Clinical Practice Algorithm For the Follow-Up of Repaired and Unrepaired Atrioventricular Septal Defects
Quick Takes
- Timing of surgical referral for pediatric atrioventricular septal defect (AVSD) is dependent on the subtype of AVSD with repair in infancy (3-6 months) for complete AVSD and repair during toddler years (12-36 months) for partial/transitional AVSD.
- Left atrioventricular valve insufficiency and left ventricular outflow tract obstruction are the most common postoperative complications and may warrant frequent follow-up and potential reintervention.
- Older children and adults with unrepaired AVSD may require diagnostic cardiac catheterization before surgery, with additional management decisions guided by hemodynamic findings.
Background
Approximately 2,700 children (~5/10,000 live births) are born annually with atrioventricular septal defect (AVSD).1 AVSD represents a spectrum of congenital heart lesions that result from abnormal embryologic development of the endocardial cushions in the primitive atrioventricular (AV) canal. Primum atrial septal defects (ASDs), inlet ventricular septal defects (VSDs), and abnormalities of the AV valve are among the possible pathologic features of AVSD. AVSD can be subclassified on the basis of the anatomy of the AV valve and the degree of shunting at the ventricular level. Patients with AVSD may require medical management with diuretics, afterload reduction, and nutritional support. AVSD nearly always requires surgical repair, with timing of repair determined by the specific anatomical type of AVSD and associated physiology. For patients with a significant ventricular-level shunt, surgical repair is typically performed in the first 3-6 months of life, with optimal timing based on the ability to adequately medically manage symptoms of pulmonary overcirculation and maintain adequate growth.2,3 In partial and transitional AVSD anatomy, surgical repair is typically performed later in keeping with the physiology of a primary atrial-level shunt.4 For all patients, long-term surveillance for residual lesions is required and reintervention may be required in as many as 10-25% with the most common indications for reintervention being residual left AV valve regurgitation and left ventricular outflow tract obstruction.5 To date, there are limited resources available to guide clinicians regarding frequency and timing of follow-up before and after repair, resulting in practice variation across providers and institutions. The goal of this clinical care algorithm is to provide a guideline for preoperative and postoperative management of pediatric and adult patients with AVSD to aid clinicians and standardize care across institutions.
Goals and Details of the Algorithm
Three separate pathways were developed for management of AVSD: 1) preoperative pediatric patients; 2) preoperative adult patients, and; 3) postoperative pediatric and adult patients. Patients were excluded if any of the following were present: additional cardiac defects outside of AVSD, the AVSD was considered unbalanced, prematurity <32 weeks, genetic conditions other than trisomy 21, and/or pregnancy. General considerations included the subtype of AVSD and consequently whether patients would solely have an ASD or both an ASD and a VSD. Subtypes of AVSD with hemodynamically significant VSDs result in higher likelihood of symptoms, need for earlier surgical repair than in patients without a VSD, and need for assessment of pulmonary arterial hypertension in unoperated older children and adults. The degree of AV valve regurgitation also influenced recommendations for frequency and timing of follow-up care. Clinical follow-up alone was recommended in patients with AVSD who only had a small ASD without abnormalities of the AV valve and without evidence of significant left-to-right shunt and/or right heart volume overload. Precise recommendations regarding frequency of imaging in follow-up (i.e., echocardiogram) were not provided owing to the unique physiology of each patient and to allow clinicians to determine imaging needs on the basis of patient presentation.
Methods: Algorithm Development
The Clinical Practice Algorithm for the Follow-Up of Repaired and Unrepaired AVSDs was developed to provide clinical guidance similar to the algorithms previously developed by the Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section.6 The working group comprises experienced pediatric and adult congenital cardiologists with multidisciplinary expertise and diverse training and practice backgrounds. The working group reviewed the medical literature to identify studies that could provide evidence to support the clinical practice algorithm. Established guidelines were referenced and used when available.7-10 The algorithm underwent iterative refinement by members of the working group to ensure consensus.
Future Directions
This guideline was designed as a reference tool to assist health care clinicians with standardizing care across institutions, reducing practice variation, and improving resource utilization. These guidelines are intended to be a reference for clinicians but are not intended to replace clinical judgment. These guidelines may require adjustment on the basis of the complexity of the patient, provider and institutional practice patterns, or new evidence arising that contradicts the recommendations in these guidelines.
References
- Mai CT, Isenburg JL, Canfield MA, et al. National population-based estimates for major birth defects, 2010-2014. Birth Defects Res 2019;111:1420-35.
- Karolcik BA, Rao SO, Lucas JF, et al. Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect. Cardiol Young 2023;33:766-70.
- Goutallier CS, Buratto E, Schulz A, et al. Repair of complete atrioventricular septal defect between 2 and 3.5 kilograms: defining the limits of safe repair. J Thorac Cardiovasc Surg 2022;164:1167-75.
- Mery CM, Zea-Vera R, Chacon-Portillo MA, et al. Contemporary results after repair of partial and transitional atrioventricular septal defects. J Thorac Cardiovasc Surg 2019;157:1117-27.
- Fong LS, Betts K, Ayer J, et al.; Australian CAVSD study group. Predictors of reoperation and mortality after complete atrioventricular septal defect repair. Eur J Cardiothorac Surg 2021;61:45-53.
- Plummer ST, Parthiban A, Sachdeva R, Zaidi AN, Statile C. Clinical Practice Algorithm for the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects (American College of Cardiology website). 2022. Available at: https://www.acc.org/Latest-in-Cardiology/Articles/2022/03/08/19/34/Clinical-Practice-Algorithm-For-the-Follow-up-of-Unrepaired-and-Repaired-SASD. Accessed 12/20/2024.
- Sachdeva R, Valente AM, Armstrong AK, et al. ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 appropriate use criteria for multimodality imaging during the follow-up care of patients with congenital heart disease: a report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography. J Am Coll Cardiol 2020;75:657-703.
- Baumgartner H, De Backer J, Babu-Narayan SV, et al.; ESC Scientific Document Group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42:563-645.
- Stout KK, Daniels CJ, Aboulhosn JA, et al. 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 Guidelines. J Am Coll Cardiol 2019;73:e81-e192.
- Otto CM, Nishimura RA, Bonow RO, et al.; Writing Committee Members. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol 2021;77:e25-e197.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement
Keywords: Heart Defects, Congenital, Heart Septal Defects, Ventricular, Heart Septal Defects, Pediatrics, Quality Improvement