Background
Ventricular septal defects (VSDs) are the most common form of congenital heart disease (CHD) diagnosed in childhood.¹ There is wide variation in clinical practice for the management of common types of VSDs as noted by Hokanson et al.² and Frandsen et. al.³ Over 90% of cardiologists recommended additional assessment for a small muscular VSD, regardless of the clinical scenario, and 36-43% of respondents recommended a repeat echocardiogram at some point in the future with an average follow-up time of around 15 weeks from diagnosis during the neonatal period.² These recommendations were made despite evidence that 89-97% of these lesions close spontaneously,^4,5 and surgical or medical management changes are rarely, if ever, made.³

The goal of the Muscular and Non-muscular VSD Clinical Practice Algorithms is to provide cardiologists and other healthcare providers with a decision-support tool for clinical evaluation and follow-up testing of these common CHDs. This guideline is not meant to replace clinical judgment of healthcare providers but rather improve the value of care by reducing practice variation and optimizing resource utilization in this common CHD.

Methods: Algorithm Development
The CHD Clinical Practice Algorithm for Muscular and Non-muscular VSDs were derived by the Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section. This algorithm was developed in a similar manner as the secundum ASD algorithm.⁶ Existing guidelines were utilized when applicable.^7-9 Important considerations included are as follows: age at diagnosis, VSD size and location, presence of cardiac symptoms, and shunt direction. Patients with associated cardiac lesions other than patent foramen ovale (PFO) were excluded. For the purpose of algorithm development, VSDs were divided into two broad categories, muscular and non-muscular, due to differences in natural history of the lesions. Regardless of the size of the defect, non-muscular VSDs can be associated with aortic valve prolapse, double chamber right ventricle, and subaortic membrane. The potential for these associations as well as progressive left sided enlargement were the basis for ongoing testing in the unrepaired non-muscular VSD population. The presence of symptoms of pulmonary over circulation are an indication for medical therapy followed by surgical or catheter-based intervention in muscular VSDs and surgical intervention in non-muscular VSDs in the event that medical therapy is unsuccessful or if there is persistent left heart enlargement on echocardiography. Small muscular VSDs can be followed clinically every 2-5 years without further testing or echocardiography until the murmur has resolved. Alternatively, if the providers and family are comfortable with the decision, small muscular VSDs may be discharged from cardiology clinic with primary care follow up only.

Repaired VSDs were reviewed based on type of closure: either a) catheterization device closure or b) surgical repair, in addition to the type of VSD. Post-closure algorithm development accounted for specific device protocols as well as standard institutional practice following initial surgical repair. The presence and type of residual VSDs were included in algorithm development and determined timing of follow up and need for additional testing.

The adult Unrepaired VSD algorithm was developed based on the current Adult Congenital Heart Disease ACC/American Heart Association (AHA) and European Society of Cardiology (ESC) guidelines.^8,9 Special considerations in the adult with an unrepaired VSD include the potential for pulmonary arterial hypertension (PAH) and Eisenmenger syndrome. These were also utilized in the pediatric algorithms with a goal of a seamless transition from pediatric to adulthood.

Challenges to the algorithm development included the need for ongoing follow-up and testing which may be influenced by provider and institutional preferences, accounting for the potential risk of endocarditis, definition of clinically significant aortic valve complications, and the variability in VSD nomenclature as well as size categorization. Notwithstanding these difficulties, the goal of the Working Group was to provide an easy-to-use clinical tool that may help standardize the approach to clinical follow-up and testing for this common and simple yet heterogeneous CHD. Cardiology providers may use this algorithm as a reference point when seeing these patients in the outpatient setting as well as a framework for creating institutional/practice-based quality improvement initiatives.

Future Directions
The aim of the ACC ACPC Quality Working Group is to provide clinical practice tools for cardiology providers with the ultimate goal of reducing practice variability for mildly complex CHD. In addition, the use of these algorithms for the education of non-cardiologists will be necessary to prevent inappropriate referrals and unnecessary resource utilization.

References

1. Minette MS, Sahn DJ. Ventricular septal defects. Circulation 2006;114:2190-97.
2. Hokanson JS, Ring K, Zhang X. A survey of pediatric cardiologists regarding non-emergent echocardiographic findings in asymptomatic newborns. Pediatr Cardiol 2022;43:837-43.
3. Frandsen EL, House AV, Xiao Y, Danford DA, Kutty S. Subspecialty surveillance of long-term course of small and moderate muscular ventricular septal defect: heterogenous practices, low yield. BMC Pediatrics 2014;14:282.
4. Roguin N, Du ZD, Barak M, Nasser N, Hershkowitz S, Milgram E. High prevalence of muscular ventricular septal defect in neonates. J Am Coll Cardiol 1995;26:1545-48.
5. Zhao QM, Niu C, Liu F, Wu L, Ma XJ, Huang GY. Spontaneous closure rates of ventricular septal defects (6,750 consecutive neonates). Am J Cardiol 2019;124:613-17.
6. Plummer ST, Parthiban A, Sachdeva R, Zaidi AN, Statile C. Clinical Practice Algorithm for the Follow-up or Unrepaired and Repaired Secundum Atrial Septal Defects. http://www.acc.org. Mar 08, 2022. Accessed [insert access date]. 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.
7. Sachdeva R, Valente A, Armstrong A, 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. J Am Coll Cardiol 2020;75:657–703.
8. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42:563-645.
9. 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.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Valvular Heart Disease, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement, Noninvasive Imaging, Pulmonary Hypertension and Venous Thromboembolism

Keywords: Heart Defects, Congenital, Eisenmenger Complex, Follow-Up Studies, Foramen Ovale, Patent, Outpatients, Patient Discharge, Institutional Practice, Heart Septal Defects, Ventricular, Aortic Valve Prolapse, Heart Murmurs, Endocarditis, Algorithms, Catheterization, Catheters, Prolapse

< Back to Listings