Background

Ventricular septal defects (VSDs) are the most common form of congenital heart disease (CHD) diagnosed in childhood.¹ There is a wide variation in clinical practice for the management of common types of VSDs.^2,3 Over 90% of cardiologists recommended additional assessment for a small muscular VSD, regardless of the clinical scenario; 36-43% recommend a repeat echocardiogram at some point in the future, with a mean follow-up interval of approximately 15 weeks from the time of diagnosis during the neonatal period.² These recommendations are despite evidence that 89-97% of these lesions close spontaneously, and surgical or medical management changes are rarely, if ever, made.^3-5 VSDs other than small muscular VSDs, repaired and unrepaired, will require surveillance because they can cause symptoms or have associated complications.

Goals and Details of the Algorithm

The goal of the muscular and nonmuscular VSDs clinical practice algorithm is to provide cardiologists and other health care providers with a decision-support tool for clinical evaluation and follow-up testing of VSDs (Figures 1a, b, c). This guidance is not meant to replace health care providers' clinical judgment but rather to improve the value of care by reducing practice variation and optimizing resource utilization.

Figure 1a: Outpatient Management of Isolated Muscular VSD

Reprinted with permission from Writing Committee, Sachdeva R, Parthiban A, et al. Outpatient management of isolated left-to-right shunt lesions in pediatric patients: 2026 ACC concise clinical guidance: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. Published online March 26, 2026. doi:10.1016/j.jacc.2025.11.020
ECG = electrocardiogram; PCP = primary care provider; TTE = transthoracic echocardiography; VSD = ventricular septal defect.

Figure 1b: Outpatient Management of Isolated Nonmuscular VSD

Reprinted with permission from Writing Committee, Sachdeva R, Parthiban A, et al. Outpatient management of isolated left-to-right shunt lesions in pediatric patients: 2026 ACC concise clinical guidance: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. Published online March 26, 2026. doi:10.1016/j.jacc.2025.11.020
*Complications include AoV prolapse and AR, progressive left heart enlargement, and the development of a DCRV or subaortic membrane.
†Some infants have no symptoms due to elevated PVR and need repair early.
AoV = aortic valve; AR = aortic regurgitation; DCRV = double-chambered right ventricle; ECG = electrocardiogram; PCP = primary care provider; PVR = pulmonary vascular resistance; TTE = transthoracic echocardiography; VSD = ventricular septal defect.

Figure 1c: Outpatient Management of Repaired VSD

Reprinted with permission from Writing Committee, Sachdeva R, Parthiban A, et al. Outpatient management of isolated left-to-right shunt lesions in pediatric patients: 2026 ACC concise clinical guidance: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. Published online March 26, 2026. doi:10.1016/j.jacc.2025.11.020
*Complications include persistent LV dilation or dysfunction and greater-than-mild AR.
AR = aortic regurgitation; ECG = electrocardiogram; LV = left ventricle; TTE = transthoracic echocardiography; VSD = ventricular septal defect.

Important considerations included age, VSD size and location, presence of cardiac symptoms, and shunt direction. Patients with associated cardiac lesions other than patent foramen ovale were excluded. Regardless of the type of VSD, small defects in children are typically asymptomatic and hemodynamically insignificant whereas moderate to large VSDs can have tachypnea, poor feeding, failure to thrive, and progressive left heart enlargement and, if left untreated, can ultimately progress to Eisenmenger syndrome.

For the purpose of algorithm development and long-term surveillance, VSDs were divided into two broad categories—muscular and nonmuscular—due to difference in natural history of the lesions. Small muscular VSDs can be followed clinically every 3-5 years without further testing, including echocardiography, until the murmur resolves, at which time the patients can be discharged from the cardiology clinic. Alternatively, if the primary care provider, cardiologist, and family are comfortable with the decision, patients with small muscular VSDs may be discharged from the cardiology clinic using a shared decision-making model. Moderate and large muscular VSDs should be followed more closely in infants due to the expected decrease in pulmonary vascular resistance, and medical therapy should be initiated if clinical signs and symptoms of pulmonary overcirculation appear. Infants and children with symptoms or persistent left heart dilation should be referred for intervention. Notably, echocardiography does not need to be performed at every visit prior to intervention but should be intermittently performed to assess for left heart size and any other associated complications.

Initial surveillance in children with nonmuscular VSDs is like those with muscular VSDs, with a focus on left heart enlargement and symptoms of pulmonary overcirculation. However, unrepaired nonmuscular VSDs have additional risks beyond left heart enlargement and pulmonary vascular disease, including aortic valve prolapse, double-chambered right ventricle, and subaortic membrane. Due to these risks, all unrepaired nonmuscular VSDs should continue to be followed by a cardiologist every 1-2 years with echocardiographic imaging.

Initiation of medical therapy with a combination of diuretics, afterload reduction, nasogastric feeding, and increased caloric density is indicated for all patients with symptoms regardless of the type of VSD. Referral for closure should occur if medical therapy does not relieve symptoms and resolve left heart enlargement. Large defects should undergo closure regardless of symptoms due to the long-term risk of pulmonary vascular disease.

Although repaired VSDs have resolved hemodynamic alterations, patients with these continue to have higher morbidity and mortality than the general population.^6,7 Initial follow-up is typically based on institutional or device manufacturer protocols. Patients, especially with initially large shunts, may have reduced left ventricular systolic function immediately after closure.⁸ For patients who are >1 year out from intervention with no lesion or only a small residual lesion, follow-up can be reduced to every 2-5 years and testing with an electrocardiogram and echocardiogram do not need to be performed at each visit. Moderate residual lesions should be followed more closely and may need to be referred for reintervention.

(For management of unrepaired VSDs in patients >18 years of age, refer to the 2025 ACC/AHA/Multisociety Guideline for the Management of Adults With CHD.⁹)

Methods: Algorithm Development

The muscular and nonmuscular VSD clinical practice algorithm was developed 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 manner similar to the secundum ASD algorithm using the existing guidelines and the 2026 ACC concise clinical guidance on Outpatient Management of Isolated Left-To-Right Shunt Lesions in Pediatric Patients.^10-12

Future Directions

The muscular and nonmuscular VSDs clinical practice algorithm can be integrated by clinicians as a decision-support tool in their practices. Furthermore, centers can use them for quality-improvement initiatives. The widespread use and implementation of the VSD algorithm could improve resource utilization and standardize the care of patients with isolated VSDs.

References

1. Minette MS, Sahn DJ. Ventricular septal defects. Circulation. 2006;114(20):2190-2197. doi:10.1161/CIRCULATIONAHA.106.618124
2. Hokanson JS, Ring K, Zhang X. A survey of pediatric cardiologists regarding non-emergent echocardiographic findings in asymptomatic newborns. Pediatr Cardiol. 2022;43(4):837-843. doi:10.1007/s00246-021-02795-8
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 Pediatr. 2014;14:282. Published 2014 Nov 4. doi:10.1186/1471-2431-14-282
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(6):1545-1548. doi:10.1016/0735-1097(95)00358-4
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(4):613-617. doi:10.1016/j.amjcard.2019.05.022
6. Sachdeva R, Parthiban A, Birnbaum B, et al. Clinical Practice Algorithm For the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects. 2026. 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 08/2026.
7. Writing Committee, Sachdeva R, Parthiban A, et al. Outpatient management of isolated left-to-right shunt lesions in pediatric patients: 2026 ACC concise clinical guidance: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. Published online March 26, 2026. doi:10.1016/j.jacc.2025.11.020
8. 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(6):657-703. doi:10.1016/j.jacc.2019.10.002
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11. Faraji E, Bolin EH, Bond EG, Thomas Collins R 2nd, Greiten L, Daily JA. Left ventricular dysfunction following repair of ventricular septal defects in infants. Pediatr Cardiol. 2025;46(2):296-304. doi:10.1007/s00246-023-03391-8
12. Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2026;87(7):822-976. doi:10.1016/j.jacc.2025.09.006