Adult Congenital Cardiology
Atrial Septal Defect
Evaluate ASD by anatomic subtype, right-heart volume loading, shunt magnitude, and pulmonary vascular status. Close hemodynamically consequential defects before irreversible pulmonary vascular disease, selecting transcatheter closure only for suitable secundum anatomy and surgery for primum, most sinus venosus, coronary sinus, or associated lesions.
First decision
Identify patients in whom closure may be unsafe
Pulmonary vascular disease and right-to-left shunting change the closure decision before device planning.
In an unrepaired ASD with suspected pulmonary hypertension, cyanosis, exertional desaturation, right-to-left or bidirectional shunting, or right-heart failure, determine whether the defect remains a net left-to-right volume lesion versus an advanced pulmonary vascular lesion. Unrepaired ASD can progress to right ventricular volume overload, right-heart failure, elevated pulmonary vascular resistance, atrial arrhythmias, systemic embolism, and, in a minority, pulmonary arterial hypertension with right-to-left shunting. jacc+1jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Reviewjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
Obtain hemodynamic assessment when pulmonary hypertension is present or suspected and closure is under consideration. The preclosure question is whether pulmonary vascular resistance is sufficiently responsive and whether a net left-to-right shunt persists; high pulmonary vascular resistance may make corrective closure intolerable, whereas a Qp:Qs greater than 1.5:1 with only modest resistance elevation is comparatively reassuring in reported experience. BMJ+2BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USAHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome
Do not proceed directly to conventional closure in Eisenmenger physiology or clinically important atrial-level right-to-left shunting. Reported exclusion criteria for device closure include pulmonary vascular resistance above 7 Wood units and right-to-left atrial shunting with peripheral arterial saturation below 94%. jaccjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
Escalate to an adult congenital heart disease and pulmonary hypertension team when pulmonary hypertension, resting or exertional cyanosis, bidirectional shunting, or ventricular decompensation is present; the decision may require medical pulmonary vascular management and consideration of a fenestrated rather than complete repair. BMJ+2BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlanceScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome
Defer elective device closure in sepsis, serious infection within 1 month, intracardiac thrombus, unstable angina, recent myocardial infarction, or decompensated heart failure; these are reported procedural contraindications or exclusion conditions. jaccjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
Imaging
Define ASD subtype before choosing a closure strategy
Anatomic misclassification can send a patient to the wrong intervention.
Start with transthoracic echocardiography to establish right-sided chamber size, shunt direction, and apparent septal anatomy. A subcostal approach is particularly useful when the ultrasound beam is perpendicular to the atrial septum; however, transthoracic imaging can be suboptimal in older patients and may miss sinus venosus anatomy. Wolters Kluwer+1Wolters KluwerCongenital heart disease - A : Heart ViewsAHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal Echocardiography
Use transesophageal echocardiography when transthoracic imaging does not confidently define the defect, rim adequacy, or pulmonary venous anatomy. TEE better visualizes the interatrial septum and is particularly important for suspected sinus venosus defect, for which transthoracic imaging has limited yield because the lesion is far-field; TEE should also establish right-sided anomalous pulmonary venous connections and confirm normal left-sided pulmonary venous connections. AHA Journals+1AHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyWolters KluwerCongenital heart disease - A : Heart Views
Classify the lesion before referral: secundum defects occur in the fossa ovalis and account for about 80% of isolated ASDs; primum defects account for approximately 15% and are part of the atrioventricular septal defect spectrum; superior and inferior sinus venosus defects are less common and are associated especially with partial anomalous pulmonary venous return; unroofed coronary sinus is rare. jacc+1jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCACCAtrial Septal Defect in Adulthood - American College of Cardiology
A suspected primum ASD should trigger assessment of mitral valve function and evaluation within the partial atrioventricular septal defect framework; primum anatomy is not suitable for device closure. BMJ+1BMJCongenital heart disease in aircrewjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
A suspected superior sinus venosus defect requires deliberate assessment for anomalous pulmonary venous return; the surgical technique differs from that for secundum ASD, making definitive anatomic confirmation essential. AHA Journals+1AHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyACCAtrial Septal Defect in Adulthood - American College of Cardiology
Use color-flow and saline contrast imaging to characterize shunt direction when bidirectional flow is suspected. AHA JournalsAHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal Echocardiography
Intervention
Close defects producing right-heart volume overload
Symptoms are not required when objective right-heart enlargement establishes physiologic consequence.
In adults with secundum ASD, right atrial and right ventricular enlargement supports surgical or percutaneous closure with or without symptoms. This strategy is intended to reduce late morbidity from ongoing right-sided volume overload and is supported by consensus recommendations in adults with right-heart enlargement. jacc+1jaccInterventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelinesjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
For primum, sinus venosus, and coronary sinus defects, surgical repair is recommended when impaired functional capacity, right atrial and/or right ventricular enlargement, and a physiologically important net left-to-right shunt such as Qp:Qs at least 1.5:1 are present, provided there is no resting or exertional cyanosis, systolic pulmonary artery pressure is less than 50% of systemic pressure, and pulmonary vascular resistance is less than one third of systemic vascular resistance. jaccjacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glance
Use surgery rather than attempting a marginal device procedure when the defect is large, adequate rims are absent, or another congenital lesion requires operative correction. This is especially relevant for primum and most sinus venosus defects, including those with partial anomalous pulmonary venous drainage. BMJ+1BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
Choose transcatheter closure for a secundum defect only after imaging confirms a secure device landing zone; inadequate rims increase the likelihood that surgery is the safer definitive option. BMJ+1BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
For unrepaired superior sinus venosus ASD, transcatheter closure may be a reasonable alternative to surgery when technically feasible, with the aim of reducing operative morbidity. jaccjacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glance
If ASD closure is indicated in pulmonary arterial hypertension, consider fenestrated repair rather than complete closure in selected patients; guideline language supports this as a consideration to improve functional class and clinical outcomes. jaccjacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glance
Longitudinal care
Monitor residual hemodynamic and rhythm risk after repair
Closure corrects the shunt but does not erase preexisting arrhythmia or pulmonary vascular risk.
After closure, reassess right-sided chamber remodeling, residual shunt, pulmonary pressure, and symptoms with echocardiography directed by the anatomy and preintervention physiology. Patients closed later in life have an increased frequency of atrial arrhythmia, so new palpitations, decline in exercise tolerance, or embolic events should trigger rhythm evaluation rather than attribution to a repaired defect alone. jaccjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
Maintain adult congenital heart disease involvement for patients with complex anatomy, prior pulmonary hypertension, residual lesions, or arrhythmias. Contemporary multisociety guidance emphasizes routine care at ACHD centers in collaboration with ACHD cardiologists and multidisciplinary management for more complex cases. jaccjacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glance
Do not extrapolate uncomplicated repaired secundum-ASD follow-up to primum, sinus venosus, or coronary sinus defects. The ACC secundum-ASD follow-up algorithm specifically excludes those lesions because they are associated with more complex anatomy; follow-up should instead account for repaired atrioventricular valve disease, pulmonary venous repair, pulmonary vascular disease, and arrhythmia burden as applicable. ACC+1ACCClinical Practice Algorithm For the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects - American College of CardiologyACCClinical Practice Algorithm For the Follow-Up of Repaired and Unrepaired Atrioventricular Septal Defects - American College of Cardiology
Re-evaluate pulmonary vascular status if pulmonary hypertension persists or progresses despite early symptomatic improvement after closure; adverse outcomes have been reported in patients with elevated PVR despite initial improvement. ScienceDirectScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome
In repaired sinus venosus ASD, ensure follow-up includes the consequence of the pulmonary venous abnormality that required anatomic correction. AHA Journals+1AHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyACCAtrial Septal Defect in Adulthood - American College of Cardiology
References
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- Transcatheter Device Closure of Atrial Septal Defects: A Safety Review — www.jacc.org · www.jacc.org
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- Diagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal Echocardiography — www.ahajournals.org · www.ahajournals.org
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- Progressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome — www.sciencedirect.com · www.sciencedirect.com
- Recent review of transcatheter closure of atrial septal defect — onlinelibrary.wiley.com · onlinelibrary.wiley.com
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- Clinical Practice Algorithm For the Follow-Up of Repaired and Unrepaired Atrioventricular Septal Defects - American College of Cardiology — www.acc.org · www.acc.org
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