Treatment Options for Atrial Septal Defects
Primary Treatment Recommendation
Closure of hemodynamically significant ASDs (those causing right atrial and right ventricular enlargement) is indicated regardless of symptoms, with percutaneous device closure preferred for secundum defects and surgical closure required for all other anatomic subtypes. 1, 2
Treatment Algorithm by ASD Type and Clinical Scenario
Secundum ASD
Percutaneous Device Closure (First-Line)
- Indicated when: Right atrial and RV enlargement present with or without symptoms 1, 3
- Device options: Amplatzer septal occluder (most defects) and HELEX septal occluder (small-to-medium defects) are FDA-approved 1, 4
- Advantages: Avoids cardiopulmonary bypass, no sternotomy scar, shorter hospitalization, lower periprocedural morbidity compared to surgery 5, 4
- Success rates: Comparable to surgical outcomes in carefully selected patients with low complication rates 1
Surgical Closure (Alternative Approach)
- Reasonable when: Concomitant tricuspid valve repair/replacement needed, or anatomy precludes percutaneous device use 1
- Technique: Pericardial patch closure or direct suture closure via right thoracotomy or sternotomy 1
- Mortality: Approximately 1% in absence of pulmonary arterial hypertension (PAH) or major comorbidities 1, 3
- Outcomes: Excellent long-term results with symptom improvement and reduced atrial arrhythmia incidence when concomitant Maze procedure performed 1
Non-Secundum ASDs (Sinus Venosus, Coronary Sinus, Primum)
Surgical Closure (Mandatory)
- All non-secundum defects require surgical repair rather than percutaneous closure 1, 3
- Sinus venosus defects: May require Warden procedure (superior vena cava translocation to right atrial appendage) when anomalous pulmonary venous drainage enters mid/upper superior vena cava 1
- Additional repairs: Anomalous pulmonary venous drainage must be corrected; significant tricuspid regurgitation requires valve repair 1
- Surgeon expertise: Operations must be performed by surgeons with training and expertise in congenital heart disease 1
Specific Clinical Indications for Closure
Class I (Definite Indications)
Class IIa (Reasonable Indications)
- Paradoxical embolism 1
- Documented orthodeoxia-platypnea syndrome 1
- Asymptomatic patients with right atrial and RV enlargement, Qp:Qs ≥1.5:1, PA systolic pressure <50% systemic, and PVR <1/3 systemic vascular resistance 2
Class IIb (May Be Considered)
- Net left-to-right shunt with PA pressure <2/3 systemic levels and PVR <2/3 systemic vascular resistance 1
- Responsive to pulmonary vasodilator therapy or test occlusion (requires pulmonary hypertension specialist involvement) 1, 2
- Concomitant Maze procedure for intermittent or chronic atrial tachyarrhythmias 1
Critical Hemodynamic Thresholds and Contraindications
When Closure May Be Considered (Borderline PAH)
- PA systolic pressure 50-67% of systemic pressure 2
- PVR 1/3 to 2/3 of systemic resistance 2
- Requires: Careful evaluation by pulmonary hypertension specialists 1, 2, 3
Absolute Contraindications (Class III)
- Severe irreversible PAH with no evidence of left-to-right shunt 1
- PA systolic pressure >2/3 systemic 2
- PVR >2/3 systemic resistance 2
- Net right-to-left shunt (Eisenmenger physiology) 2
- Critical warning: Closure with established severe pulmonary vascular disease causes acute RV failure and death 2
Small Defects (<5 mm)
- Generally do not require closure unless associated with paradoxical embolism 1, 2, 3
- No evidence of RV volume overload and no impact on natural history 1, 2
Special Considerations and Adjunctive Therapies
Arrhythmia Management
- Atrial arrhythmias should be treated to restore and maintain sinus rhythm 1
- Anticoagulation required for atrial fibrillation 1
- Pre-closure ablation: Reasonable for patients with supraventricular tachycardia undergoing ASD closure 1
- Concomitant Maze procedure: May be performed for intermittent/chronic atrial fibrillation/flutter during surgical closure 1
Timing Considerations
- Closure before age 25 years associated with better long-term outcomes and lower incidence of atrial arrhythmias 1, 6
- Closure even later in life improves morbidity and survival, though associated with new (7%) or recurrent (60%) atrial tachycardia 1
- Larger defects with RV volume overload typically cause symptoms in third decade of life 1
Post-Procedure Monitoring
- Monitor for postpericardiotomy syndrome symptoms: fever, fatigue, vomiting, chest pain, abdominal pain (may indicate tamponade) 3
- Echocardiography to assess device position/stability, residual shunting, pericardial effusion, and RV function 3
- Long-term complications: Device thrombosis, cardiac erosion (most severe), atrial arrhythmias (most common), nickel allergy, conduction abnormalities, valvular damage, device endocarditis 5
Critical Pitfalls to Avoid
Do not assume small shunts are benign in older adults - acquired conditions can increase left-to-right shunting over time, making previously insignificant ASDs hemodynamically relevant 2
Always exclude severe PAH before closure - this is the most critical assessment, as closure with established severe pulmonary vascular disease is fatal 2
Do not attempt percutaneous closure of non-secundum defects - sinus venosus, coronary sinus, and primum ASDs require surgical expertise 1, 3
Beware of unexpected anatomy during surgery - surgeons not trained in congenital heart disease may encounter unexpected primum ASD or partial anomalous pulmonary venous drainage 1