Management of Atrial Septal Defect (ASD) in Pediatric Patients
Indications for ASD Closure
The primary indication for ASD closure in pediatric patients is right heart volume overload (right atrial and right ventricular enlargement) with a significant left-to-right shunt (Qp:Qs ≥1.5:1), regardless of symptom presence. 1
Key Decision Points for Intervention
Closure is reasonable when there is evidence of right-sided volume overload with Qp:Qs ≥1.5:1 and systolic pulmonary artery pressure <50% of systemic pressure, with pulmonary vascular resistance <1/3 systemic resistance 1
For children with progressive pulmonary hypertension and right ventricular failure despite optimal medical therapy, ASD intervention (creation or enlargement) may be considered as a palliative measure 1
ASD closure should NOT be performed when pulmonary artery systolic pressure exceeds 2/3 systemic pressure, pulmonary vascular resistance exceeds 2/3 systemic resistance, or there is a net right-to-left shunt 1
Timing of Repair
For secundum ASD with significant shunting, closure before age 40 years may reduce atrial arrhythmias; closure after age 40 has minimal effect on preventing arrhythmias 1
In adults with unrepaired ASD, atrial fibrillation or flutter occurs in approximately 20%, with incidence increasing with patient age 1
Symptomatic infants with intractable heart failure from isolated secundum ASD may require surgical repair in the first year of life, though this presentation is rare 2
Approach to Closure
Percutaneous vs. Surgical Closure
Percutaneous device closure is the preferred approach for secundum ASD when anatomically suitable 3, 4
Surgical closure is indicated for primum ASD, sinus venosus defects, and secundum ASDs not amenable to percutaneous closure 3
Surgical closure is reasonable when performed concomitantly with another cardiac surgical procedure if there is a hemodynamically significant shunt (Qp:Qs ≥1.5:1) causing right heart enlargement 1
Pre-Intervention Evaluation
Essential Diagnostic Studies
Two-dimensional transthoracic echocardiography with Doppler is the central diagnostic modality and should evaluate: ASD anatomical characteristics, flow direction, associated abnormalities (anomalous pulmonary veins), right ventricular anatomy and function, pulmonary pressures, and Qp:Qs ratio 4
Pulse oximetry at rest and with exercise is useful to define shunt direction and identify patients with increased pulmonary arterial resistance and shunt reversal 1
For large secundum ASDs, cardiac MRI or cardiac catheterization should be performed before repair to fully evaluate the defect 3
Transesophageal echocardiography provides excellent visualization of the entire atrial septum and pulmonary venous connections in adults when transthoracic imaging is inadequate 1
Special Considerations for Pulmonary Hypertension
In children with progressive pulmonary hypertension and right ventricular failure despite optimal therapy, ASD intervention (creation/enlargement) is suggested as a conditional recommendation, though evidence certainty is very low 1
ASD intervention in this context provides volume unloading rather than pressure unloading, with relief occurring only when right ventricular diastolic pressures are elevated (typically a late, end-stage process) 1
The procedure has evolved from balloon atrial septostomy to more controlled approaches using atrial stents, fenestrated devices, or atrial flow regulator devices, which may be safer 1
Post-Closure Management
Indefinite follow-up is recommended, especially for larger defects 3
Echocardiography is the most frequently used investigative modality during follow-up visits (>80%), followed by electrocardiography 3
Patients with intracardiac shunts face risk of systemic embolism from clots that may form on pacing leads even in right-sided cardiac chambers 1
Critical Pitfalls to Avoid
Do not close ASDs in patients with severe pulmonary hypertension (PA systolic pressure >2/3 systemic, PVR >2/3 systemic, or net right-to-left shunt), as this causes clinical deterioration 1
Recognize that coexistent sinus node dysfunction is common after surgical repair and can be aggravated by antiarrhythmic therapy, potentially requiring pacemaker implantation 1
Be aware that neurological complications including ischemic stroke can occur within hours after transcatheter closure; frequent coagulation monitoring during and after the procedure is necessary with close observation 5
In patients with Ebstein anomaly and pulmonary stenosis or right heart failure, ASD physiology is substantially more complex, and closure could result in clinical deterioration 1
Management of Autism Spectrum Disorder (ASD) in Pediatric Patients
Core Treatment Approach
Evidence-based structured educational and behavioral interventions are the foundation of Autism Spectrum Disorder treatment, not pharmacotherapy. 6
First-Line Interventions
Early intensive behavioral and developmental interventions, particularly those incorporating applied behavior analysis principles, have the highest-quality data supporting effects on cognitive and language outcomes 1
These interventions can be delivered in home or school settings and are generally time-intensive, with some programs requiring up to 40 hours per week 1
Parent training components and play- or interaction-based interventions should be incorporated into behavioral treatment plans 1
Pharmacotherapy Indications
Pharmacotherapy in ASD should target specific symptoms or comorbid conditions rather than core features of autism. 6
FDA-Approved Medication
Risperidone is FDA-approved for treatment of irritability associated with autistic disorder in children and adolescents ages 5-17 years, including symptoms of aggression toward others, deliberate self-injuriousness, temper tantrums, and quickly changing moods 7
Efficacy was established in three short-term trials in children and adolescents 7
Weight gain is common during risperidone treatment in pediatric patients; clinical monitoring of weight is recommended throughout treatment 7
Somnolence was frequently observed in placebo-controlled trials, with most cases mild or moderate in severity, early onset (peak during first two weeks), and transient (median duration 16 days) 7
Risperidone elevates prolactin levels; in placebo-controlled studies, 49% of pediatric patients receiving risperidone had elevated prolactin compared to 2% on placebo 7
Management of Co-occurring ADHD
When ADHD co-occurs with autism, ADHD medications can be safely prescribed, but require additional oversight and collaboration between prescribing clinicians and specialists. 8
Behavioral therapies can be transdiagnostic and address challenges associated with both autism and ADHD symptoms 8
In children with congenital heart disease, autism, and ADHD, medication decisions must be made in collaboration with cardiology 8
Complementary/Alternative Treatments
Leukovorin (Folinic Acid)
The American Academy of Child and Adolescent Psychiatry does not recommend leukovorin as a standard treatment for ASD core symptoms, considering it a complementary/alternative medicine approach without sufficient evidence for routine use 6
Leukovorin should not replace established evidence-based interventions for ASD 6, 9
If cerebral folate deficiency is confirmed, a trial of leukovorin might be considered under specialist supervision with appropriate monitoring 6
The primary risk of leukovorin treatment is bone marrow suppression (neutropenia, anemia, thrombocytopenia), though this risk is generally low when used as standalone treatment 9
Complete blood count monitoring should be considered, particularly with higher doses 6, 9
Screening Recommendations
The U.S. Preventive Services Task Force concludes there is insufficient evidence to assess the balance of benefits and harms of screening for ASD in young children for whom no concerns have been raised 1
Clinicians should listen carefully to parents when concerns are raised and make prompt use of validated tools (such as M-CHAT-R/F) to assess the need for further diagnostic testing and services 1
The M-CHAT-R/F is a parent-rated scale; a positive finding leads to a follow-up interview, and if the follow-up interview is positive, a full diagnostic workup for ASD is indicated 1
Management of Common Comorbidities
Constipation
Establish proper toilet posture including buttock support, foot support, and comfortable hip abduction to prevent activation of abdominal muscles and pelvic floor co-contraction 10
Implement timed toileting routines, particularly scheduling regular bathroom visits after meals to create consistent toileting habits 10
Maintenance phase of bowel management may need to be continued for many months; initial disimpaction with oral laxatives may be necessary before maintenance therapy 10
Do not discontinue maintenance therapy too early, before the child regains bowel motility and rectal perception 10
Critical Pitfalls to Avoid
Do not use pharmacotherapy to treat core features of autism; medications should only target specific symptoms or comorbid conditions 6
Recognize that disparities exist in frequency and age of ASD diagnosis by race/ethnicity, socioeconomic status, and language of origin; certain groups may be systematically underdiagnosed 1
Long-term effects of risperidone on growth and sexual maturation have not been fully evaluated in children and adolescents 7
Tardive dyskinesia, though rare (0.1% in clinical trials), can occur with risperidone; it resolved upon discontinuation in reported cases 7