Version May 2024
INTRODUCTION — Ventricular septal defect (VSD) is one of the most common congenital heart defects (second only to bicuspid aortic valve), but accounts for only 10 percent of congenital heart defects in adults because many close spontaneously [1,2]. VSDs are of various sizes and locations and can be single or multiple. VSDs can occur as isolated defects or they can be part of complex congenital heart defects. Depending on their size, their physiology, and complications (eg, pulmonary hypertension, aortic or tricuspid valve regurgitation), clinical presentation can be variable and management may be challenging. VSDs as part of complex congenital heart defects are discussed within the pertinent topics. (See "D-transposition of the great arteries (D-TGA): Anatomy, clinical features, and diagnosis" and "Tetralogy of Fallot (TOF): Pathophysiology, clinical features, and diagnosis".)
This topic focuses on prognosis and management of isolated congenital VSDs in adults. The clinical manifestations and diagnosis of VSDs are presented separately. (See "Clinical manifestations and diagnosis of ventricular septal defect in adults".)
VSDs can also occur as an acquired condition, such as a complication after acute myocardial infarction, surgical aortic valve replacement [3,4], transcatheter aortic valve implantation, septal myectomy for hypertrophic cardiomyopathy, from erosion of a strut of a bioprosthetic mitral valve [5], or from stress (Takotsubo) cardiomyopathy [4]. These conditions differ in many aspects from congenital VSDs and are discussed separately. (See "Acute myocardial infarction: Mechanical complications", section on 'Rupture of the interventricular septum' and "Transcatheter aortic valve implantation: Complications", section on 'Ventricular perforation' and "Hypertrophic cardiomyopathy: Management of patients with outflow tract obstruction", section on 'Septal reduction therapy'.)
VSD CLASSIFICATION
Anatomic types — VSDs are classified into five anatomic types as discussed further separately (figure 1) (see "Clinical manifestations and diagnosis of ventricular septal defect in adults", section on 'Anatomic types'):
●Infundibular (outlet) VSDs (type 1, also referred to as supracristal, subarterial, subpulmonary, conal, or doubly-committed VSD) result from deficiency in the septum above and anterior to the crista supraventricularis, beneath the aortic and pulmonary valves (figure 2 and image 1 and movie 1 and movie 2). The resultant loss of support of the right (less commonly the left) aortic valve cusp causes cusp prolapse into the VSD, leading to aortic regurgitation that could be progressive [6], and occasionally aortic sinus dilatation [7,8].
●Membranous VSDs (type 2, also known as conoventricular) result from deficiency of the membranous septum and is the most common type of VSD (figure 2 and image 2 and movie 3). The defect may extend into the muscular septum and is then referred to as a perimembranous (or paramembranous) VSD.
●Inlet defects (type 3, also known as atrioventricular [AV] canal type) result from deficiency of the inlet septum located beneath both mitral and tricuspid valves (image 3). (See "Clinical manifestations and diagnosis of atrioventricular (AV) canal defects".)
●Muscular defects (type 4) are bordered only by muscle within the trabecular septum.
●Gerbode defect – The least common native VSD is the AV VSD or Gerbode defect, caused by deficiency of the membranous septum separating the left ventricle (LV) from the right atrium, resulting in an LV-to-right atrial shunt.
Spectrum of clinical presentation — The spectrum of isolated residual VSDs in adults includes the following clinical and hemodynamic types, which are discussed further separately [9]. In patients with VSDs, the magnitude and direction of shunting are dependent on the size of the defect and the ratio between systemic and pulmonary vascular resistances. (See "Clinical manifestations and diagnosis of ventricular septal defect in adults", section on 'Spectrum of clinical presentation'.)
●Small restrictive VSDs are associated with small left-to-right shunts (pulmonary to systemic flow ratio [Qp:Qs] <1.5:1). There is typically no LV volume overload or pulmonary hypertension (PH).
●Moderate-sized VSDs (also called moderately restrictive VSDs) typically measure >25 but <75 percent of the aortic annulus diameter and result in mild to moderate volume overload of the pulmonary arteries, left atrium, and LV. Patients with this lesion typically have various degrees of PH.
●Large nonrestrictive VSDs (diameter ≥75 percent of that of the aortic annulus) cause early large left-to-right shunt and are generally closed during the first year of life. If left uncorrected, the persistent shunt can cause progressive pulmonary vascular obstructive disease and PH. The right ventricle (RV) pressure may reach systemic or suprasystemic levels, leading to reversal of the shunt (right-to-left) with resultant hypoxemia and cyanosis; this is known as Eisenmenger syndrome [10]. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis".)
●Some adults with prior VSD closure have a residual VSD; these can be hemodynamically insignificant depending on size and relative PVR and SVR as above. Residual VSD and other post-closure sequelae are discussed below. (See 'Residual VSD' below and 'Postoperative course' below.)
CLINICAL COURSE — The clinical course and prognosis of isolated VSDs depend on the type, size of the defect, and associated hemodynamic abnormalities. VSDs often decrease in size in the first few years of life or close spontaneously. Small persistent VSDs are associated with a lower risk of complications than unclosed or closed larger VSDs, but both are associated with risk of cardiovascular morbidity.
Spontaneous closure — Most small VSDs close during childhood, and large defects tend to become smaller; the rate of spontaneous VSD closure is lower in adults (eg, 10 percent in adults with VSD followed for a mean of 13 years [11]). Spontaneous closure more often occurs in muscular and membranous defects but generally does not occur in inlet or infundibular (outlet) defects. Closure results from muscular growth around the defect, ingrowth of border-forming proliferative fibrous tissue, or, in the case of membranous defects, from the tricuspid valve septal leaflet adhering to the edges of the defect, which may have an aneurysmal appearance.
A VSD that has closed spontaneously is generally associated with an excellent prognosis. These patients no longer face the risks of developing pulmonary hypertension (PH) and endocarditis. However, closure of a membranous defect by apposition of the septal leaflet of the tricuspid valve may cause tricuspid regurgitation [12]. Patients with a spontaneously closed VSD may still be at risk for developing a sinus of Valsalva aneurysm with occasional rupture, double-chambered right ventricle (DCRV), and heart block [13,14]. (See "Isolated ventricular septal defects (VSDs) in infants and children: Anatomy, clinical features, and diagnosis", section on 'Natural history' and 'Complications and their management' below.)
Persistent VSDs — VSDs that persist into adulthood may be small, moderately restrictive, or large.
●Small VSDs – Adults with small persistent VSDs with normal pulmonary artery pressure generally do not require intervention and have an excellent prognosis; however, these patients remain at risk of development of endocarditis, DCRV, or aortic regurgitation (AR), as illustrated by the following series:
•Low risk of requiring closure – In a series of 229 patients with small VSDs (with normal pulmonary artery pressure, less than 50 percent shunt, pulmonary vascular resistance ≤200 dyn-s/cm5, no VSD-related AR, and no symptoms), the reported rate of requiring closure was less than 1 percent during mean seven-year follow-up (at mean age at last follow-up of 30 years) [15].
•Risk of complications – A series of 231 patients with isolated small VSDs were followed to a mean age of 34 years [16]. Most patients (97 percent) were in New York Heart Association (NYHA) functional class I, and no deaths were reported. However, during follow-up, 11 percent underwent surgery for complications associated with VSD including DCRV (17 patients), endocarditis (six patients), LV enlargement (two patients), and AR (one patient).
In a series of 188 patients with small, unoperated VSDs, 47 percent had no complications during mean 13-year follow-up (mean age 30 years) [11].
•Endocarditis developed in 11 percent of patients (by mean age 30 years) [11] and 10 percent of patients (by mean age 34 years) [16] in two series.
•Aortic regurgitation – AR developed in 20 percent of patients (by mean age 30 years) [11].
•Arrhythmias, most commonly atrial fibrillation, developed in 8.5 percent (by mean age 30 years) [11].
•Reduced exercise tolerance – A study found that patients with small VSDs (pulmonary to systemic flow ratio [Qp:Qs] <1.5) have reduced functional aerobic capacity when compared with controls [17].
●Medium or large VSDs – Adults with moderate or large VSDs are at risk for development of progressive pulmonary vascular disease, which may lead to irreversible severe PH with shunt reversal and subsequent cyanosis (Eisenmenger complex), HF, and arrhythmias. Other potential complications include AR, DCRV, thromboembolic complication, and endocarditis. (See 'Complications and their management' below.)
In patients with VSD and associated PH, prognosis depends upon the severity of PH. In patients with Eisenmenger complex, survival is limited, as illustrated by a study of 47 patients with this disorder aged 23 to 69 years that found a 70 percent survival rate at mean follow-up of seven years [18]. The prognosis of patients with persistent pulmonary arterial hypertension after VSD closure has been found to be particularly impaired. The prognosis and management of adults with congenital heart disease with PH and Eisenmenger syndrome are discussed in more detail separately. (See 'Pulmonary hypertension' below and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis", section on 'Prognosis'.)
Long-term prognosis — Survival ranges from excellent for patients with small isolated restrictive VSDs with normal ventricular function and normal pulmonary artery pressure to limited for those who develop Eisenmenger complex [15,19,20].
In the Second Natural History Study (NHS-2) of 1252 patients (mostly children) with VSDs (ranging from small to large) who were followed for >15 years with medical and surgical management, the overall estimated 20-year survival rate was 87 percent [19]. The most common reported causes of death in patients with VSD are heart failure (HF) and sudden death, followed by pulmonary embolism, myocardial infarction, and endocarditis.
In a population-based cohort study in Denmark, outcomes in 8006 patients with VSDs were compared with 79,568 matched controls with a median follow-up of 23 years [21]:
●The hazard ratio (HR) of HF was high both in patients with unrepaired VSDs (HR 5.4, 95% CI 4.6-6.3) and in those with surgically closed VSDs (HR 30.5, 95% CI 21.8-42.7) compared with controls. Analysis limited to the period starting one year after VSD diagnosis in patients with unrepaired defects and one year after VSD closure in patients with a surgically closed defect showed reduced but persistent risk of HF in both groups (HR 2.9, 95% CI 2.3-3.6 and HR 7.0, 95% CI 4.2-23.2).
●Similarly, the risks of arrhythmia and pulmonary hypertension were elevated in patients with unrepaired or closed VSDs.
●The risk of infectious endocarditis was markedly elevated in patients with VSDs that were not repaired (HR 28.0, 95% CI 19.2-40.9) and in patients with VSDs that were eventually closed (HR 82.7, 95% CI 37.3-183.2). The risk of endocarditis was lower following VSD closure (HR 2.4, 95% CI 0.3-20.6).
●For patients with unrepaired VSDs, the risks of arrhythmia, infectious endocarditis, HF, and pulmonary arterial hypertension accelerated after age 40. For patients with closed VSDs, the risks of complications accelerated earlier, particularly the risks of HF and pulmonary arterial hypertension.
Complications in patients with unrepaired and repaired VSDs are discussed below. (See 'Complications and their management' below and 'Postoperative course' below.)
GENERAL MANAGEMENT
Approach to management — The management of patients with VSD depends on the type of defect, its size, the resulting magnitude of shunting, and associated acquired complications including double-chambered right ventricle (DCRV), aortic regurgitation (AR), and pulmonary hypertension (PH).
●General management of patients with VSD (unrepaired or repaired) includes periodic, individualized follow-up by an adult congenital heart disease (CHD) specialist to assess for symptoms or potential complications. The frequency of follow-up is based upon the patient's functional status (New York Heart Association [NYHA] functional class) (table 1) as well as risk of complications [9]. (See 'Complications and their management' below.)
•Small unrepaired VSDs – Most patients with small, asymptomatic, uncomplicated VSDs require no intervention, but are at risk of development of double-chambered right ventricle (DCRV) or aortic regurgitation (AR), and periodic follow-up by a congenital heart disease (CHD) cardiologist is recommended. The risk of complications such as arrhythmias, HF, pulmonary arterial hypertension, and endocarditis may rise after age 40 years. (See 'Clinical course' above.)
•Repaired or unrepaired VSDs with complications – Adults with previously repaired VSDs who have residual shunts and patients with unrepaired or repaired VSDs who have HF, AR, pulmonary hypertension, or other hemodynamic abnormalities should be seen at least annually at an adult CHD center.
●Indications for VSD closure – Patients with an indication for VSD closure should be referred to an adult CHD expert for confirmation and to guide the choice of intervention (surgical or percutaneous repair). (See 'Indications for VSD closure' below.)
●Measures to avoid or treat complications (including PH) – PH management options in patients with VSD include VSD closure in selected patients without irreversible severe PH (see 'Indications for VSD closure' below). Selected patients with VSD and PH are candidates for advanced medical therapy for pulmonary arterial hypertension. (See 'Pulmonary hypertension' below.)
●Other surgical procedures (such as aortic valve surgery for AR or surgery for DCRV) may be performed at the time of VSD closure or subsequently, depending upon clinical status and severity of complications requiring surgery. (See 'Aortic regurgitation' below and 'Double-chambered right ventricle' below.)
VSD closure
Indications for VSD closure — The following indications for VSD closure are similar to those included in the 2018 American Heart Association/American College of Cardiology (AHA/ACC) adult CHD guidelines (algorithm 1) [9]. Similar recommendations are included in the European Society of Cardiology (ESC) and the Canadian Cardiovascular Society (CCS) guidelines [22,23]. Patients with an indication for VSD closure should be referred to an adult CHD expert for confirmation and to guide the choice of intervention (surgical or percutaneous repair).
●Hemodynamically significant shunt – The rationale for VSD closure in patients with a hemodynamically significant shunt with acceptable pulmonary artery pressures is to avoid complications observed in natural history studies, including progressive PH and HF [19,24]. Observational series suggest that surgical closure in patients with significant shunts reduces pulmonary artery pressure and improves long-term survival [20,25]. Therefore, VSD repair is warranted in adult patients who are symptomatic or have signs of LV volume overload without irreversible pulmonary vascular disease. (See 'Clinical course' above.)
•Without significant PH – For adults with VSD with a hemodynamically significant net left-to-right shunt without significant PH, we recommend VSD closure. We define a hemodynamically significant shunt as a pulmonary to systemic flow ratio [Qp:Qs] ≥1.5:1 with LV dilation. In this context, we define lack of significant PH as pulmonary artery systolic pressure <50 percent of systemic arterial pressure, and pulmonary vascular resistance less than one-third of systemic vascular resistance. This recommendation is based upon natural history data suggesting that patients with a hemodynamically significant shunt are at risk for the development of PH and data showing excellent outcomes in patients without PH undergoing VSD repair. (See 'Pulmonary hypertension' below and 'Choice of intervention' below.)
•With significant PH – For adults with VSD with a hemodynamically significant net left-to-right shunt (as defined above), and pulmonary artery pressure ≥50 percent but less than two-thirds of systemic pressure, or pulmonary vascular resistance greater than or equal to one-third but less than two-thirds of systemic vascular resistance, management is based upon an individualized assessment of the benefits and risks of VSD closure by adult CHD and PH experts. It is important to note that persistent or progressive pulmonary arterial hypertension after shunt closure bears a particularly grim prognosis [26] (See 'Pulmonary hypertension' below and 'Choice of intervention' below.)
●Significant or worsening AR – For adults with a perimembranous or infundibular (outlet) VSD with significant or worsening AR who do not have other indications for VSD closure, we suggest surgical closure of the VSD. The aortic valve is commonly repaired at the time of VSD closure. Surgical aortic valve replacement is reserved for patients with AR with an indication for aortic valve surgery when the aortic valve cannot be durably repaired. (See 'Aortic regurgitation' below and "Natural history and management of chronic aortic regurgitation in adults", section on 'Indications for aortic valve surgery'.)
Surgical repair of VSD in patients with progressive or more than mild AR should not be delayed in asymptomatic patients until there is significant LV enlargement or dysfunction as is recommended in isolated severe AR (see "Natural history and management of chronic aortic regurgitation in adults", section on 'Indications for aortic valve surgery'). Early intervention, with closure of the VSD and repair of the aortic valve, is based upon the defect type (perimembranous or infundibular), defect size, degree of valve distortion, and AR severity, with better results achieved in younger patients [27,28].
●History of infective endocarditis – For patients with VSD with history of infective endocarditis, we suggest an individualized assessment of the risks and benefits of VSD closure. If there is no contraindication to closure, shared decision making is appropriate. Observational data suggest that surgical closure decreases the risk of endocarditis by at least one-half [29].
VSDs should not be closed in patients with severe irreversible pulmonary artery hypertension. Closure of a nonrestrictive VSD in a patient with Eisemenger syndrome is associated with high risk of mortality and is therefore not performed [9,30-32]. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)
Patients with VSD who do not meet the above criteria for pulmonary artery pressure and pulmonary vascular resistance may become candidates for VSD closure if these criteria are met after advanced therapy for pulmonary arterial hypertension. (See 'Pulmonary hypertension' below.)
Choice of intervention — Repair of VSD has been historically performed surgically. However, percutaneous VSD closure is also feasible for isolated uncomplicated muscular VSDs, and for membranous VSDs, in selected patients with suitable anatomy. The frequency has increased given the desire of young patients to avoid surgery [33,34]. Surgical and percutaneous VSD closure should be performed by surgeons and cardiologists with appropriate training and expertise.
Surgical repair — Primary surgical repair for isolated VSD was originally performed through a ventriculotomy, but with the recognition of long-term risks after ventriculotomy, surgical techniques now allow repair of perimembranous VSD through a right atriotomy. Infundibular VSDs are generally repaired through a transpulmonary approach. Access to the heart is generally through a sternotomy incision [35]. Thoracoscopic techniques with or without a robotically assisted surgical system are used at some centers [36].
Concomitant repair of other cardiac defects is frequently indicated. For example, in one series, 34 of 46 adult patients undergoing VSD repair needed concomitant repair of other defects, most commonly aortic or tricuspid valve repair [12].
●When DCRV is present, the RV outflow obstruction should be surgically resected.
●When aortic valve prolapse and AR are present, aortic valve repair is recommended when possible to reduce the risk of progressive AR. This often includes commissural suspension and plication of the elongated prolapsing cusp. In patients in whom aortic valve repair is not feasible because of extensive distortion, fenestration, fibrosis, or calcification, valve replacement should be considered [37]. A study of 122 adolescents and adults followed with VSD and AR found that 68 (56 percent) required surgical intervention, 49 had aortic valve repair, and 21 had ultimate aortic valve replacement [38].
Intraoperative transesophageal echocardiography (TEE) is recommended to assess VSD anatomy, aortic valve, and adequacy of surgical repair. (See "Intraoperative transesophageal echocardiography for noncardiac surgery".)
Limited data are available on operative outcomes for VSD repair in adults. The risk of operative mortality with surgical VSD closure was substantial in early series [19], but later studies of surgical VSD closure in children showed very low operative mortality rates [39,40]. A report from the Mayo Clinic describing outcomes of surgical VSD repair in 46 adults demonstrated no operative mortality [12]. Improved perioperative outcomes are likely related to improved myocardial preservation, surgical techniques, patient selection, and postoperative care; these techniques have resulted in a decline in operative mortality in isolated, uncomplicated VSD to <2 percent [12].
As noted above, observational data suggest that surgical closure decreases the risk of endocarditis [29]. Observational series also suggest that surgical closure in patients with significant shunts reduces pulmonary artery pressure and improves long-term survival [20,25]
Percutaneous repair — Transcatheter device VSD closure is a treatment option for isolated, uncomplicated, muscular VSDs, and for membranous VSDs in selected patients with suitable anatomy. Appropriate anatomy for transcatheter closure includes a VSD location remote from the tricuspid and aortic valves with an adequate rim of tissue. Successful transcatheter closure has been accomplished in the presence of multiple muscular or membranous fenestrations [33,41].
The technical success rate of transcatheter closure of selected muscular and membranous VSDs is high, and the mortality rate is low [42].
●In experienced hands, device closure of muscular VSDs using an Amplatzer device has a reported success rate of 93 to 100 percent and a mortality rate of 0 to 3 percent [43].
●In a review of percutaneous closure of perimembranous VSD in 133 patients, aged 9 months to 28 years, the success rate was 97 percent using 13 different devices. At one year, 9.6 percent had small residual VSD, and 9.6 percent had trivial AR [44].
Similar complication rates have been reported for percutaneous closure of muscular VSDs (3 to 10 percent [43]) and membranous VSDs (0 to 12 percent [33]). Procedural-related complications include rhythm disturbance, conduction abnormalities, and hypotension [45].
Development of complete AV block is the most significant procedural complication [33,43]. In a series of 104 patients who underwent transcatheter closure of membranous defects, 6 percent developed complete heart block, necessitating pacemaker implantation [33]. The use of ductal occluder devices or coil devices for membranous VSD closure has led to a lower incidence of complete heart block [46-49]. Real-time three-dimensional TEE has been used to guide such procedures [50]. Given the lack of data on long-term outcomes following catheter closure of VSD in adults, patients should be followed every one to two years at an adult CHD center.
A systematic review and meta-analysis included seven observational studies with a total of 3134 patients (1312 who underwent percutaneous device closure and 1822 who underwent surgery) [51]. Procedural success and composite endpoints for safety (early death/reoperation/permanent pacemaker) were similar in the two treatment groups. Rates of advanced heart block, significant aortic or tricuspid regurgitation, and residual shunt were also similar. The need for blood transfusion and duration of hospital stay were significantly reduced in the percutaneous device group.
COMPLICATIONS AND THEIR MANAGEMENT
Aortic regurgitation — Patients with VSD are at risk for development of aortic regurgitation (AR) and require monitoring of valve function. AR occurs 2.5 times more commonly with infundibular type VSD than membranous VSD because the associated deficiency or hypoplasia of the conal septum causes an anatomically unsupported right or left aortic valve cusp and sinus. As a result, the unsupported aortic valve cusp is driven into the RV by a Venturi effect, making the VSD smaller during systole. During diastole, intraaortic pressure causes the aortic valve cusps to close, but the unsupported cusp prolapses down into the LV outflow tract away from the opposing cusps, resulting in typically eccentric AR that may become progressive (figure 3). The prevalence of AR increases with age (occurring in 87 percent of patients by age 20) [8,37]. The progressive nature of the AR, the potential associated morbidity, along with improved surgical results with VSD and aortic valve repair have led to the recommendation to consider early surgical intervention. However, there is a residual risk of AR progression in children with infundibular VSD who have undergone VSD repair (eg, 7.7 percent during mean 13-year follow-up); some (eg, 43 percent) but not all progressive AR was associated with a residual VSD shunt [52].
In adults with infundibular VSD, the risk of progression of AR may be lower than in children, as illustrated by a series of 62 adults (median age 41 years) with infundibular VSD, in which the freedom from AR progression was 86 percent at 15 years [6]. In this series, age ≤40 years was a predictor of AR progression.
For patients with VSD with significant or worsening AR, we suggest VSD closure. Surgical aortic valve repair is performed if feasible to avoid or delay the need for aortic valve replacement. (See 'Indications for VSD closure' above.)
In patients with AR and infundibular VSD who undergo VSD closure without aortic valve surgery (repair or replacement), there is a risk of late postoperative AR progression [52].
Sinus of Valsalva aneurysm — Patients with infundibular VSDs are at risk for development of right sinus of Valsalva aneurysm, which may rupture. The rupture of the aneurysm of the sinus of Valsalva occurs within the right atrium or right ventricle. Patients generally present with acute HF and a continuous murmur. This was illustrated in the above cited study of 62 adults with infundibular VSD in which there was a high risk of sinus of Valsalva aneurysm development (21 percent), even in those patients who had been repaired [6]. In a separate series of 60 adults with infundibular VSD, rupture of the sinus of Valsalva aneurysm was noted in five patients (8 percent), and endocarditis was noted in three patients (5 percent) [53].
Therefore, watchful monitoring is recommended, especially in patients with aortic cusp prolapse. The threshold for elective repair of a dilated aortic sinus related to infundibular VSDs depends on patient and aneurysm characteristics. (See 'Aortic root dilation' below and "Management of thoracic aortic aneurysm in adults".)
Pulmonary hypertension — Moderately-sized and large VSDs are associated with pulmonary hypertension (PH) that may progress to severe irreversible pulmonary vascular disease (Eisenmenger syndrome). (See "Clinical manifestations and diagnosis of ventricular septal defect in adults", section on 'Spectrum of clinical presentation' and "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis".)
PH management options in patients with VSD include VSD closure in selected patients with net left-to-right shunt without irreversible severe PH (see 'Indications for VSD closure' above). Patients with persistent PH after defect closure are a particularly high-risk patient group, requiring close follow-up by a dedicated multi-disciplinary team [26].
Patients with VSD who do not meet pulmonary artery pressure and pulmonary vascular resistance criteria for closure may become candidates for VSD closure on an individualized basis if these criteria are met after treatment with advanced therapy for pulmonary artery pressure. When this strategy is undertaken, targeted therapy is continued after VSD closure. This approach is supported by a study reporting favorable outcomes in 41 adults with nonrestrictive VSD and severe PH treated with PH-targeted therapy for 3 to 15 months followed by surgical VSD closure [54]. Two patients died postoperatively (4.9 percent). PH-targeted therapy was continued in the majority of survivors with documented improvement in pulmonary vascular resistance and RV systolic pressure in the majority of patients. No death was observed on late follow-up at a mean of 37 months. (See 'Indications for VSD closure' above.)
Medical management of patients with VSD and PH, including indications for advanced therapy for pulmonary arterial hypertension, is discussed separately. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)
VSD with Eisenmenger syndrome (known as Eisenmenger complex) is rarely encountered in the current era due to recognition and closure of moderately restrictive or large VSDs early in life. When such VSDs are not closed early in life, patients may develop symptoms of Eisenmenger complex in the second and third decade [14]. Affected patients may present with dyspnea, cyanosis, chest pain, hemoptysis, arrhythmias, syncope, and secondary erythrocytosis. Patients with Eisenmenger complex are at risk for complications of cyanotic heart disease and severe PH, which are discussed in detail separately.
Patients with Eisenmenger complex are managed conservatively as VSD closure is contraindicated. Given the specialized care that patients with Eisenmenger complex require, referral and follow-up at a tertiary care facility with expertise in the care of adult patients with congenital heart disease (CHD) are recommended. Management of adult patients with CHD with PH (PH-CHD) (including those with Eisenmenger complex) is discussed separately. (See "Medical management of cyanotic congenital heart disease in adults" and "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)
Endocarditis
Magnitude of risk — Endocarditis risk persists in those with unrepaired VSD with an incidence of 22 to 24 per 10,000 patient years [15,29]. In a study from Sweden, the incidence of endocarditis in patients with small, unoperated VSD was reported to be approximately 2 per 1000 years [55], more than 20 times the risk in a contemporaneous general population (7.7 per 100,000) [56]. The risk of endocarditis is higher in those with unrepaired VSD compared with those who have undergone repair (relative risk 3.3) [29]. Other risk factors for endocarditis include age >20 years old and male sex. VSD size is not a significant risk factor for development of endocarditis.
Management — Despite the increased risk of infective endocarditis associated with VSD, we agree with the American Heart Association/American College of Cardiology(AHA/ACC) guidelines recommending no antibiotic prophylaxis for patients with acyanotic, uncomplicated VSD with no prior history of infective endocarditis; this is predominantly due to the lack of evidence supporting widespread use of antibiotic prophylaxis and concerns about developing antimicrobial resistance. For patients with a VSD patch leak or prior history of infective endocarditis, antibiotic prophylaxis for prevention of infective endocarditis is recommended. The role of VSD closure in patients with prior history of infective endocarditis is discussed elsewhere. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures" and 'Indications for VSD closure' above.)
Proper patient education about symptoms of infective endocarditis and appropriate evaluation and management if symptoms of endocarditis develop (including the need for timely blood cultures and review by an expert multidisciplinary team) are of paramount importance to avoid delayed diagnosis of infective endocarditis, particularly since most patients with small defects and an uncomplicated clinical course are followed infrequently in specialized clinics. In addition, patients with VSD should receive education on how to minimize endocarditis risk (including careful oral hygiene and appropriate prompt treatment of infections) and the importance of seeking prompt medical attention if symptoms of endocarditis develop.
When endocarditis is suspected, a transthoracic echocardiogram (TTE) is recommended to identify vegetations, which may be related to the VSD or on adjacent valves in the direction of the high velocity jet created by the VSD (often involving the tricuspid valve). Thus, endocarditis in patients with VSD often presents as right heart endocarditis, which may be complicated by septic pulmonary embolism, and this complication may be confused with pneumonia. TEE remains the diagnostic procedure of choice in the evaluation of patients with suspected endocarditis, given its greater sensitivity and specificity compared with TTE. However, TTE provides complementary imaging to TEE in patients with VSD and suspected endocarditis given the propensity to involve right heart structures; thus, both are usually performed. Advanced imaging with computed tomography or positron emission tomography may be beneficial in select patients with VSD and endocarditis, especially those with prosthetic material and in patients with suspected septic embolism. (See "Role of echocardiography in infective endocarditis" and "Clinical manifestations and evaluation of adults with suspected left-sided native valve endocarditis".)
Arrhythmias — Arrhythmias, including atrial and ventricular premature beats and tachycardias, have been observed in adults with unrepaired or repaired VSD [19]. The reported incidences of ventricular tachycardias and sudden death in natural history studies of VSDs are 5.7 and 4.0 percent, respectively. In a series of patients with repaired and unrepaired VSDs, age and pulmonary artery pressure were the best predictors for ventricular arrhythmias on 24-hour ambulatory electrocardiographic monitoring [57]. Arrhythmia may be the presenting sign of VSD in adults, and a comprehensive evaluation is recommended to assess candidacy for repair in all adults with VSD and cardiac arrhythmias.
In patients with VSD complicated by high right ventricular pressure, such as PH, Eisenmenger complex, and double-chambered right ventricle (DCRV), the reported incidence of ventricular tachycardia is 19 percent [19]. The hypertrophied RV in these patients represents an ideal substrate for ventricular arrhythmias. Therefore, some advocate annual Holter monitoring in patients with VSD and elevated RV pressure.
Atrial arrhythmias, mostly atrial fibrillation, are also prevalent in adult VSD, especially with increasing age, LV dysfunction, and elevated pulmonary or right ventricular artery pressure. Treatment of these arrhythmias should be individualized, depending on their associated symptoms, severity, frequency, the presence of conduction delay, or ventricular dysfunction. (See "Atrial fibrillation: Overview and management of new-onset atrial fibrillation".)
Double-chambered right ventricle — DCRV most commonly occurs in association with a small membranous VSD. Progressive hypertrophy of a RV muscle bundles at the infundibular or superior margin of the VSD create a DCRV with a proximal high-pressure RV chamber in the RV inflow and a distal low-pressure RV chamber near the pulmonic valve. This condition develops in 3 to 10 percent of patients with membranous VSD. The resultant RV hypertrophy and right atrial enlargement are associated with ventricular and atrial arrhythmias, respectively. Although patients with VSD and DCRV may remain asymptomatic with severe obstruction, symptoms tend to develop during adult life. In a series of 50 patients with DCRV, symptoms occurred at a median age of 26 years [58]. DCRV is characterized by the development of a loud crescendo-decrescendo systolic murmur at the left sternal border, often associated with a thrill, replacing the typical holosystolic murmur of VSD [59] (see "Clinical manifestations and diagnosis of ventricular septal defect in adults", section on 'Physical signs').
For adults with DCRV with moderate or greater outflow obstruction causing symptoms of HF, exercise limitation, or cyanosis, we recommend surgical repair [9]. For asymptomatic adults with DCRV with severe gradient, we suggest referral to evaluate the benefits and risks of surgical repair [9].
Surgery for DCRV involves transatrial and/or transventricular resection of obstructing muscle bundles and closure of any accompanying VSD [9]. In some patients with this condition, patch enlargement of the RV outflow tract is required to relieve the obstruction. The VSD may be difficult to differentiate from DCRV by echocardiography.
The above recommendations are based upon limited observational data suggesting that surgical repair of DCRV is effective in resolving the hemodynamic lesion. A study of 29 patients with DCRV described complete resolution of RV outflow obstruction after surgical repair at a mean echocardiographic follow-up period of 11 years [60]. RV systolic pressure was nearly normalized immediately after surgery and on late follow-up, with none of the patients exhibiting an RV systolic pressure >50 mmHg postoperatively.
Heart failure — HF due to chronic volume overload of the LV occurs in patients with isolated medium or large VSDs and is rarely seen in adults, since most of these defects are repaired during childhood. However, left-sided HF can develop in the setting of severe AR in association with a residual infundibular or membranous VSD. Right-sided HF may occur in adults with VSD, particularly when there is progressive tricuspid valve regurgitation (due to leaflet adherence to the VSD), RV dysfunction and significant RV hypertension from DCRV, or PH. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis", section on 'Echocardiography'.)
Complications of Gerbode defect — VSD involving the AV septum (Gerbode defect, leading to shunting from LV outflow tract to the right atrium), can be secondary to infective endocarditis and in these patients, periodic assessment includes surveillance for sinus node dysfunction and tricuspid regurgitation [61].
POSTOPERATIVE COURSE — Although the prognosis after surgical repair of uncomplicated VSD is excellent in the majority of patients, lifelong follow-up is important, as long-term residua and sequelae can occur, including conduction disease, cardiac arrhythmias, residual/recurrent VSD, endocarditis, tricuspid or aortic valve regurgitation, ventricular dysfunction, and pulmonary hypertension (PH) [62].
Mortality — Adults with repaired VSD without residua have excellent outcomes and normal survival [13]. In a series of 516 patients who had undergone VSD repair, the 25year survival rate was 83 percent [19]. Another series demonstrated a survival rate of 86 percent at 40 years after operation, with an event-free survival rate of 72 percent [63]. However, long-term survival is less favorable when VSD closure is performed at an older age or in the presence of PH [20,25]. A later series of 46 adults followed at the Mayo Clinic demonstrated survival rates at 5, 10, and 15 years after surgery of 100, 95, and 88 percent, respectively, which did not differ from the expected survival rate derived from a reference population [12].
Surgical repair of VSD and double-chambered RV has been associated with good outcome with no early or late operative mortality, and no need for re-operation in a series of 33 patients during a median follow-up of eight years [58].
Aortic root dilation — Ascending aortic dilation involving the aortic root is common after surgical repair of VSD. Echocardiographic assessment in 152 consecutive patients with surgically repaired VSD revealed aortic root dilatation (>2.1 mm/m2) in 50 patients (33 percent) [64]. In multivariate analysis, the presence of preoperative right or noncoronary cusp prolapse was independently correlated with aortic root dilatation irrespective of VSD type, but the presence of at least moderate AR was not significantly associated with aortic root dilation. Ascending aortic dilation was also noted in 7 of 47 patients (15 percent) in the Belgium Registry on Adult Congenital Heart Disease (CHD) [65]. Although ascending aortic dilation could be explained on the basis of anatomic and surgical alteration, a genetic predisposition may be a factor, particularly given the finding of SMAD3 mutation in some patients with VSD [64,65]. Thresholds for aortic root intervention have not been established for this clinical setting. When marked aortic root dilation is present in a patient with VSD, genetic consultation and testing should be considered to exclude a concomitant condition. (See "Management of thoracic aortic aneurysm in adults".)
Hemodynamic changes — VSD repair promotes normalization of LV mass and function in patients with prior LV volume overload. However, ventricular dysfunction with HF may persist or even worsen after repair, particularly in patients with residual VSD, aortic regurgitation (AR), abnormal septal wall motion, and/or concomitant acquired cardiovascular diseases such as systemic hypertension and ischemic coronary disease. The degree of persistent PH after VSD repair depends on the severity of preoperative PH and the presence of residual VSD. Persistent PH is an indicator of impaired postoperative cardiac performance and is a risk factor for late sequelae.
Although VSD has traditionally been considered a lesion that predominantly affects the LV, a cardiac magnetic resonance imaging study of 27 VSD patients (median age 20 years, repaired in childhood at median age of two years) demonstrated significant changes in RV structure and function when compared with 28 age-matched controls, including larger RV, higher RV mass index, and lower fractional area change but no change in longitudinal strain. This might be reflective of the hemodynamic alterations involving the RV in infancy and childhood or surgical technique [66]. A 17 percent prevalence of RV systolic dysfunction at long-term follow-up post-VSD repair has been reported [67]. A study from the Belgium registry including 47 patients post-VSD repair (age 29 to 40) demonstrated abnormal echocardiographic parameters of RV function, including reduced fractional area contraction (<35 percent) and tricuspid annular plane systolic excursion (<17 mm) in 15 and 36 percent, respectively, on follow-up [65]. RV dysfunction was more common in patients who had patch repair compared with primary repair of VSD. The long-term clinical implications of these findings are yet to be determined. However, this might be an explanation for the reduced exercise capacity and minute ventilation reported in patients following VSD repair [63,67,68].
Arrhythmias and AV block — Ventricular arrhythmias including ventricular premature beats (20 to 34 percent) and less commonly ventricular tachycardia can occur many years after VSD repair [25,57,69]. Their incidence increases with higher preoperative New York Heart Association (NYHA) functional classification, cardiomegaly, older age at time of operation, increased pulmonary artery pressure, prior ventriculotomy, and postoperative conduction disease. Sudden death accounts for as much as 39 percent of all cardiac deaths after surgery [57].
Atrial fibrillation develops in a minority of patients following VSD repair (eg, 10 percent in one post-surgical series [12]). Atrial fibrillation typically occurs with persistent left atrial enlargement due to chronic volume overload, or right atrial enlargement with residual PH. There is also an increased risk of atrial flutter related to surgical scars, similar to other patients with operated congenital cardiac lesions. Thus, even patients with surgical closure of VSD early in life and a subsequently uncomplicated clinical course may be at an increased risk of developing atrial arrhythmias during long-term follow-up. Thus, even patients with no apparent complications should not be discharged from follow-up and should receive periodic follow-up in adult CHD clinics, including education about these long-term risks.
Conduction diseases including right bundle branch block (RBBB; 18 to 100 percent) and left anterior hemiblock (30 percent), especially after ventriculotomy and patch closure of the defect, are common following surgical VSD repair. Late AV node dysfunction, including complete heart block requiring pacemaker placement, was infrequently reported in early series (≤2 percent) [12,25,69]. A study demonstrated impaired heart rate variability in adults following VSD repair in early childhood, particularly those who had complete RBBB, when compared with adults with unrepaired VSD and control groups [70].
Late ventricular arrhythmias could be related to postoperative conduction disease [19]; an association between postoperative conduction defect and late sudden death and ventricular arrhythmia has been observed [71]. Therefore, patients who develop a bifascicular block or transient trifascicular block after VSD repair should be followed annually with clinical assessment, electrocardiogram, and periodic ambulatory monitoring and/or exercise stress testing to assess heart rate response to exercise and to exclude exercise-induced arrhythmias [72].
Residual VSD — Residual VSD was reported in up to one-third of patients in early surgical series. A later Mayo Clinic study of patients undergoing surgical VSD closure during 1958 to 2008 reported postoperative residual VSD in 7 of 46 (15 percent) [12]. A systematic review that included seven studies with a total of 3134 patients who underwent closure in study periods between 2002 and 2012 reported residual shunt in 3 percent with surgical repair, and 2 percent with percutaneous device closure. The reduced frequency of residual VSD during the last two decades may reflect routine intraoperative TEE imaging.
Residual VSDs are often small and hemodynamically insignificant, and reoperation is necessary in only 5 to 6 percent of patients [13]. Periodic followup evaluation is required to monitor for potential need for reoperation [9]. Residual VSD with prosthetic material is an indication for endocarditis prophylaxis. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)
The surgical repair of multiple muscular VSDs using patch exclusion of the involved segment from the RV apex has been associated with good early results in children. However, a report of four adults studied 22 to 45 years after such repair was performed found that the resultant smaller RV cavity was associated with RV diastolic dysfunction, leading to HF and secondary liver cirrhosis [73].
Valve disorders
Postoperative aortic regurgitation — AR is improved in up to 80 percent of patients following surgical VSD closure and aortic valve repair, with better results achieved in younger patients [74]. A 5 percent incidence of significant AR necessitating reoperation was observed in one series of patients who had undergone VSD repair, with a mean interval between the first and second operation of seven years [74]. In a later study, 172 patients underwent repair of VSD and AR using a single-cusp replacement repair technique [75]. At a mean follow-up of 53 months, redo aortic valve surgery was performed in three patients, and moderate to severe AR not necessitating surgery was noted in three patients. Continued clinical and imaging follow-up is therefore recommended irrespective of the surgical technique used.
Tricuspid regurgitation — Tricuspid regurgitation due to septal leaflet traumatic detachment or iatrogenic entrapment during VSD repair has been reported. Tricuspid regurgitation may be aggravated by PH with associated tricuspid annular dilation and should therefore be carefully monitored. In a series of 46 patients, moderate or greater tricuspid regurgitation was observed in 27 percent following VSD repair at a mean follow-up of 10 years [12]. Often, detachment or incision of the tricuspid valve is required for full intraoperative visualization of the VSD through a transatrial approach. Retrospective series suggest that this technique does not seem to negatively impact late tricuspid valve function, but prospective randomized data are lacking [76].
Risk of endocarditis — Endocarditis risk after surgical repair decreases by one-half compared with unoperated VSD patients with a reported incidence of 0.8 to 1.7 per 1000 patient years [15,25,29]. As noted above, in the absence of a prior history of endocarditis or residual VSD with prosthetic material, endocarditis prophylaxis is not recommended after the first six months following VSD repair. (See 'Endocarditis' above.)
MANAGEMENT OF PREGNANCY — Women with VSDs should receive preconception counseling concerning the risks of pregnancy as well as the risk of congenital heart disease (CHD) in offspring. The offspring of women with VSDs have a risk of CHD of 3 to 7 percent [77]. (See "Pregnancy in women with congenital heart disease: General principles".)
The risks of pregnancy in women with a VSD varies according to the functional size of the defect and associated complications such as pulmonary hypertension (PH) and HF:
●Women of reproductive age with small VSDs as well as those with successfully repaired VSDs without significant residua can complete pregnancy with no increase in risk to the mother or fetus. The risk is small when the shunt ratio is <1.7 with normal pulmonary pressure and preserved functional aerobic capacity [78,79].
●Women with larger VSD shunts and those with a history of arrhythmias, ventricular dysfunction, or PH are at higher risk of developing cardiovascular pregnancy-related complications than VSD patients without these features. The most common complications include arrhythmias and HF. High risk is posed by VSD in combination with moderate or greater pulmonic stenosis, PH, double-chambered right ventricle, or LV ejection fraction <40 percent. Management of pregnancy in patients with PH and CHD or with other risk factors, is discussed separately. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis" and "Pregnancy in women with congenital heart disease: General principles".)
●Pregnancy is associated with high maternal and fetal risks in patients with Eisenmenger complex (Eisenmenger syndrome with a VSD). Therefore, such patients should be counseled against pregnancy and advised about appropriate contraception options [80,81]. The management of contraception and pregnancy in patients with Eisenmenger syndrome is discussed separately (See "Pregnancy in women with congenital heart disease: General principles" and "Pulmonary hypertension with congenital heart disease: Pregnancy and contraception".)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Congenital heart disease in adults".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail 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 topics (see "Patient education: Ventricular septal defects in adults (The Basics)")
SUMMARY AND RECOMMENDATIONS
●The clinical course and prognosis of isolated ventricular septal defects (VSDs) depend on the type, size of the defect, and associated hemodynamic abnormalities.
•Most small VSDs close during childhood. A VSD that has closed spontaneously is generally associated with an excellent prognosis, although there is a very low risk of complications such as double chambered right ventricle and sinus of Valsalva dilatation/aneurysm. Patients with closed VSDs no longer face the risks of development of pulmonary hypertension (PH) and endocarditis associated with persistent VSD. (See 'Spontaneous closure' above.)
•Persistent small VSD are associated with a low risk of complications. Adults with small persistent VSDs with normal pulmonary artery pressure generally do not require intervention and have an excellent prognosis; however, these patients are at risk of development of endocarditis, double-chambered right ventricle (DCRV), or aortic regurgitation (AR), and periodic follow-up by a congenital heart disease (CHD) cardiologist is recommended. (See 'Persistent VSDs' above.)
•Larger VSDs are likely to persist and entail greater risk of complications. Adults with moderately restrictive or large VSDs are at risk for development of progressive pulmonary vascular disease, which may lead to severe irreversible PH with shunt reversal and cyanosis (Eisenmenger complex), heart failure (HF), and arrhythmias. Other potential complications include AR, DCRV, and endocarditis. Indications for VSD closure should be assessed, and regular follow-up by a CHD cardiologist is recommended for these patients. (See 'Persistent VSDs' above and 'Complications and their management' above.)
●The management of patients with VSD depends on the shunt severity, the type of defect, its size, and associated acquired complications, including DCRV, AR, and PH. (See 'Approach to management' above.)
●The general management of patients with VSD includes periodic follow-up to assess symptoms and signs, look for the development of associated complications and identify measures to reduce the risk of endocarditis. (See 'Approach to management' above and 'Endocarditis' above.)
●The following are indications for VSD closure (algorithm 1) (see 'Indications for VSD closure' above):
•For adults with VSD with hemodynamically significant shunt without significant PH, we recommend VSD closure (Grade 1B). We define a hemodynamically significant shunt as a pulmonary to systemic flow ratio (Qp:Qs) ≥1.5:1 with left ventricular (LV) dilation. We define lack of significant PH as pulmonary artery systolic pressure <50 percent of systemic arterial pressure, and pulmonary vascular resistance less than one-third of systemic vascular resistance. This recommendation is based upon natural history data suggesting that patients with hemodynamically significant shunt are at risk for the development of PH, and data showing excellent outcomes in patients without PH undergoing VSD repair. (See 'Pulmonary hypertension' above and 'Choice of intervention' above.)
•For adults with VSD with net left-to-right shunt with a Qp:Qs ≥1.5 with LV dilation, with pulmonary artery pressure ≥50 percent but less than two-thirds of systemic pressure and pulmonary vascular resistance greater than or equal to one-third but less than two-thirds of systemic vascular resistance, management is based upon an individualized assessment of the benefits and risks of VSD closure by adult CHD and PH experts. (See 'Pulmonary hypertension' above and 'Choice of intervention' above.)
•For adults with significant or worsening AR caused by a perimembranous or infundibular VSD who do not have other indications for closure, we suggest surgical closure of the VSD (Grade 2C). The aortic valve is commonly repaired at the time of VSD closure. Surgical aortic valve replacement is reserved for patients with AR with an indication for aortic valve surgery when the aortic valve cannot be durably repaired. (See "Natural history and management of chronic aortic regurgitation in adults", section on 'Indications for aortic valve surgery'.)
•For patients with VSD with a history of infective endocarditis, we suggest an individualized assessment of the risks and benefits of VSD closure. If there is no contraindication to closure, shared decision making is appropriate. (See 'Endocarditis' above and 'Indications for VSD closure' above.)
●For patients with severe irreversible pulmonary artery hypertension (Eisenmenger syndrome), we recommend not closing the VSD (Grade 1B). Closure of a VSD in patients with Eisenmenger syndrome is associated with high risk of mortality and is therefore not performed. (See 'Indications for VSD closure' above and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)
●Patients with VSD and PH who do not meet the above criteria for pulmonary artery pressure and pulmonary vascular resistance may occasionally become candidates for VSD closure if these criteria are met after advanced therapy for pulmonary arterial hypertension. This decision requires multidisciplinary care review and discussion including involvement of an adult CHD and PH-CHD specialist. (See 'Pulmonary hypertension' above.)
●Other surgical procedures (such as aortic valve surgery for AR or surgery for DCRV) may be performed at the time of VSD closure or subsequently, depending upon when complications requiring surgery develop. (See 'Approach to management' above and 'Aortic regurgitation' above and 'Double-chambered right ventricle' above.)
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