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Ventricular Septal Defect, General Concepts
Updated: Feb 10, 2009
Introduction
Background
A ventricular septal defect (VSD) is a hole or a defect in the septum that divides the 2 lower chambers of the heart and that results in a communication between the ventricular cavities. The defect may occur as a primary anomaly with or without additional major associated cardiac defects. A ventricular septal defect may occur as a single component of a wide variety of intracardiac anomalies, including tetralogy of Fallot (TOF), complete atrioventricular (AV) canal defects, transposition of great arteries, and corrected transpositions.
In this article, the term ventricular septal defect refers to an isolated ventricular septal defect, or a defect in a heart with AV concordance. That is, the atria are attached to the correct ventricle and the normally related arteries (great arteries arising from the appropriate ventricle [ie, an otherwise normal heart]) with no other major lesions. Isolated ventricular septal defect occurs in approximately 2-6 of every 1000 live births and accounts for more than 20% of all congenital heart diseases. Ventricular septal defects are the most common congenital heart defects encountered after bicuspid aortic valves.
Credit for the first clinical description is generally given to Roger's article published in 1879.1 The phrase maladie de Roger is still used to refer to a small asymptomatic ventricular septal defect. In 1898, Eisenmenger described a patient with ventricular septal defect, cyanosis, and pulmonary hypertension. This combination of a ventricular septal defect, pulmonary vascular disease, and cyanosis has been termed the Eisenmenger complex. Pulmonary vascular disease and cyanosis in combination with any other systemic-to-pulmonary connection has been called the Eisenmenger syndrome.2 Heath and Edwards described the morphologic changes associated with pulmonary vascular disease in 1958, and their 6 categories of vascular change have remained the standard of comparison to the present.3
Since 1979, real-time 2-dimensional echocardiography has dramatically improved the noninvasive anatomic assessment of ventricular septal defect.
Definition
Ventricular septal defect is a developmental defect of the interventricular septum, wherein communication between the cavities of the 2 ventricles is observed.
Embryology
At 4-8 weeks' gestation, the single ventricular chamber is effectively divided into 2. This division is accomplished with the fusion of the membranous portion of the ventricular septum, the endocardial cushions, and the bulbous cordis (proximal portion of the truncus arteriosus).
The muscular portion of the ventricular septum grows cephalad as each ventricular chamber enlarges, eventually meeting with the right and left ridges of the bulbous cordis. The right ridge fuses with the tricuspid valve and the endocardial cushions, separating the pulmonary valve from the tricuspid valve. The left ridge fuses with a ridge of the interventricular septum, leaving the aortic ring in continuity with the mitral ring.
The endocardial cushions develop concomitantly and finally fuse with the bulbar ridges and the muscular portion of the septum.
The fibrous tissue of the membranous portion of the interventricular septum makes the final closure and separates the 2 ventricles.
Anatomy
The interventricular septum is a curvilinear complex structure and can be divided into 4 zones by anatomic landmarks in the right ventricle (RV). The RV has many heavy trabeculations. The stoutest of these is a Y -shaped bundle (ie, the trabecula septomarginalis), which proceeds toward the apex and which gives rise to the moderator band that courses transversely near the apex. The trabecula septomarginalis is an important structure that helps in the identification of the RV, regardless of its location in the chest. The 2 limbs of the Y travel superiorly, and the anterior, or parietal, limb supports the pulmonic valve and the posterior limb (septal band) extends to the membranous septum.
The 4 parts of the ventricular septum are as follows:
- the inlet septum is smooth walled and extends from the septal attachments of the tricuspid valve to the distal attachments of the tricuspid tensor apparatus. This region has also been called the AV canal septum.4
- The apical trabecular zone separates the coarse trabeculations of the RV from the fine ones seen in the left ventricle (LV). Van Praagh et al refer to this as the muscular septum or the ventricular sinus septum.4
- The smooth-walled outlet or infundibular septum is separated from the trabeculated portion of the RV by the septal band of the trabecula marginalis. Van Praagh et al called this area the parietal band or the distal conal septum and refer to defects in this area as conal septal defects.4
- The last and the smallest region in the ventricular septum is the membranous septum. This lies between the anterior and the septal tricuspid leaflets and below the right and the noncoronary cusps of the aortic valve.
- The 3 muscular components of the ventricular septum described above abut on the membranous septum and fan out from it as triangles, with the apices touching this septum. In the normal heart, the tricuspid and mitral valves are attached to the ventricular septum at different levels so that the tricuspid-valve attachment is apically displaced compared with the mitral-valve attachment. Therefore, a portion of the interventricular septum, called the AV septum, lies between the right atrium (RA) and the LV. This portion consists of a membranous part anteriorly and a muscular part posteriorly and is usually present in most hearts with an isolated ventricular septal defect.
- In the anterior aspect, the tricuspid-valve attachment divides the area of membranous septum into an interventricular component (between the LV and RV) and an AV component (between the LV and RA). When a ventricular septal defect is isolated, the AV component of membranous septum is usually intact.
Classifications of ventricular septal defects
Many classifications of ventricular septal defects have been proposed. An underlying classification that is surgically and clinically useful is described below.
Perimembranous (infracristal, conoventricular) ventricular septal defects lie in the LV outflow tract just below the aortic valve. Because they occur in the membranous septum with defects in the adjacent muscular portion of the septum, they are subclassified as perimembranous inlet, perimembranous outlet, or perimembranous muscular. These are the most common types of ventricular septal defects and account for 80% of such defects. Perimembranous ventricular septal defects are associated with pouches or aneurysms of the septal leaflet of the tricuspid valve, which can partially or completely close the defect. In addition, an LV-to-RA shunt may be associated with this defect.
Supracristal (conal septal, infundibular, subpulmonic, subarterial, subarterial doubly committed, outlet) ventricular septal defects account for 5-8% of isolated ventricular septal defects in the United States but 30% of isolated ventricular septal defects in Japan. These defects lie beneath the pulmonic valve and communicate with the RV outflow tract above the supraventricular crest and are associated with aortic regurgitation secondary to the prolapse of the right aortic cusp.
Muscular ventricular septal defects (trabecular) are entirely bounded by the muscular septum and are often multiple. The term Swiss-cheese septum has been used to describe multiple muscular ventricular septal defects. Other subclassifications depend on the location and include central muscular or midmuscular, apical, or marginal when the defect is along the RV-septal junction. These ventricular septal defects account for 5-20% of all defects. Any single defect observed from the LV aspect may have several openings on the RV aspect.
Posterior (canal-type, endocardial cushion–type, AV septum–type, inlet, juxtatricuspid) ventricular septal defects lie posterior to the septal leaflet of the tricuspid valve. Although locations of posterior ventricular septal defects are similar to those of ventricular septal defect observed with AV septal defects, they are not associated with defects of the AV valves. About 8-10% of ventricular septal defects are of this type.
Other anatomic considerations
The relationship of the AV conduction pathways to the defect is important to surgical repair. The AV node occupies the apex of the triangle of Koch that is limited posteriorly by the tendon of Todaro, inferiorly by the os of the coronary sinus and superiorly by the tricuspid valve annulus. The bundle of His arises from the AV node. In perimembranous defects, the bundle of His lies in a subendocardial position as it courses along the posterior-inferior margin of the defect. In inlet defects, the bundle of His passes anterosuperiorly to the defect. In muscular ventricular septal defects and outlet defects, the risk of heart block is minimal because the bundle is remote from the defect.
Patients with subpulmonary conal defects usually have deficiency of muscular or fibrous support below the aortic valve with subsequent herniation of the right aortic leaflet. However, in patients with perimembranous ventricular septal defects with aortic insufficiency, it may be the right or the noncoronary cusp that prolapses.
Classification of congenital malformations - Phenotype
For purposes of etiologic analysis, clustering defects by potential pathogenic mechanisms is beneficial. The following pathologic classification allows for comparison of similar defects.
- Subarterial ventricular septal defect can be classified as abnormalities of ectomesenchymal tissue migration.
- Perimembranous ventricular septal defect can be classified as abnormal intracardiac blood flow.
- Muscular ventricular septal defect can be classified as abnormalities in cell death.
- Type III in-flow ventricular septal defect can be classified as abnormalities of the extracellular matrix and defects in the endocardial cushion.
Pathophysiology
A defect in the ventricular septum allows a communication between the systemic and pulmonary circulations. As a result, flow moves from a region of high pressure to low pressure (from the LV to the RV [ie, left-to-right shunt]). The pathophysiologic effects of a ventricular septal defect are secondary to hemodynamic effects secondary to a left-to-right shunt and changes in the pulmonary vasculature.
Left-to-right shunt
A left-to-right shunt at the ventricular level has 3 hemodynamic consequences: increased LV volume load, excessive pulmonary blood flow, and reduced systemic cardiac output.
Blood flow through the defect from the LV to the RV results in oxygenated blood entering the pulmonary artery (PA). This extra blood in addition to the normal pulmonary flow from the vena cava increases blood flow to the lungs and subsequently increases pulmonary venous return into the left atrium (LA) and ultimately into the LV. This increased LV volume results in LV dilatation and then hypertrophy. It increases the end-diastolic pressure and consequently LA pressure and then pulmonary venous pressure.
The increased pulmonary blood flow raises pulmonary capillary pressure, which can increase pulmonary interstitial fluid. When this condition is severe, patients can present with pulmonary edema. Therefore, both PA pressure and pulmonary venous pressure are elevated in a ventricular septal defect. The increase in pulmonary venous pressure is not seen with an atrial septal defect because LA pressures are low, as blood can readily exit it through the atrial communication.
Finally, as blood is shunted through the ventricular septal defect away from the aorta, cardiac output decreases, and compensatory mechanisms are stimulated to maintain adequate organ perfusion. These mechanisms include increased catecholamine secretion and salt and water retention by means of the renin-angiotensin system.
The degree of the left-to-right shunt determines the magnitude of the changes described above. The left-to-right shunt depends on 2 factors: One is anatomic, and the other is physiologic. The anatomic factor is the size of the ventricular septal defect. In a normal heart, RV pressure is about 25% that of the LV. In a large ventricular septal defect, this pressure difference is no longer maintained because a large hole offers no resistance to blood flow. They are consequently called nonrestrictive ventricular septal defects. On the other hand, in a small ventricular septal defect, the normal pressure difference between the ventricles is maintained. These are called restrictive ventricular septal defects because blood flow across the defects is somewhat restricted, such that the normal pressure difference is maintained.
The physiologic factor is the resistance of the pulmonary vascular bed.
The location of the ventricular septal defect is irrelevant in terms of the degree of the shunt.
Changes in the pulmonary vasculature
The terms pulmonary hypertension, high pulmonary resistance, and pulmonary vascular disease are often confused.
Pulmonary hypertension merely indicates a high blood pressure in the pulmonary circuit, and, depending on the duration, it can be reversible. Pulmonary resistance is a function of numerous factors, including age, altitude, hematocrit, and diameter of the pulmonary arterioles.
A neonate has increased resistance secondary to the increase in the media of the pulmonary arterioles and this decreases the effective diameter of the vessels. In addition to this, neonates have a relative polycythemia. This elevated pulmonary resistance usually declines to adult levels by 6-8 weeks.
Pulmonary vascular disease is ultimately an irreversible condition and may occur in individuals with a large left-to-right shunt over time. It may also occur in the absence of a shunt; this condition is called primary pulmonary hypertension.
A characteristic series of histologic changes ranging from grade I to grade VI are described.3
The ultimate consequences of pulmonary vascular obstructive disease are irreversible vascular changes and pulmonary resistance equal to or exceeding systemic resistance.
Natural history
The natural history has a wide spectrum, ranging from spontaneous closure to congestive heart failure (CHF) to death in early infancy.
Spontaneous closure frequently occurs in children, usually occurs by age 2 years. Closure is uncommon after age 4 years. Closure is most frequently observed in muscular defects (80%), followed by perimembranous defects (35-40%). Outlet ventricular septal defects have a low incidence of spontaneous closure, and inlet ventricular septal defects do not close.
Closure may occur by means of hypertrophy of the septum, formation of fibrous tissue, subaortic tags, apposition of the septal leaflet of tricuspid valve, or (in rare cases) prolapse of a leaflet of the aortic valve. When perimembranous ventricular septal defects close because of development of fibrous tissue or the apposition of the tricuspid valve, an aneurysm of the interventricular septum may appear.
A small ventricular septal defect that does not spontaneously close is generally associated with a good prognosis. Patients are at risk for infective endocarditis, but small muscular ventricular septal defects pose no other adverse possibilities. However, small perimembranous VSD are associated with an increased risk of prolapse of the aortic cusp over time. In addition, a small but definite risk of malignant ventricular arrhythmia was reported by Kidd et al.5 This study group, the Second Natural History Study, consisted of about 1000 patients who formed about 76% of the original cohort. The original cohort was the First Natural History study and included 1280 patients (mostly children) with ventricular septal defects admitted after cardiac catheterization for a period from 1958-1969.
Wu et al reported a 45% incidence of LV-to-RA shunts and a 6% incidence subaortic ridges during 20-year follow-up of about 900 patients with perimembranous.6 This group later reported an increased incidence of infective endocarditis in patients who have LV-to-RA shunts.7
Frequency
United States
Ventricular septal defects affect 2-7% of live births.
The patient's area of residence may influence the prevalence of known ventricular septal defects. For example, small muscular ventricular septal defects are most likely to be identified in urban locations possibly because of ready access to sophisticated healthcare in these locations.
An echocardiographic study revealed a high incidence of 5-50 ventricular septal defects per 1000 newborns. The defects in this study were small restrictive muscular ventricular septal defects, which typically spontaneously close in the first year of life.
Ventricular septal defects are the most common lesion in many chromosomal syndromes, including trisomy 13, trisomy 18, trisomy 21, and relatively rare syndromes. However, in more than 95% of patients with ventricular septal defects, the defects are not associated with a chromosomal abnormality.
Race
Reports are inconclusive regarding racial differences in the distribution of ventricular septal defects. However, the doubly committed or outlet defect occurs is most common in the Asian population. These constitute 5% of the defects in the Unites States but 30% of those reported in Japan.
Sex
Ventricular septal defects are slightly more common in female patients with in male patients (56% vs 44%). The incidence of abnormalities of the ectomesenchymal tissue migration (ie, subarterial ventricular septal defect outlet) is highest in boys.
Clinical
History
Symptoms and physical findings associated with ventricular septal defects (VSDs) depend on the size of the defect and the magnitude of the left-to-right shunt.
- Infants with small defects
- Patients have mild or no symptoms.
- These cases are most often brought to the cardiologist's attention because a murmur is detected during routine examination.
- Feeding or weight gain is usually not affected.
- Infants with moderate defects
- Babies may have excessive sweating due to increased sympathetic tone. This sweating is especially notable during feeds.
- An important symptom is fatigue with feeding. Because feeding results in a need for increased cardiac output, this activity may unmask exercise intolerance in a baby.
- A sensitive sign may be the lack of adequate growth, which is due to an increased caloric requirement and an inability of the infant to feed adequately.
- Frequent respiratory infections may occur secondary to the pulmonary congestion.
- Symptoms, which begin as pulmonary vascular resistance (PVR) decreases, may be clearly apparent by age 2-3 months.
- Symptoms occur earlier in the premature infant than in the full-term infant because pulmonary resistance decreases earlier in preterm babies than in term babies.
- Infants with large ventricular septal defects
- Symptoms and signs are similar to, but more severe than, those observed in infants with moderate defects.
- Symptoms may be delayed, as they are with large defects, because of a delayed decrease in pulmonary vascular pressures.
- Poor weight gain and frequent respiratory infections are common.
- Patients with Eisenmenger syndrome, or ventricular septal defect with severe pulmonary vascular disease
- At rest, patients may have no symptoms
- With exercise, symptoms include exertional dyspnea, cyanosis, chest pain, syncope, and hemoptysis.
Physical
In a patient with small defects, physical findings are primarily cardiac manifestations. In patients with moderate-to-large defects, growth may be affected that they cause abnormalities apparent on general examination.
- Infants with small defects
- Patients may have normal vital signs.
- Physiologic splitting of S2 is usually retained.
- The characteristic harsh, holosystolic murmur is loudest along the lower left sternal border (LSB), and it is well localized. Small defects can produce a high-pitched or squeaky noise.
- The murmur is usually detected after the PVR decreases at about 4-8 weeks of age.
- Infants with moderate defects
- Infants often have a normal length and decreased weight. Poor weight gain is a sensitive indicator of congestive heart failure (CHF).
- Infants may have mild tachypnea, tachycardia, and an enlarged liver.
- The precordial activity is accentuated.
- The murmur with moderate-sized defects is usually associated with thrill. A holosystolic harsh murmur is most prominent over the lower LSB.
- The intensity of the pulmonary component is usually normal or slightly increased.
- In addition to the harsh holosystolic murmur, a diastolic rumble may be detected in the mitral area. This rumble suggests functional mitral stenosis secondary to a large left-to-right shunt and indicates a surgical-level shunt (pulmonary-to-systemic flow ratio [Qp:Qs], 2:1)
- Infants with large ventricular septal defects
- As with moderate defects, signs of CHF are present. The cardinal signs of heart failure include tachycardia, tachypnea, and hepatomegaly. In addition, cardiomegaly is present and helps in differentially diagnosing heart failure as opposed to a respiratory condition, such bronchiolitis.
- The murmur is holosystolic but poorly localized and is usually associated with a diastolic rumble.
- A ventricular septal defect is not typically associated with cyanosis. It is a "pink" condition; persistent cyanosis from birth indicates a relatively complicated lesion than isolated ventricular septal defect. The occurrence of cyanosis after infancy suggests reversal of the shunt. Patients with large ventricular septal defects and marked elevations of PVR frequently appear well in childhood because the blood flow in their systemic and pulmonary circuits is well balanced.
- Infants with ventricular septal defects and high PVR
- Children with Eisenmenger syndrome may have tachypnea only with exercise and not at rest.
- They may be only mildly cyanotic at rest but then develop profound cyanosis with exercise.
Causes
- Epidemiology: The incidence of ventricular septal defect in all live births is 1.5-3.5 cases per 1000 term infants and 4.5-7 cases per 1000 premature infants. The lowered prevalence in adults is because many of the defects spontaneously close.
- Inheritance: At present, a multifactorial etiology based on an interaction between hereditary predisposition and environmental influences is assumed to cause the defects. The following questions have relevance to children, their family, and their parents alike:
- What caused a child's heart defect?
- What is the risk of the other children and grandchildren having a heart defect?
- Maternal factors
- Maternal diabetes: Maternal diabetes has long been recognized as a risk factor for congenital cardiovascular malformations (CCVMs).
- Maternal phenylketonuria: The risk of CCVMs remains high for infants of women with poorly controlled elevated phenylalanine levels.
- Maternal alcohol consumption and fetal alcohol syndrome: No population-based data are available to define the range of risk alcohol consumption poses to the developing cardiovascular system. Investigators from the Baltimore-Washington Infant Study (BWIS) reported that maternal alcohol consumption was associated with only muscular ventricular septal defect.8
- Genetic risk factors (familial aggregation of cardiac and noncardiac abnormalities)
- The single largest determinant in the BWIS data set is the presence of a genetic risk factor defined as a preoccurrence of a congenital cardiovascular defect in the family.
- A family history of a cardiac or noncardiac defect in either a parent or a preceding sibling is a major risk factor.
- The incidence of ventricular septal defect in siblings of patients with the same malformation is about 3 times that of the general population.
- Ventricular septal defects have been reported in identical twins, but the frequency of discordance is high, even in identical twins.
- Familial congenital heart defects are often concordant by phenotype and developmental mechanism. Among cases with ventricular septal defects, preoccurrence of transposition, tetralogy of Fallot (TOF), and truncus arteriosus is higher than expected.
- Genotype-phenotype correlation
- The challenge for the next generation of pediatric cardiologists is to collaborate with geneticists to define genotype-phenotype correlations.
- Regarding genetic counseling and prospects for prevention, the single greatest change in counseling regarding the recurrence risk for CCVMs is the recognition of familial and chromosomally based defects. Thorough evaluation includes the following:
- An accurate clinical diagnosis of the cardiovascular defect(s) organized in a hierarchy (This is necessary to specify the type of ventricular septal defect.)
- Carefully detailed noncardiac defects
- Careful family history of first-degree and second-degree relatives, including detailed analysis of pregnancy loss, racial origin, and consanguinity
- A search for risk factors, such as gestational diabetes mellitus
- Associated syndromes Aneuploid Syndromes Associated with Ventricular Septal Defect
Open table in new window
[ CLOSE WINDOW ]Table
Syndrome CCVM (%) Type of CCVM Del 4q, 21, 32 60 Ventricular septal defect, atrial septal defect Del 5p 30-60 Ventricular septal defect Trisomy 13 80 Atrial septal defect, ventricular septal defect, TOF Trisomy 18, Edwards syndrome 100 Ventricular septal defect, TOF, double-outlet right ventricle (DORV) Trisomy 21, Down syndrome 40-50 Ventricular septal defect, atrioventricular canal (AVC) Del 22q11, DiGeorge syndrome (single gene etiology, autosomal dominant) 50 Truncus arteriosus, TOF, ventricular septal defect Syndrome CCVM (%) Type of CCVM Del 4q, 21, 32 60 Ventricular septal defect, atrial septal defect Del 5p 30-60 Ventricular septal defect Trisomy 13 80 Atrial septal defect, ventricular septal defect, TOF Trisomy 18, Edwards syndrome 100 Ventricular septal defect, TOF, double-outlet right ventricle (DORV) Trisomy 21, Down syndrome 40-50 Ventricular septal defect, atrioventricular canal (AVC) Del 22q11, DiGeorge syndrome (single gene etiology, autosomal dominant) 50 Truncus arteriosus, TOF, ventricular septal defect
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References
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Further Reading
Keywords
ventricular septal defect, VSD, isolated ventriculoseptal defect, isolated ventricular defect, maladie de Roger, Eisenmenger complex, Eisenmenger's syndrome, Eisenmenger syndrome, tetralogy of Fallot, TOF), complete atrioventricular canal defects, transposition of great arteries, corrected transpositions, cyanosis, hypertension, perimembranous ventricular septal defect, perimembranous VSD, conal septal, infundibular, subpulmonic, subarterial, subarterial doubly committed, outlet, supracristal ventricular septal defect, polycythemia, congestive heart failure, CHF, infective endocarditis, cardiomegaly, tachycardia, congenital cardiovascular malformations, CCVM, gestational diabetes mellitus
