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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.

Clinical question: How should clinicians evaluate ASD anatomy and hemodynamics, determine closure candidacy, and select transcatheter versus surgical repair?

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. jaccTranscatheter 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. BMJInteratrial 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%. jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review

Hemodynamic findings that direct ASD closure decisions. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlancejaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome
FindingInterpretationNext action
Right-heart enlargement with net left-to-right shuntHemodynamically consequential ASD; closure prevents ongoing right-sided volume load. jaccInterventional 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 ... - JACCDefine anatomy and assess suitability for percutaneous versus surgical closure. jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewACCAtrial Septal Defect in Adulthood - American College of Cardiology
Qp:Qs ≥1.5:1 with primum, sinus venosus, or coronary sinus ASD; systolic PA pressure <50% systemic; PVR <one third systemic vascular resistanceMeets guideline conditions supporting surgical repair when there is impaired functional capacity and right atrial and/or RV enlargement. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlanceRefer for surgical repair unless comorbidity precludes intervention. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glance
Pulmonary hypertension with high or uncertain PVRClosure can precipitate intolerance if pulmonary vascular disease is advanced. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict OutcomePerform invasive hemodynamic assessment and evaluate pulmonary vascular responsiveness before deciding on closure. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USAHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal Echocardiography
Right-to-left shunt with saturation <94% or PVR >7 Wood unitsReported contraindication/exclusion profile for transcatheter ASD closure. jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewAvoid routine device closure; manage through specialized ACHD and pulmonary hypertension evaluation. jaccTranscatheter 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 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 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. jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCACCAtrial Septal Defect in Adulthood - American College of Cardiology

ASD subtype determines associated-lesion assessment and closure route. BMJCongenital heart disease in aircrewjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Reviewjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCACCAtrial Septal Defect in Adulthood - American College of Cardiology
SubtypeHigh-value anatomic issueUsual closure approach
Secundum ASDFossa ovalis location; define defect size and septal rims for device anchoring. jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewACCAtrial Septal Defect in Adulthood - American College of CardiologyTranscatheter device closure is preferred when anatomy is feasible; surgery is used for large defects, inadequate rims, or associated lesions requiring repair. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USACCAtrial Septal Defect in Adulthood - American College of Cardiology
Primum ASDPartial atrioventricular septal defect anatomy and potential mitral valve dysfunction. BMJCongenital heart disease in aircrewjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCSurgical repair; not suitable for device closure. BMJCongenital heart disease in aircrewjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
Sinus venosus ASDConfirm anomalous pulmonary venous return, especially on the right side; TEE may be needed after nondiagnostic TTE. AHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyACCAtrial Septal Defect in Adulthood - American College of CardiologyHistorically surgical repair; covered-stent transcatheter intervention has emerged for selected sinus venosus defects. ACCAtrial Septal Defect in Adulthood - American College of Cardiology
Coronary sinus ASDUnroofed coronary sinus anatomy. jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCSurgical closure. jaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review

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. jaccInterventional 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. jacc2025 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. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review

Practical selection of ASD closure route. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USBMJCongenital heart disease in aircrewjacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlancejaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewACCAtrial Septal Defect in Adulthood - American College of Cardiology
Clinical-anatomic settingPreferred strategyReason to avoid routine device closure
Hemodynamically consequential secundum ASD with suitable rimsPercutaneous device closure. ACCAtrial Septal Defect in Adulthood - American College of CardiologyChoose surgery if the defect is large, rims are inadequate, or associated lesions need repair. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review
Primum ASDSurgical repair. BMJCongenital heart disease in aircrewjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety ReviewPrimum anatomy is not suitable for device closure and may involve mitral valve dysfunction. BMJCongenital heart disease in aircrew
Sinus venosus ASD with anomalous pulmonary venous returnSurgical repair is the established approach; selected superior lesions may undergo technically feasible transcatheter closure. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlanceACCAtrial Septal Defect in Adulthood - American College of CardiologyPulmonary venous anatomy requires correction and must be fully defined. AHA JournalsDiagnosis of Sinus Venosus Atrial Septal Defect With Transesophageal EchocardiographyACCAtrial Septal Defect in Adulthood - American College of Cardiology
ASD with advanced pulmonary vascular disease or right-to-left shuntingSpecialized individualized assessment; fenestrated repair may be considered only when closure remains indicated. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlanceComplete closure may not be tolerated when PVR remains high or Eisenmenger physiology is present. BMJInteratrial communications (atrial septal defects) - Symptoms, diagnosis and treatment | BMJ Best Practice USjaccTranscatheter Device Closure of Atrial Septal Defects: A Safety Review

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. jacc2025 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. jacc2025 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. ACCClinical 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

Features requiring more intensive post-repair surveillance. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glancejacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict OutcomeACCClinical 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
FeatureWhy it mattersFollow-up focus
Closure later in adulthoodAtrial arrhythmia occurs more frequently in patients closed later in life. jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCInvestigate symptomatic rhythm disturbance and maintain congenital-cardiology follow-up. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-Glancejacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
Preclosure pulmonary hypertension or elevated PVREarly clinical improvement does not exclude progressive pulmonary hypertension. ScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict OutcomeReassess pulmonary hemodynamics and manage with ACHD/pulmonary hypertension expertise. jacc2025 Adults With Congenital Heart Disease Guideline-at-a-GlanceScienceDirectProgressive Pulmonary Hypertension Post Atrial Septal Defect Device Closure—Early Symptomatic Improvement may not Predict Outcome
Primum, sinus venosus, or coronary sinus anatomyThese lesions have associated structural complexity beyond isolated secundum ASD. jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCACCClinical Practice Algorithm For the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects - American College of CardiologyUse lesion-specific ACHD surveillance rather than an isolated secundum-ASD pathway. ACCClinical 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

References

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