Management of isolated ventricular septal defects (VSDs) in infants and children

INTRODUCTION — Ventricular septal defects (VSDs) are among the most common congenital heart lesions. VSDs occur in isolation or in combination with other congenital heart disease defects, as in an atrioventricular canal, tetralogy of Fallot, and, occasionally, D-transposition of the great arteries. (See "Clinical manifestations and diagnosis of atrioventricular (AV) canal defects" and "Tetralogy of Fallot (TOF): Pathophysiology, clinical features, and diagnosis" and "D-transposition of the great arteries (D-TGA): Anatomy, clinical features, and diagnosis".)

The management of infants and children with isolated VSDs will be discussed here. The anatomy, pathophysiology, natural history, clinical features, and evaluation of isolated VSDs are discussed separately. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis" and "Echocardiographic evaluation of ventricular septal defects".)

The management of other complex congenital heart lesions that include VSD is discussed separately:

●AV canal defects (see "Management and outcome of atrioventricular (AV) canal defects")

●TOF (see "Tetralogy of Fallot (TOF): Management and outcome")

●D-TGA (see "D-transposition of the great arteries (D-TGA): Management and outcome")

OVERVIEW — The management approach for VSD is dependent on the presence and severity of symptoms, the likelihood of spontaneous closure, and the risk of long-term complications that could be prevented by early intervention. These factors are primarily dictated by the size of the defect and magnitude of left-to-right shunting (algorithm 1). The clinical examination and echocardiography (which also confirms the diagnosis) provides information on the size of the defect and degree of shunting. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Clinical features' and "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Diagnosis'.)

Management options include:

●No intervention – For patients with small defects, intervention is usually not required. These patients are typically asymptomatic and have a reasonable expectation of spontaneous closure or decrease in the size of the defect over time. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Small ventricular septal defect'.)

●Medical therapy – Medical therapy is required for patients with symptoms of heart failure. For patients with moderate defects, medical management may adequately address the needs of the patients. For those with more severe symptoms, surgical correction is usually necessary and medical management is aimed at reducing symptoms in the interim. (See 'Medical management of heart failure' below.)

●Closure of the defect – For patients who are not adequately managed by medical therapy, and in those at risk for long-term significant sequelae (eg, pulmonary hypertension or valvar damage) surgical repair is generally warranted. (See 'Indications' below.)

NEONATES — The degree of shunting during the first weeks of life varies with the transition from intrauterine to extrauterine life due to changes in pulmonary and systemic vascular resistance that normally occur during this time period. As a result, ongoing monitoring in the neonatal period is important to determine which infants will remain asymptomatic and require no intervention versus those who develop heart failure and require intervention. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Post-delivery'.)

In our practice, once the diagnosis of VSD is established in the neonate, the next appointment is scheduled at three to four weeks of after birth, provided the infant is well. The visit is timed to coincide with the anticipated onset of symptoms of heart failure in at-risk patients with moderate to large VSDs. In addition, parents and primary care providers are asked to monitor the patient for signs or symptoms of failure, which should prompt an earlier visit with the pediatric cardiologist. (See 'Health care maintenance' below and "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)

SMALL VENTRICULAR SEPTAL DEFECT — Patients with small VSDs are expected to remain asymptomatic and have a reasonable likelihood of spontaneous closure. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Small ventricular septal defect'.)

Our approach to managing infants with small VSDs is as follows:

●Patients should undergo follow-up assessment at three to four weeks of age by a pediatric cardiologist to detect any signs or symptoms of increased left ventricular volume overload. The exception is trivial muscular VSDs. If initial imaging and Doppler demonstrate a very small defect, follow-up is not generally necessary until three to six months of age, by which time many will have closed.

●For those patients who remain asymptomatic, a follow-up evaluation is scheduled with the cardiologist at around six months of age.

●Between visits with the cardiologist, routine care is provided by the primary care provider. If the patient becomes symptomatic (eg, poor weight gain, tachypnea), the patient should be promptly referred to the specialist for cardiac evaluation. (See 'Health care maintenance' below and 'Symptomatic patients' below.)

●If at the six-month visit the murmur is gone, repeat echocardiogram is not necessary, unless clinical concerns arise (eg, endocarditis).

●Patients who continue to have a murmur, but are otherwise asymptomatic and growing well at the six-month visit, are seen again by the pediatric cardiologist at approximately 12 months of age.

●If at the 12-month visit with the cardiologist the murmur is gone, repeat echocardiogram is not necessary, unless clinical concerns arise (eg, endocarditis). If there is no evidence of a defect, no additional follow-up is necessary [1]. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Complications'.)

●If the murmur persists at the 12-month cardiology visit and the patient remains asymptomatic and clinically stable, no further intervention is required. Echocardiographic follow-up is typically performed at three years of age for patients with membranous defects. In those with a muscular defect, no echocardiography is required if the patient remains asymptomatic.

●Asymptomatic patients with residual small defects are usually followed every two to five years for overall assessment. Potential complications of residual small defects are rare and may include infective endocarditis, aortic regurgitation, right or left ventricle outflow tract obstruction and shunting from the left ventricle to right atrium. These complications are discussed in greater detail separately. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Complications'.)

●Medical therapy is initiated in any symptomatic patient. However, since heart failure is not expected in association with small VSDs, the development of new symptoms, particularly late, should prompt reassessment of the original diagnosis and evaluation for other causes of the symptoms. (See 'Symptomatic patients' below.)

MODERATE TO LARGE VENTRICULAR SEPTAL DEFECT — Infants with moderate to large VSDs usually become symptomatic within the first months of life as pulmonary vascular resistance (PVR) declines. The primary care provider should monitor the infant during the first weeks of life for manifestations of heart failure (eg, tachypnea, increased work of breathing, poor weight gain or failure to thrive, and diaphoresis particularly with feeding). (See 'Health care maintenance' below and "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)

If an assessment has not been done earlier, the infant is evaluated at three to four weeks of age by a pediatric cardiologist because of the expected decrease in PVR resulting in increased left ventricular (LV) flow. At that visit, the infant is assessed for early signs of heart failure. If symptoms develop, medical therapy is warranted as detailed in the following sections. Infants who remain asymptomatic should have ongoing follow-up and monitoring. (See 'Symptomatic patients' below and 'Asymptomatic patients' below.)

Asymptomatic patients — Regular follow-up should occur throughout the first year of life for all infants with moderate to large VSDs, even if symptoms are lacking. It is important to assess these patients for evidence of pulmonary hypertension. If the murmur is gone but the pulmonic component of second heart sound (S2) is increased in intensity (movie 1), an echocardiogram is obtained to estimate pulmonary artery pressure (PAP). For patients with estimated PAP <50 percent of systemic arterial pressure, the risk of developing pulmonary hypertensive vascular disease (PHVD) is low [1]. Patients with PAP ≥50 percent of systemic arterial pressure are at risk of developing PHVD and they should be referred for surgical closure by six months of age and no later than one year of age. (See 'Management of patients with pulmonary hypertension' below and 'Closure interventions' below.)

Controversy exists regarding the optimal management of asymptomatic patients (no heart failure or failure to thrive) without pulmonary hypertension (ie, PAP <50 percent of systemic arterial pressure), but who have a persistent large left-to-right shunt (ie, ratio of pulmonary to systemic blood flow [Qp:Qs] >2:1) with corresponding LV dilation. Although many centers advocate closure by one year of age for these patients, others allow for more time for resolution of LV dilation beyond the first year of life. In one case series of patients with pressure-restrictive VSD and moderate to severe LV dilation without heart failure or pulmonary hypertension, conservative medical management with observation (mean follow-up of 7.8 years) resulted in spontaneous resolution of LV dilation and avoidance of surgical closure in 29 of 33 patients [2]. However, until larger long-term studies are performed to evaluate this conservative approach, it remains uncertain whether complications may occur in adults with sizeable residual VSDs [3]. Surgical intervention early in life may be necessary to avoid long-term sequelae. If symptoms and/or evidence of pulmonary hypertension develop, the patient should be referred for closure. Otherwise, the decision to close the defect is based largely upon the degree of LV dilation and provider and patient preferences. In most instances, the decision can be made by the end of the first year. (See 'Closure interventions' below.)

For patients who remain asymptomatic after the first year of life without evidence of pulmonary hypertension or LV dilation, follow-up visits are scheduled every one to two years with an echocardiographic evaluation approximately every two years in stable patients. In addition, echocardiography is performed in children >2 years old whose defects have not closed if outflow tract obstruction or aortic regurgitation is suspected [4]. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Complications'.)

Symptomatic patients — For symptomatic patients, medical intervention may postpone and possibly avoid the need for surgical correction. The goals of therapy are to alleviate heart failure symptoms and normalize growth. Management of symptomatic patients includes nutritional support and pharmacologic treatment of heart failure (eg, diuretic therapy). Measures to prevent respiratory tract infections are also an important component of management (eg, influenza immunization and respiratory syncytial virus immunoprophylaxis if eligible). (See 'Health care maintenance' below and "Heart failure in children: Management".)

Nutritional support — Nutritional support may facilitate weight gain in infants with moderate to large VSDs. These infants have increased caloric needs due to an increased metabolic demand and may need a caloric intake that is >150 kcal/kg per day [1]. Infants with heart failure often tire with feeding and their intake may be limited. Increasing the caloric density of feeds can provide the necessary caloric intake in a smaller total daily volume, which the patient is more likely to be able to consume each day. The caloric density is increased by the addition of carbohydrate and/or medium-chain triglyceride preparations to conventional formulas [4]. (See "Poor weight gain in children younger than two years in resource-abundant settings: Management", section on 'Nutritional therapy'.)

One strategy to increase daily caloric intake is to provide more frequent feedings; however, parents may find this challenging. These infants usually take a long period of time to eat and the amount of time required to ensure adequate intake can be substantial. The use of nasogastric feedings may be necessary to improve caloric intake. This may include bolus or continuous feeds, which can be given at night or during the day. The use of these measures usually indicates that closure of the VSD will be required [5].

Fluid restriction is generally not helpful in the management of infants with heart failure related to VSDs because it tends to result in inadequate caloric intake. Although fluid restriction is often used in the management of adults and older children with heart failure, it is counterproductive in infants because they are totally dependent on a liquid diet. Diuretic therapy rather than fluid restriction should be used to reduce and prevent volume overload as discussed in the following section.

Infants with iron deficiency anemia should be treated with supplemental iron to increase the hematocrit and oxygen-carrying capacity. (See "Iron deficiency in infants and children <12 years: Treatment".)

Medical management of heart failure — The medical management varies depending on the severity of heart failure symptoms. Diuretics are the mainstay of therapy. Though angiotensin-converting enzyme inhibitors have been used in the past, their effectiveness appears to be minimal and they are no longer routinely used. In severe cases, intravenous (IV) inotropic agents may be used (eg, milrinone, dopamine) as a temporizing measure. We typically do not prescribe oral digoxin given the potential for adverse effects with this drug and because it is debatable whether it is beneficial in patients with ventricular volume overload and normal underlying ventricular function [6-8]. (See "Heart failure in children: Management".)

In our practice, decisions regarding medical therapy are based on growth and the degree of heart failure:

●Mild congestive failure (mild tachypnea or diaphoresis while feeding and adequate growth) – Initial diuretic therapy with furosemide (1 mg/kg per dose given orally two to three times a day) is usually sufficient for these patients. Surgery is considered by the end of the first year of life for patients in whom the need for medication persists because the defect has not adequately narrowed or resolved. (See 'Closure interventions' below.)

●Moderate heart failure (tachypnea or diaphoresis with feeding and signs of growth failure) – More aggressive diuretic therapy is typically prescribed in this setting, using higher doses of furosemide (2 mg/kg per dose given orally two to three times per day) and the possible addition of spironolactone for potassium sparring effects (1 mg/kg/dose given one to two times per day). Daily caloric intake is maximized to 150 kcal/kg. After achieving maximal therapeutic targets, patients who show no or little improvement in symptoms are referred for surgical correction. (See 'Closure interventions' below.)

●Severe heart failure (respiratory distress [tachypnea, grunting, retractions or diaphoresis] at rest and failure to thrive) – Patients with severe heart failure typically require early surgical repair (often by three months of age). Initial medical supportive measures should be initiated prior to surgical intervention to stabilize the infant's condition. In most cases, patients with severe heart failure require inpatient management. Interventions may include:

•IV diuretic therapy

•Caloric supplementation (goal caloric intake is 150 kcal/kg per day), typically via nasogastric feeding (see 'Nutritional support' above)

•Alleviation of temperature stress (see "Fever in infants and children: Pathophysiology and management")

•Antibiotic therapy if bacterial pneumonia is suspected (after obtaining blood and possibly tracheal cultures) (see "Pneumonia in children: Inpatient treatment")

•Red blood cell transfusion for clinically significant anemia (see "Red blood cell transfusion in infants and children: Indications")

•Administration of inotropic agents if warranted (eg, dopamine, milrinone) (see "Heart failure in children: Management", section on 'Inotropes')

•Supplemental oxygen as indicated (oxygen should be used with caution since it is a pulmonary vasodilator and may decrease pulmonary vascular resistance [PVR], increasing left-to-right shunt and exacerbating failure)

In some cases (eg, if heart failure symptoms were triggered by an acute respiratory tract infection from which the infant subsequently recovers), patients may improve considerably with medical management and surgery can be deferred. However, most infants with moderate to large VSDs who develop severe heart failure do not to respond adequately to medical therapy and are referred for early surgical closure. (See 'Closure interventions' below.)

Follow-up — For patients who respond to medical therapy (including satisfactory growth), regular follow-up should occur throughout the first year of life. The frequency of follow-up visits is dictated by the severity of symptoms. Patients are monitored for dose adjustment of the medical regimen and to detect any evidence of pulmonary hypertension that may indicate a need for surgical repair. (See 'Management of patients with pulmonary hypertension' below.)

For patients who have resolution of heart failure symptoms (including normal growth) and are successfully weaned off of heart failure therapies, ongoing monitoring is performed as previously described. (See 'Asymptomatic patients' above.)

For infants who continue to require medical therapy, the timing and need for surgical closure is determined by symptoms of heart failure and the estimated PAP. Symptomatic infants with normal PAP, but with a large left-to-right shunt, should have surgery by one year of age. If PAP is elevated (ie, >50 percent of systemic arterial pressure), surgical repair is performed in the first six months of life. Earlier repair may be warranted in infants with Down syndrome because they have an increased risk of developing irreversible pulmonary hypertension. (See 'Closure interventions' below.)

Management of patients with pulmonary hypertension — Infants with large VSDs are at risk for PHVD, which can develop as early as six months of age. Patients with Down syndrome, those with a large degree of left-to-right shunting, and those with elevated PAP have the greatest risk of developing PHVD. By contrast, the risk of PHVD is low in patients with normal PAP.

Evaluation for pulmonary hypertension includes clinical examination (eg, quality of the murmur and intensity of S2 (movie 1)) and echocardiography (eg, regurgitant tricuspid jet flow, septal position, or right-to-left shunting). Though rarely necessary, patients who have echocardiographic evidence of pulmonary hypertension may require further assessment with cardiac catheterization. (See "Pulmonary hypertension in children: Classification, evaluation, and diagnosis", section on 'Echocardiography' and "Echocardiographic evaluation of ventricular septal defects", section on 'Assessment of right ventricular and pulmonary artery pressures'.)

Cardiac catheterization is not often performed in isolated VSD; however, it may be helpful in evaluating the hemodynamic status and PVR in the following settings:

●Infants and children who have large defects but small left-to-right shunts.

●Patients who are referred for initial assessment late in the first year of life or beyond with normal or mildly increased LV size who have a large anatomical VSD (ie, diameter ≥50 percent of the size of the aortic annulus).

Compared with echocardiography, cardiac catheterization provides a more accurate measurement of PAP, provides additional hemodynamic measurements (eg, PVR, Qp:Qs), and allows vasoreactivity testing (ie, measurement of the response to pulmonary vasodilators). Data from the cardiac catheterization (particularly the PVR measurement) may help guide management decisions. (See "Pulmonary hypertension in children: Classification, evaluation, and diagnosis", section on 'Cardiac catheterization'.)

For children with VSDs associated with echocardiographic and/or cardiac catheterization evidence of pulmonary hypertension, surgical considerations based upon the calculated PVR in Wood units (WU) or PVR index (PVRI in WU∙m²) are generally as follows:

●If PVRI is <8 WU∙m², surgical closure is recommended. (See 'Indications' below.)

●If PVRI is >12 WU∙m², the patient is at high risk for persistent pulmonary hypertension and surgical closure of the defect is usually not recommended. Closure of defects with such elevated PVR may result in low cardiac output and increased perioperative mortality. Rarely in these circumstances, there may be consideration for placement of a restrictive VSD patch. (See 'Contraindications' below.)

●For PVRI in the range of 8 to 12 WU∙m², additional information (eg, vasoreactivity testing) is helpful in guiding decisions for surgery and postoperative management.

Patients who have PVR values >8 mmHg/L/min/m² should be evaluated and followed at a center with expertise in treatment of patients with pulmonary hypertension. Medical therapy may be helpful for many of these patients. Management of pulmonary hypertension in pediatric patients is discussed in greater detail separately. (See "Pulmonary hypertension in children: Management and prognosis".)

CLOSURE INTERVENTIONS

Overview — Decisions regarding closure of VSDs and the type of procedure are made on a case-by-case basis. Factors to consider include the severity of heart failure, likelihood of progression of pulmonary hypertensive vascular disease (PHVD) or other complications, likelihood of reduction in size or spontaneous closure of the defect, the morbidity and mortality of the procedure in young infants in the center where the surgery is to be performed, and the likelihood of successful surgical closure. Infants with Down syndrome are at increased risk of developing PHVD and therefore early surgical intervention may be warranted in patients with moderate to large defects.

The goal of VSD closure is to alleviate or prevent heart failure symptoms and to intervene before irreversible PHVD develops, which may occur as early as six months in high risk patients. Patients with moderate to large VSDs who remain symptomatic despite medical therapy should generally be referred for closure intervention by the age of one year. Earlier intervention is warranted in patients with severe symptoms and those at increased risk for PHVD (eg, Down syndrome, elevated pulmonary artery pressure [PAP] on echocardiography). (See 'Indications' below.)

Primary patch surgical closure is the preferred procedure in most cases. Transcatheter closure is generally reserved for patients with defects that are technically challenging to close operatively (eg, remote apical muscular defect, multiple muscular defects ["Swiss cheese" septum]) or for patients unable to undergo cardiopulmonary bypass for various reasons. Transcatheter VSD closure is technically challenging and should be performed in centers with considerable experience and expertise in interventional catheterization techniques and with surgical backup. (See 'Surgical repair' below and 'Transcatheter closure' below.)

Indications — In our practice, VSD closure is generally recommended for patients with any of the following findings [1,4]:

●Persistent symptoms despite maximal medical therapy (including diuretics and nutritional support). (See 'Symptomatic patients' above.)

●Moderate or large defects associated with pulmonary hypertension (ie, PAP >50 percent of systemic arterial pressure). However, VSD closure should not be performed if pulmonary hypertension is severe (ie, suprasystemic PAP). (See 'Management of patients with pulmonary hypertension' above and 'Contraindications' below.)

●Persistent left-to-right shunt with associated left ventricular (LV) dilation (ie, ratio of pulmonary to systemic blood flow [Qp:Qs] >2:1) in the absence of symptoms. The optimal timing for intervening on these patients is not clear and generally center dependent. (See 'Asymptomatic patients' above.)

●Subpulmonic and membranous defects with associated aortic valve prolapse and aortic regurgitation. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Aortic regurgitation'.)

●Double-chambered right ventricle – Infrequently, in association with membranous VSDs, right ventricular (RV) muscle bundles may hypertrophy, leading to subpulmonic obstruction. This condition is known as double-chambered right ventricle since the cavity is divided into two regions of differing pressure. Though there is no specific agreement on timing for intervention, we generally proceed to surgical resection when the higher pressure chamber exceeds one-half of systemic levels [9-14]. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Right ventricular outflow obstruction'.)

Surgery is not typically required for asymptomatic patients with residual shunts in the absence of left ventricular dilation or elevated PAP.

Contraindications — VSD closure is generally contraindicated if PAP is suprasystemic or if pulmonary vascular resistance (PVR) is >12 Wood units (WU). As previously noted, in these patients with severe pulmonary hypertension, closure of the defect may result in low cardiac output and increased perioperative mortality. In these circumstances, there may be consideration for placement of a restrictive VSD patch, but this is a rare circumstance. (See 'Management of patients with pulmonary hypertension' above.)

PAP between 75 and 100 percent of systemic pressure and PVR between 8 and 12 WU∙m² are not absolute contraindications to VSD closure; however this degree of pulmonary hypertension can be associated with increased risk of perioperative complications and decisions regarding closure of the defect should be made on a case-by-case basis. In these patients, closure of the defect may not result in a concomitant decrease in PVR. As a result, these patients may have persistent RV hypertension, possibly suprasystemic with development of RV failure without the presence of a pop-off from the RV to the LV. These patients require judicious postoperative ventilator and vasodilator management. (See "Pulmonary hypertension in children: Management and prognosis".)

Surgical repair — For most children who require a VSD closure intervention, direct patch closure under cardiopulmonary bypass is the procedure of choice. Surgical closure is associated with an excellent outcome with low rates of mortality and reoperation [15-19]. The approach (eg, transatrial, transaortic, right ventriculotomy, apical ventriculotomy) depends upon the type and location of the defect, as well as the preference of the surgeon [4,20]. Ventriculotomy is infrequently used in contemporary practice and should be avoided when possible.

Operative mortality for surgical closure in most centers is <1 percent [15]. Surgical complications are infrequent and include residual shunts, typically around the patch (which usually are trivial and may resolve spontaneously), and in patients with membranous or inlet defects, there is a risk of right bundle branch block and complete heart block [21]. The risks of surgery are increased in children who have elevated PVR. These children must be monitored closely in the postoperative period because PAP is labile. Hypoxia may result in sudden and dramatic changes in resistance with a concomitant decrease in cardiac output.

Transcatheter closure — Successful transcatheter closure for muscular, perimembranous, and residual VSDs following surgical repair has been reported in several series [22-25]. Transcatheter closure of VSD remains technically challenging with a higher complication rate than surgery, though it has gained popularity in some countries [26]. At our institution, transcatheter VSD closure is offered for patients with defects that are technically challenging to close operatively (eg, remote apical muscular defect, multiple muscular defects ["Swiss cheese" septum]) or for patients unable to undergo cardiopulmonary bypass for various reasons. Transcatheter closure of VSD is associated with a higher incidence of atrioventricular block and valve injury as compared with surgery. Transcatheter VSD closure should be performed in centers with considerable experience and expertise in interventional catheterization techniques and with surgical backup [1,4].

Ventricular arrhythmia, heart block, bleeding, and valvular damage are all potential complications of transcatheter closure [23,24]. In general, patients require careful monitoring during follow-up to detect any post-procedure complications (eg, complete heart block) that may require further intervention. Closure of perimembranous defects is particularly associated with complete heart block [27]. A presumed mechanism is that the RV retention disk overlaps the ventricular conduction system as it passes above or anterosuperiorly to the defect. Affected patients may require a permanent pacemaker post-procedure [24].

HEALTH CARE MAINTENANCE — Important aspects of long-term health care maintenance in children with VSDs, particularly those who have not undergone repair, include:

Immunizations — Children with VSDs should receive all routine childhood vaccinations, including pneumococcal vaccine, yearly influenza vaccine, COVID-19 vaccine, and respiratory syncytial virus (RSV) immunoprophylaxis for eligible infants. (See "Standard immunizations for children and adolescents: Overview" and "Pneumococcal vaccination in children" and "Seasonal influenza in children: Prevention with vaccines" and "COVID-19: Vaccines", section on 'Children' and "Respiratory syncytial virus infection: Prevention in infants and children".)

Monitoring of growth parameters — It is important to monitor growth and development in children with VSDs, as it is in all children. Feeding difficulties and poor weight gain are often the earliest clinical signs of heart failure in young infants and children. Infants should be followed every two weeks initially to assess growth parameters. If nutritional needs are not met, the infant will initially fail to gain weight appropriately and nutritional support may be warranted. (See "Normal growth patterns in infants and prepubertal children" and 'Nutritional support' above.)

Monitoring for symptoms of heart failure — Between visits with the cardiac specialist, the primary care provider should monitor for symptoms related to heart failure. If the patient becomes symptomatic (eg, poor weight gain, tachypnea), the patient should be promptly referred to the specialist for cardiac evaluation. (See "Heart failure in children: Etiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)

Exercise and sports participation — The 2015 scientific statement of the American Heart Association and American College of Cardiology (AHA/ACC) provides competitive athletic participation guidelines for patients with congenital heart disease (CHD), including VSD [28]. They utilize the classification of sports based on increasing static and dynamic components (figure 1). These recommendations are in place to reduce and prevent sudden death; however, it should be noted that VSDs are not associated with sudden death in the absence of significant pulmonary hypertension. As with any guidelines, recommendations need to be tailored to the patient and a comprehensive evaluation by an experienced clinician is required.

Our practice regarding competitive sports participation in patients with VSDs is generally consistent with the AHA/ACC guidelines:

●Patients with untreated small or restrictive VSDs can participate in all sports if they have no pulmonary hypertension.

●Patients with unrepaired large, hemodynamically significant VSDs and pulmonary hypertension may participate in only low-intensity (class IA) sports (figure 1).

●Patients who have undergone VSD repair (surgical or catheter-based) may participate in all sports two to three months after repair if they are asymptomatic with no or a small residual defect and no evidence of pulmonary hypertension, tachyarrhythmia, or ventricular dysfunction.

●Patients with persistent mild to moderate pulmonary hypertension or moderate ventricular dysfunction following VSD repair may participate in only low-intensity (class IA) sports.

●Patients with symptomatic tachyarrhythmias or second- or third-degree atrioventricular block following VSD repair should not participate in competitive sports until further evaluation by an electrophysiologist.

Physical activity and sports participation in patients with CHD are discussed in detail separately. (See "Physical activity and exercise in patients with congenital heart disease".)

Antibiotic prophylaxis — Our practice regarding endocarditis prophylaxis is consistent with the guidelines of the AHA, which recommend no antibiotic prophylaxis in children with an isolated VSD, except in the following circumstances:

●In children with repaired VSD, which required the use of prosthetic material or device, prophylactic antibiotics are recommended for dental and respiratory tract procedures during the first six months after the repair.

●In children with repaired VSD with a residual defect at the site or adjacent to the site of a prosthetic device, prophylactic antibiotics are recommended for dental and respiratory tract procedures.

Antimicrobial prophylaxis for bacterial endocarditis is discussed in greater detail separately. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

OUTCOME — The long-term outcome for children with VSDs is generally excellent. In a report of the United Kingdom Northern Congenital Abnormality Survey, the estimated 20-year survival rate of children with VSDs born between 1985 and 2003 was 98.3 percent [29].

In a national registry study that included data on 3495 children with isolated VSD born between 1994 and 2009, surgical or transcatheter closure was performed in only 5 percent of cases [30]. There was no operative mortality, and no excess mortality in children with isolated VSDs compared with children without VSDs (overall mortality was 0.3 percent; adjusted hazard ratio [HR] 0.8 [95% CI 0.5 to 1.4]). Long-term cardiac complications observed in this cohort were rare, including arrhythmias (0.5 percent), aortic regurgitation (0.3 percent), endocarditis (0.1 percent), and pulmonary hypertension (<0.1 percent).

●Outcome following surgical repair – Operative mortality for surgical VSD repair in most centers is <1 percent [15]. Early surgical repair results in near-normal long-term growth in the vast majority of patients, and most survivors remain asymptomatic and lead normal lives [16-19,31-34]. In a report from a single center of 215 patients with isolated VSD who underwent surgical repair at a median age of 10 months from 2000 to 2006, there were three deaths over a mean follow-up of 2.1 years (one death occurred during surgery) [35]. Of the 212 survivors, 211 were asymptomatic from a cardiac standpoint and required no antiarrhythmic agents or pacing. (See 'Surgical repair' above.)

●Outcome following transcatheter closure – Procedure-related mortality with transcatheter VSD closure is rare (0.2 percent in one large registry study) [36]. The procedure is associated with higher complication rate than is surgery, particularly complete heart block, which occurs in 2 to 6 percent of patients following transcatheter VSD closure [24,36,37]. Data are limited on long-term outcomes following transcatheter VSD closure because the procedure is still relatively new and it is performed far less often than surgical VSD repair. In addition, many published case series include both pediatric and adult patients (including patients with post-myocardial infarction VSD) and therefore do not accurately reflect the outcomes of young patients with isolated congenital VSD. (See 'Transcatheter closure' above.)

●Outcomes for children managed without VSD closure – Many children with hemodynamically insignificant VSDs remain asymptomatic and therefore do not undergo closure of the defect. For the vast majority of these patients, the VSD closes spontaneously and/or there are no long-term consequences [38]. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Natural history'.)

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Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword[s] of interest.)

●Basics topic (see "Patient education: Ventricular septal defects in children (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Newborn follow-up – Patients diagnosed as newborns with moderate to large ventricular septal defects (VSDs) need to be closely followed in the first weeks of life, as this is the time period when onset of heart failure is most likely. In our practice, patients who are well appearing are scheduled for follow-up with a pediatric cardiologist after delivery at three to four weeks of age. In the interim, patients are seen by their primary care provider who monitors growth, changes in the cardiac examination, and the development of signs related to heart failure (eg, tachypnea, increased work of breathing, poor weight gain or failure to thrive, and diaphoresis particularly with feeding). Any of these signs or symptoms should prompt an earlier appointment with the pediatric cardiologist. (See 'Neonates' above and "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Clinical features'.)

●Management approach – The management of VSDs is primarily dependent on the size of the defect, degree of shunting, and the likelihood of spontaneous closure. Our general approach is as follows (algorithm 1):

•Asymptomatic patients with small VSDs – For asymptomatic patients who typically have a small defect, we suggest no intervention (Grade 2C). There is a reasonable expectation of spontaneous closure in such patients. (See 'Small ventricular septal defect' above.)

•Medical therapy for symptomatic patients with moderate to large VSDs – Symptoms typically occur in patients with moderate to large defects. Medical therapy for these patients focuses on reducing the symptoms and complications of heart failure, and includes the following (see 'Symptomatic patients' above and "Heart failure in children: Management"):

-Nutritional support to ensure normal growth by increasing caloric intake (see 'Nutritional support' above)

-Diuretic therapy to reduce volume overload (see 'Medical management of heart failure' above and "Heart failure in children: Management", section on 'Diuretics')

-Immunoprophylaxis to reduce the risk of respiratory syncytial virus, influenza, and other infections (see 'Health care maintenance' above)

•Follow-up during first year – Regular follow-up should occur throughout the first year of life. The frequency of follow-up visits is dictated by the severity of symptoms. Goals of follow-up monitoring are (see 'Follow-up' above):

-To ensure adequate response to medical therapy (including satisfactory growth)

-To detect any evidence of pulmonary hypertension that may indicate a need for surgical repair

-To adjust the medical regimen as the patient grows

-To watch for evidence of spontaneous VSD closure, which would allow for discontinuation of medical therapy

•Indications for VSD closure – We suggest VSD closure for patients with moderate or large defects associated with any of the following (Grade 2C) (see 'Closure interventions' above and 'Indications' above):

-Persistent symptoms of heart failure despite maximal medical intervention

-Pulmonary hypertension (ie, pulmonary artery pressure [PAP] between 50 to 100 percent of systemic arterial pressure)

-Persistent large left-to-right shunt (ie, ratio of pulmonary to systemic blood flow [Qp:Qs] >2:1) with corresponding left ventricular dilation

-Development of aortic valve prolapse and aortic regurgitation, which can occur as a complication of subpulmonic and membranous VSDs

-Development of double-chambered right ventricle, which can infrequently occur as a complication of with membranous VSDs

VSD closure is not typically required for asymptomatic patients with only mild to moderate left-to-right shunting and normal PAP.

•Contraindications to VSD closure – VSD closure should not be performed in patients with severe pulmonary hypertension (ie, suprasystemic PAP) because closure of the defect may result in low cardiac output and increased perioperative mortality. (See 'Contraindications' above.)

●Surgical versus transcatheter closure – Most patients who require VSD closure undergo primary patch surgical closure, which is associated with excellent outcomes (ie, low surgical mortality, low complication rates, and low reoperation rates). Transcatheter closure is generally reserved for patients with defects that are not amenable to surgical repair (eg, multiple muscular defects that may be difficult to visualize at the time of surgery). Transcatheter VSD closure is technically challenging and should be performed only in centers with considerable experience and expertise in interventional catheterization techniques and with surgical backup. (See 'Surgical repair' above and 'Transcatheter closure' above.)

●Long-term management – Important aspects of long-term health care maintenance in children with VSDs, particularly those who have not undergone repair, include administering routine childhood vaccinations, monitoring growth parameters, monitoring for symptoms of heart failure, and providing guidance regarding exercise and sports participation. Antibiotic prophylaxis for bacterial endocarditis is not necessary for children with VSDs except during the first six months following repair with prosthetic material or device and/or following repair if there is a residual defect at or adjacent to the site of a prosthetic device. (See 'Health care maintenance' above and "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Thomas Graham Jr, MD, and Kirsten Dummer, MD, who contributed to an earlier version of this topic review.