranted in these settings [1]. The very low risk of endocarditis was illustrated in a review of 882 adults with a secundum ASD; only three had a history of endocarditis [2]. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

Pregnancy

Unrepaired ASD — Young women with a small, uncomplicated ASD generally tolerate pregnancy well. A prepregnancy consultation with an adult CHD specialist is suggested.

Longevity in patients with an unoperated ostium secundum ASD spans the reproductive years. ASD is the most common repaired or unrepaired lesion occurring in pregnant women with CHD other than bicuspid aortic valve [3,4]. Many women with an ASD are unaware of their heart defect prior to pregnancy. (See "Clinical manifestations and diagnosis of atrial septal defects in adults".)

Despite the gestational increase in cardiac output and stroke volume, young women with an uncomplicated ASD generally tolerate pregnancy (even multiple pregnancies) with no apparent ill effects [5]. However, when women with secundum ASDs also have a history of supraventricular arrhythmias, especially AF or atrial flutter, or RV failure, the pregnancy risk increases [4,6].

An important concern is the risk of paradoxical embolization from leg or pelvic vein deep venous thrombosis because emboli carried by the inferior vena cava can be directed across the ASD into the systemic circulation [7-9]. Meticulous leg care and ambulation minimizes venous stasis during gestation and in the puerperium and reduces the risk of paradoxical embolization. Use of filters on all intravenous lines should be employed to decrease the risk of paradoxical air emboli. Anticoagulation should be considered in select patients at very high risk for venous thrombosis and paradoxical embolism. (See "Atrial septal abnormalities (PFO, ASD, and ASA) and risk of cerebral emboli in adults", section on 'Paradoxical emboli' and "Prevention of venous thromboembolic disease in adult nonorthopedic surgical patients".)

Also important, but less well known, are the potentially deleterious effects of acute blood loss. Hemorrhage during delivery results in a rise in systemic vascular resistance and a fall in systemic venous return, a combination that augments the left-to-right shunt [10].

When ASDs are associated with significant pulmonary vascular disease, pregnancy should be avoided due to the high risk of maternal and fetal mortality. (See "Pulmonary hypertension with congenital heart disease: Pregnancy and contraception".)

Repaired ASD — Women who have successfully undergone closure of a secundum ASD and have no significant residua can anticipate very low maternal cardiac risk during pregnancy. Successful closure of the defect also eliminates the hazard of paradoxical embolization.

Residual postoperative sequelae are uncommon except for atrial tachyarrhythmias with frequency related to age at intervention and prior history of arrhythmia [11]. Secundum ASDs are frequently closed percutaneously, and adverse pregnancy-related concerns have not been described.

Participation in sports — An ASD can have a varying degree of impact on the right heart and on cardiac performance during athletic activity. We agree with the recommendations for competitive athletics in the 2015 scientific statement from the AHA and American College of Cardiology, which vary with the severity of the defect [12]:

Athletes with small defects, normal right-sided heart volumes, and no PH should be allowed to participate in all sports.

Athletes with a large ASD and no PH should be allowed to participate in all sports.

Athletes with an ASD and PH may be considered for participation in low-intensity sports (class IA) (figure 1).

Athletes with associated pulmonary vascular obstructive disease who have cyanosis and a large right-to-left shunt should not participate in competitive sports, with the possible exception of class IA sports (figure 1).

The recommendations for athletics in patients with a closed ASD are as follows [12]:

Three to six months after the operation or intervention, athletes without PH, myocardial dysfunction, or arrhythmias can participate in all sports.

After operation or intervention, patients with PH, myocardial dysfunction, or arrhythmias may be considered for participation in low-intensity sports (class IA) (figure 1).

ASD CLOSURE

Indications — ASD closure is indicated in the following settings (algorithm 1) [13-15]:

Without significant pulmonary hypertension (PH)

With functional impairment For patients with ASD (any type) causing impaired functional capacity with a hemodynamically significant net left-to-right shunt and without significant PH, we recommend ASD closure. We define a hemodynamically significant left-to-right shunt as one causing right atrial and/or RV enlargement with a pulmonary-to-systemic blood flow (shunt) ratio (Qp/Qs) ≥1.5:1. We define lack of significant PH as pulmonary artery systolic pressure <50 percent of systemic systolic arterial pressure, pulmonary vascular resistance (PVR) less than one-third of systemic vascular resistance, and with no cyanosis at rest or during exercise. (See 'Significant shunt' below.)

Without symptoms – For asymptomatic patients with an ASD (any type) with a net left-to-right shunt, right atrial and/or RV enlargement with a Qp/Qs ≥1.5:1, pulmonary artery systolic pressure <50 percent of systemic blood pressure, PVR less than one-third of systemic vascular resistance and no cyanosis at rest or during exercise, we suggest ASD closure (Grade 2C). (See 'Evidence' below.)

With significant PH

With moderate PH – For patients with ASD (any type) with impaired functional capacity with a net left-to-right shunt, right atrial and/or RV enlargement with a Qp/Qs ≥1.5:1, and pulmonary artery systolic pressure ≥50 percent and less than or equal to two-thirds of systemic systolic arterial pressure or PVR greater than or equal to one-third of and less than or equal to two-thirds of systemic vascular resistance, decisions regarding ASD closure are made on a case-by-case basis dependent on individualized assessment of benefits and risks by adult congenital heart disease (CHD) and PH experts. (See 'Pulmonary hypertension' below.)

The decision to close an ASD in the setting of elevated pulmonary artery systolic pressures is complex given uncertain benefit and substantial risk. This decision should be made in conjunction with adult CHD and PH experts. Cardiac catheterization and hemodynamic assessment should be obtained in patients with ASD and PH, left heart disease, and for older patients with signs of, or risk factors for, coronary artery disease.

For patients with PVR >5 Wood units, pharmacologic targeted pulmonary arterial hypertension (PAH) therapy with reevaluation of hemodynamics at follow-up should be considered. (See 'Pulmonary hypertension' below.)

For patients with ASD, PH, and left heart disease, test balloon occlusion of the ASD and reassessment of hemodynamics may aid in the assessment of whether ASD closure is likely to be beneficial [16]. ASD test occlusion involves temporarily closing the ASD with a balloon in the cardiac catheterization laboratory by an operator experienced in performing the procedure and interpreting the hemodynamic data. Hemodynamics are assessed before and after ASD balloon occlusion and may be helpful in determining suitability for ASD closure. Features that suggest that ASD closure will not be tolerated include decrease in cardiac output and/or increase in left ventricular end-diastolic pressure or pulmonary artery wedge pressure.

The limited data on the effects of ASD closure in this setting are discussed separately. (See "Surgical and percutaneous closure of atrial septal defects in adults", section on 'With pulmonary hypertension'.)

With severe PH – For patients with ASD (any type) with net right-to-left shunt and irreversible pulmonary artery systolic pressure greater than two-thirds of systemic systolic arterial pressure or irreversible PVR greater than two-thirds of systemic vascular resistance, we recommend against ASD closure (Grade 1B). (See 'Pulmonary hypertension' below and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

As mentioned below and discussed further separately, some patients with CHD and PH are candidates for advanced therapy for pulmonary arterial hypertension. In some patients with ASD and moderate to severe PH, it may be possible to reduce pulmonary artery pressure and PVR to meet the threshold for ASD closure. (See 'Pulmonary hypertension' above and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

In contrast, patients with severe irreversible PH (PVR greater than two-thirds of systemic vascular resistance or pulmonary artery pressure greater than two-thirds of systemic arterial pressure) should not undergo ASD closure [13-15]. Irreversible severe PH identifies a group of patients with reduced expected survival with prohibitively high morbidity and mortality with attempted defect repair, as discussed separately [13,17,18] (see "Surgical and percutaneous closure of atrial septal defects in adults", section on 'With pulmonary hypertension'). It has been suggested that, in this setting, maintenance of interatrial communication provides a mechanism to maintain cardiac output at the expense of desaturation, and this may be advantageous. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

Other presentations

For platypnea-orthodeoxia – For patients with documented platypnea-orthodeoxia caused by an ASD (any type) in the absence of severe PH, we suggest ASD closure (Grade 2C). (See 'Platypnea-orthodeoxia' below.)

For paradoxical embolism – For patients with ASD (any type) with a net left-to-right shunt who have had an embolic-appearing cryptogenic ischemic stroke, we suggest referral to adult CHD and neurology experts to assess the potential benefits and risks of ASD closure. Patients most likely to benefit from ASD closure are those who are young (eg, <60 years old) without evident source of stroke despite a comprehensive evaluation by a stroke neurologist and a cardiologist. (See 'Paradoxical embolization' below.)

Qp/Qs measured in selected cases — Cardiac catheterization and calculation of the Qp/Qs ratio is not required to determine the need for ASD closure when right atrial and/or RV enlargement is present (in the absence of another cause for such enlargement and important PH) Also, most patients with ASD do not require cardiac catheterization to calculate PVR prior to intervention. However, cardiac catheterization is helpful in cases in which the cause of right atrial or RV enlargement is uncertain or PH is possible. In the absence of PH, Qp/Qs is closely correlated with the size of the ASD [19].

There are no systematic data that identify a threshold value for Qp/Qs for repair of an ASD. Authors have advocated Qp/Qs thresholds ranging from Qp/Qs >2:1 [20] to 1.7:1 [21] and 1.5:1 [13,22]. Methods for calculation of Qp/Qs are discussed separately (figure 2). (See "Pulmonary artery catheterization: Interpretation of hemodynamic values and waveforms in adults", section on 'Detection of left-to-right shunts'.)

Evidence

Significant shunt — Indications for ASD closure are based upon limited data suggesting improved functional status and decreased risk of pneumonia in patients undergoing ASD closure compared with those without ASD closure; there is also limited evidence of a mortality benefit [13-15]. In asymptomatic patients, the long-term benefit of ASD closure is less certain [13,23].

A systematic review and meta-analysis included 11 nonrandomized studies comparing secundum ASD closure (surgical or percutaneous) with medical therapy in a total of 603 adults [24]. This paper also reviewed two additional studies that assessed mortality rates (a single-center randomized controlled trial and a retrospective multicenter cohort study) in a total of 652 patients with ASD (but not limited to secundum-type) [21,25].

To assess the effect on mortality rate of ASD closure, the results of the randomized trial and the controlled trial were pooled despite their differing designs [24]. While the effect of ASD closure was not significant for unadjusted mortality, there was a significant decrease in adjusted mortality (odds ratio [OR] 0.28, 95% CI 0.13-0.58). The randomized trial compared surgical closure and medical therapy in 473 adults with isolated ASD (ostium secundum [88.2 percent] or sinus venosus [11.8 percent] defect), age 40 and over at diagnosis (mean age 51), with Qp/Qs ≥1.7, and pulmonary artery systolic pressure <70 mmHg [21]. After a median follow-up of 7.3 years, the following results were observed:

The composite primary end point (death, pulmonary embolism, major arrhythmias, stroke, recurrent pulmonary infection, functional class deterioration, or heart failure [HF]) occurred significantly more frequently with medical than surgical therapy (21 versus 11 percent, hazard ratio [HR] 1.85, 95% CI 1.08-3.17). This difference was almost entirely due to a higher incidence of recurrent pneumonia among the medically-treated patients.

The overall unadjusted mortality rate was not statistically different with medical versus surgical therapy (5.8 versus 4.3 percent).

On multivariate analysis (adjusted for age at entry, mean pulmonary artery systolic pressure >35 mmHg, previous atrial tachyarrhythmia, and cardiac index <3.5 L/m2), there was a significantly higher mortality with medical management (HR 4.09, 95% CI 1.41-11.89).

Pooled analysis of three observational studies (with a total of 107 patients) on the effect of secundum ASD closure on functional capacity found that the odds of being in New York Heart Association (NYHA) functional class I were significantly higher with ASD closure than with medical therapy (OR 13.78, 95% CI 3.33-57.08) [24]. However, an observational study (not included in the meta-analysis) involving largely asymptomatic adults with ASD with (n = 48) or without (n = 34) surgical ASD closure with a mean follow-up of 25 years revealed no difference in symptoms or mortality with ASD closure [23].

Pooled analysis of eight observational studies found that secundum ASD closure was associated with reduction in RV size. In an analysis of data on patients >50 years of age in two studies, there was no significant difference in the odds of atrial fibrillation (AF) after ASD closure (OR, 1.78, 95% CI 0.90-3.52).

Younger patients have better long-term outcomes following surgical ASD closure than older patients, but both younger patients as well as older patients (>50 years of age) with appropriate indications may benefit from ASD closure. (See "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Effect of age'.)

Data comparing surgical and percutaneous transcatheter closure of secundum ASD are discussed separately. (See "Clinical manifestations and diagnosis of atrial septal defects in adults", section on 'Natural history' and "Surgical and percutaneous closure of atrial septal defects in adults".)

Pulmonary hypertension — The efficacy of ASD closure is uncertain, but it may be an option in select patients with net left-to-right shunt (Qp/Qs >1.5) who have pulmonary artery systolic pressure ≥50 percent but less than or equal to two-thirds of systemic systolic arterial pressure or PVR greater than or equal to one-third but less than or equal to two-thirds of systemic vascular resistance; these parameters may be assessed at baseline or when challenged with vasodilators, preferably inhaled nitric oxide, or after advanced therapy for pulmonary arterial hypertension or with test occlusion of the defect [13-15].

Platypnea-orthodeoxia — The platypnea-orthodeoxia syndrome is a rare disorder characterized by both dyspnea (platypnea) and arterial desaturation (orthodeoxia) in the upright position with improvement in the supine position. This syndrome has been described with ASD and patent foramen ovale (PFO), as well as with intrapulmonary shunting or ventilation perfusion mismatch [26]. In a patient with ASD and platypnea-orthodeoxia, the right atrial pressure may or may not be elevated; in patients with normal right atrial pressure, blood flow may be directed from the right atrium across the ASD as a consequence of altered anatomic relationships caused by an intracardiac condition (eg, prominent Eustachian valve) or an extracardiac condition (eg, severe kyphosis) [26]. (See "Patent foramen ovale", section on 'Platypnea-orthodeoxia syndrome'.)

An observational multicenter study of patients with platypnea-orthodeoxia and PFO suggested that transcatheter device closure is safe and effective, resulting in prompt increase in saturation and also symptomatic improvement [27]. This study provides indirect support for ASD closure in patients with platypnea-orthodeoxia and ASD.

Paradoxical embolization — For patients with ASD who have evidence of paradoxical embolism such as an embolic-appearing cryptogenic ischemic stroke (ie, no evidence of source of stroke other than ASD despite a comprehensive evaluation), we suggest referral to adult CHD and neurology experts to assess the benefits and risks of ASD closure. The evaluation of the likely cause of stroke should include consideration of cardiovascular risk factors (eg, AF, age, and presence of diabetes mellitus) associated with increased risk of alternate causes of stroke. (See "Cryptogenic stroke and embolic stroke of undetermined source (ESUS)", section on 'Evaluation and diagnosis'.)

The incidence of ASD in adults who present with paradoxical embolism is higher than expected, thus ASD should be considered as a possible cause in patients with cryptogenic stroke [28]. The risk for paradoxical embolism with ASD appears to be highest in patients with other risk factors, such as tricuspid regurgitation related to Ebstein anomaly [29]. There are limited data on the efficacy of ASD closure in patients with ASD and presumed paradoxical embolism. The evidence for ASD closure is largely indirect from randomized trials in patients with PFO and cryptogenic stroke showing decreased risk of recurrent stroke after PFO closure compared with medical therapy alone. (See "Stroke associated with patent foramen ovale (PFO): Evaluation".)

Patients with an ASD are at risk for paradoxical embolization when right-to-left shunting occurs (leading to stroke, transient ischemic attack, or peripheral emboli). In the majority of patients with net left-to-right shunts, intermittent right-to-left shunting can occur during transient increases in right-sided pressure (eg, with a Valsalva maneuver or coughing). For patients with primarily right-to-left shunting in the presence of severe PH, ASD closure is contraindicated. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

Issues related to the causes, diagnosis, and management of paradoxical embolization and PFO and atrial septal aneurysm are discussed in detail separately. (See "Atrial septal abnormalities (PFO, ASD, and ASA) and risk of cerebral emboli in adults" and "Stroke associated with patent foramen ovale (PFO): Evaluation".)

Choice of closure procedure — When ASD closure is indicated, most secundum ASDs can be closed percutaneously or surgically. Surgical closure is recommended for patients with secundum ASD requiring closure when percutaneous repair is not feasible or appropriate [13]. Sinus venosus, coronary sinus, and ostium primum defects are closed surgically by congenital heart surgeons, as they are not generally amenable to percutaneous device closure (although there are case reports of percutaneous closure of these defects). In addition, most of these ASDs have associated cardiac lesions that benefit from intervention, such as anomalous pulmonary veins and atrioventricular valve abnormalities. Surgical and device ASD closure procedures are discussed further separately. (See "Surgical and percutaneous closure of atrial septal defects in adults".)

ISSUES WITH UNREPAIRED ASD — Issues to address in patients with unrepaired ASDs include monitoring, participation in self-contained underwater breathing apparatus (SCUBA) diving, and high-altitude exposure.

Monitoring — Patients with small ASDs (less than 10 mm) with no evidence of RV enlargement or pulmonary hypertension (PH) should receive periodic routine follow-up to monitor for development of symptoms (eg, arrhythmias or paradoxical embolism), RV enlargement, or PH. We suggest clinical follow-up and echocardiography, including assessment of RV size and function and pulmonary artery pressure every three to five years as recommended by the 2008 American College of Cardiology/American Heart Association adult CHD guidelines [13]. More frequent follow-up is recommended for patients with symptoms or hemodynamic sequelae such as RV enlargement or PH or other complications. For example, patients with ASD and RV enlargement should generally receive follow-up every two years, and patients with PH should receive follow-up at least every year.

SCUBA diving — Individuals with ASDs are at increased risk for complications when they experience fluctuations in ambient air pressure, as with SCUBA diving. ASD has been associated with increased risks of decompression illness and paradoxical emboli among individuals who SCUBA dive. SCUBA diving is generally contraindicated in patients with an unrepaired ASD (or other intracardiac shunt). (See "Complications of SCUBA diving".)

High-altitude exposure — Patients with ASDs are at risk for increased right-to-left shunting and oxygen desaturation at high altitudes. Patients with Eisenmenger syndrome are particularly advised to avoid chronic high-altitude exposure. Patients with ASDs are advised to consult with a cardiologist specializing in CHD prior to high-altitude exposure. (See "Approach to patients with heart disease who wish to travel by air or to high altitude", section on 'Congenital heart disease'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Congenital heart disease in adults".)

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 e-mail 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 adults (The Basics)")

SUMMARY AND RECOMMENDATIONS

Atrial septal defect (ASD) closure is indicated in the following settings (algorithm 1):

Without significant pulmonary hypertension (PH)

-With functional impairment – For patients with ASD (any type) causing impaired functional capacity with a hemodynamically significant net left-to-right shunt and without significant PH, we recommend ASD closure (Grade 1B). We define a hemodynamically significant left-to-right shunt as one causing right atrial and/or RV enlargement with a pulmonary-to-systemic blood flow(shunt) ratio (Qp/Qs) ≥1.5:1. We define lack of significant PH as pulmonary artery systolic pressure <50 percent of systemic systolic arterial pressure, pulmonary vascular resistance (PVR) less than one-third of systemic vascular resistance, and with no cyanosis at rest or during exercise. (See 'Significant shunt' above.)

-Without symptoms – For asymptomatic patients with an ASD (any type) with a net left-to-right shunt, right atrial and/or RV enlargement with a Qp/Qs ≥1.5:1, pulmonary artery systolic pressure <50 percent of systemic blood pressure, PVR less than one-third of systemic vascular resistance and with no cyanosis at rest or during exercise, we suggest ASD closure (Grade 2C). (See 'Evidence' above.)

With significant PH

-With moderate PH – For patients with ASD with impaired functional capacity, a net left-to-right shunt with right atrial and/or RV enlargement with a Qp/Qs ≥1.5:1,and pulmonary artery systolic pressure ≥50 percent and less than or equal to two-thirds of systemic systolic arterial pressure or PVR greater than or equal to and less than or equal to two-thirds of systemic vascular resistance, decisions regarding ASD closure are made on a case-by-case basis dependent on individualized assessment of benefits and risks by adult congenital heart disease (CHD) and PH experts. (See 'Pulmonary hypertension' above.)

-With severe PH – For patients with ASD with net right-to-left shunt and irreversible pulmonary artery systolic pressure greater than or equal to two-thirds of systemic systolic arterial pressure or irreversible PVR greater than two-thirds of systemic vascular resistance, we recommend against ASD closure (Grade 1B). (See 'Pulmonary hypertension' above and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

For platypnea-orthodeoxia – For patients with documented platypnea-orthodeoxia caused by an ASD in the absence of severe PH, we suggest ASD closure (Grade 2C). (See 'Platypnea-orthodeoxia' above.)

For paradoxical embolism – For patients with ASD with a net left-to-right shunt who have had an embolic-appearing cryptogenic ischemic stroke, we suggest referral to adult CHD and neurology experts to assess the potential benefits and risks of ASD closure. Patients most likely to benefit from ASD closure are those who are young (eg, <60 years of age) without evident source of stroke despite a comprehensive evaluation by a stroke neurologist and a cardiologist. (See 'Paradoxical embolization' above.)

Selected patients with PH secondary to CHD are candidates for advanced therapy for pulmonary arterial hypertension, which may improve symptoms and in some cases may enable the patient to meet criteria for ASD closure, with advanced therapy continued after ASD closure. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis" and 'Pulmonary hypertension' above.)

For patients with an unrepaired ASD, self-contained underwater breathing apparatus (SCUBA) diving is generally contraindicated. (See 'SCUBA diving' above.)

Patients with ASDs (particularly those with PH) are advised to consult with a cardiologist specializing in CHD before high-altitude exposure. (See 'High-altitude exposure' above.)

For women with an ASD contemplating pregnancy, prepregnancy consultation with an adult CHD specialist is suggested. Young women with a small, uncomplicated ASD generally tolerate pregnancy well. Women with an ASD and a history of supraventricular arrhythmias or RV failure are at increased risk for complications during pregnancy. (See 'Pregnancy' above.)

ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Susan Wiegers, MD, FACC, FASE, and Thomas P Graham Jr, MD, who contributed to earlier versions of this topic review.

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Topic 1417 Version 27.0

References

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