Ventricular Septal Defect (VSD) – 8 Interesting Facts

Sharing is caring!

What is Ventricular Septal Defect (VSD)?

A ventricular septal defect is a hole in the wall (septum) between the bottom chambers of the heart (ventricles). This is a problem that is present at birth (congenital heart defect). A VSD can change the normal flow of blood in the body.

A VSD is often found during a routine exam in the first few months of a child’s life. Your child may or may not have symptoms, depending on the size and location of the hole. Small VSDs may not cause symptoms and may go away on their own. Some larger VSDs may require treatment.

Interesting Facts

  1. Ventricular septal defect accounts for 30% to 50% of congenital cardiac defects, with the majority being isolated, small, and hemodynamically insignificant 1
  2. Clinical presentation and natural history depend on size of the defect
    • Small ventricular septal defects are typically asymptomatic and usually discovered on routine clinical examination
    • Moderate to large ventricular septal defects typically manifest during infancy with symptoms and signs of congestive heart failure and holosystolic murmur
  3. Echocardiography confirms diagnosis, demonstrates size and location of defect, and evaluates hemodynamic consequences of defect
  4. Conservatively manage patients who have small asymptomatic ventricular septal defects, as most will close spontaneously
  5. Medically treat patients who have moderate to large ventricular septal defects and symptoms of congestive heart failure to alleviate symptoms before surgical closure
  6. Most patients with moderate to large ventricular septal defects should undergo surgical repair before age 1 year; without intervention, these defects may cause left to right shunting, which leads to pulmonary hypertension, heart failure, and Eisenmenger syndrome 2
  7. Open surgical ventricular septal defect repair is the procedure of choice for most patients requiring repair; transcatheter approach is an alternative for muscular ventricular septal defects in selected patients
  8. Prognosis is excellent when ventricular septal defect is surgically corrected during infancy

What are the causes?

The cause of this condition is not known.

What increases the risk?

This condition is more likely to develop in:

  • Children with a family history of congenital heart defects.
  • Children with certain genetic conditions, such as Down syndrome.

What are the signs or symptoms?

Signs and symptoms of VSD depend on the size of the hole. Small VSDs often do not cause problems. The only symptom may be an abnormal sound (murmur) that your child’s health care provider hears when listening to your child’s heart. Moderate and large VSDs may cause symptoms that start several weeks after birth. Symptoms may include:

  • Shortness of breath.
  • Excess sweating, especially during feeding or eating.
  • Poor appetite.
  • Tiring easily during exercise.
  • Trouble gaining weight.
  • Rapid breathing.
  • Blue skin color.

How is this diagnosed?

This condition may be diagnosed based on:

  • A physical exam.
  • Chest X-ray.
  • Electrocardiogram (ECG). This test records the electrical activity of the heart.
  • Echocardiogram. This test uses sound waves to create an image of the heart.
  • Cardiac catheterization. During this procedure, a flexible tube (catheter) is inserted into a blood vessel and guided into the heart. This procedure provides information about the heart structures as well as blood pressure and oxygen levels within the heart chambers.

How is this treated?

Treatment for this condition depends on your child’s age and the size and location of the VSD. Many VSDs will close on their by age 2 without treatment. Others may stay the same.

  • If your child has a small VSD that causes no symptoms:
    • Your child will need to have regular checkups with a health care provider to make sure there are no problems.
    • There will usually be no activity limitations.
  • If your child has symptoms of a VSD, but there is a chance that the VSD may close, your child may need to take medicines that strengthen the heart and help control blood pressure. Your child may take these medicines until the VSD closes or surgery becomes necessary.
  • If your child has a medium or large VSD, surgery may be needed to close the hole.
    • This surgery is usually done before age 2. In some cases, it may be done during adolescence.
    • Children who have surgery for a VSD may need to take antibiotic medicine for 6 months after the procedure. This is to protect against an infection of the inner surface of the heart (infective endocarditis).

Follow these instructions at home:

Medicines

  • Give your child over-the-counter and prescription medicines only as told by the health care provider.
  • If your child was prescribed an antibiotic medicine, give it to him or her as told by the health care provider. Do not stop giving the antibiotic even if your child starts to feel better.
  • Do not give your child aspirin because of the association with Reye syndrome.

Dental care

  • Make sure your child gets regular dental care and brushes and flosses regularly. This will help reduce the risk for infective endocarditis.
  • Some children with VSDs or repaired VSDs need to take antibiotics before having dental work or other surgical procedures. These medicines help prevent infective endocarditis. Be sure to tell your child’s health care providers, including the dentist if your child:
    • Has a VSD.
    • Has a repaired VSD.
    • Has had infective endocarditis in the past.
    • Has an artificial (prosthetic) heart valve.

General instructions

  • Have your child avoid body piercings. Piercings increase the chance that bacteria can get into the body and cause infective endocarditis. If your child has a heart defect and wants a piercing, talk with your child’s health care provider first.
  • If your child has trouble gaining weight, ask the health care provider if your child needs calorie-boosting supplements.
  • Your child may need to work with a heart specialist (pediatric cardiologist). Ask your child’s health care provider if this is necessary.
  • Keep all follow-up visits as told by your child’s health care provider. This is important.

Contact a health care provider if:

  • Your child has a fever.
  • Your child is eating poorly.
  • Your child has trouble gaining weight.
  • Your child has sudden weight gain.
  • Your child’s symptoms change or your child has new symptoms.

Get help right away if:

  • Your child has shortness of breath.
  • Your child who is younger than 3 months has a fever of 100°F (38°C) or higher.
  • Your child is pale, cold, or clammy.
  • Your child’s lips or fingers are bluish in color.

Summary

  • A ventricular septal defect (VSD) is a hole in the wall (septum) between the bottom chambers of the heart (ventricles). This is a problem that is present at birth (congenital heart defect).
  • Signs and symptoms of VSD depend on the size of the hole. Small VSDs often do not cause problems.
  • If your child has a medium or large VSD, surgery may be needed to close the hole.
  • Be sure to tell your child’s health care providers, including the dentist if your child has a VSD.

Detailed Information

Pitfalls

  • Eisenmenger syndrome, a complication of unrepaired ventricular septal defect, results in shunt reversal. Consequently, physical signs (eg, murmurs, thrills) can disappear; this should not be mistaken for evidence that ventricular septal defect is closing spontaneously

Terminology

Clinical Clarification

  • Ventricular septal defect is a defect in the interventricular septum that results in communication between the 2 ventricles
    • Isolated ventricular septal defect, 1 of the most common congenital cardiac defects, occurs in approximately 0.3 to 7.7 per 1000 live births and accounts for approximately 30% to 50% of congenital cardiac defects worldwide 1 3

Classification

  • Most commonly occurs as an isolated defect; however, may be complicated by additional intracardiac lesions or occur as part of more complex cardiac defects (eg, tetralogy of Fallot, double outlet right ventricle, transposition of great vessels, functionally univentricular heart) 4
  • Most commonly classified according to location of defect within interventricular septum
    • Supracristal (also called infundibular, outlet, conal, conoventricular, or subpulmonary) ventricular septal defect
      • Defect in infundibular component of muscular septum below pulmonary valve 3
      • May exist in isolation but most frequently is associated with other defects (eg, tetralogy of Fallot) 3
      • Accounts for 5% to 8% of ventricular septal defects in western countries; more common (21%-30%) in Asian countries 5
    • Perimembranous (also called paramembranous or infracristal)
      • Septal defect adjacent to membranous portion of septum 6
      • Subclassified as inlet, muscular, or outlet 3
      • Most common form (70%-80%) of ventricular septal defect overall 7
    • Inlet (atrioventricular canal)
      • Located posteriorly at inlet region of right ventricular septum (corresponding to outlet of left ventricle)
      • Often associated with an atrioventricular septal defect 7
      • Accounts for 5% of ventricular septal defects 6 7
    • Muscular
      • Single or multiple defects in lower muscular portion of ventricular septum 3
      • Accounts for 20% of ventricular septal defects 7
  • Eisenmenger syndrome 3
    • Irreversible pulmonary hypertension occurring as a result of a large ventricular septal defect (or other systemic to pulmonary intracardiac communication) that is uncorrected or undergoes delayed correction
    • Longstanding left to right shunt in ventricular septal defect leads to increasing right heart pressures, pulmonary vascular changes, and ultimately shunt reversal (right to left) with hypoxemia and cyanosis

Diagnosis

Clinical Presentation

  • From Kaya H et al: A rare case of ventricular septal defect associated with patent ductus arteriosus and coarctation of aorta: a multimodality approach. J Cardiovasc Comput Tomogr. 7(2):138-40, 2013, Figure 1.Echocardiographic views show a 1.3-cm perimembranous ventricular septal defect with bidirectional shunt.
  • From Nichols DG et al: Critical Heart Disease in Infants and Children. 2nd ed. Philadelphia, PA: Elsevier; 2006: 579-92, Figure 24-7.Various types of ventricular septal defects viewed from within the right ventricle. – A, Supracristal or subarterial. B, Membranous or paramembranous. C, Inlet or atrioventricular (AV) canal type. D, Muscular or trabecular.

History

  • Small ventricular septal defects are usually asymptomatic and detected during the first few months of life when a murmur is heard on routine examination 2
  • Moderate to large ventricular septal defects present with symptoms and signs of congestive heart failure that develop a few weeks to months after birth, as pulmonary vascular resistance (which is characteristically high in early neonatal period) decreases, allowing left to right interventricular shunt to develop 2
    • Infants may present with:
      • Poor feeding
      • Fatigue during feeding
      • Tachypnea during feeding or at rest
      • Diaphoresis
      • Wheezing or grunting
      • Poor weight gain
      • Frequent respiratory infections
    • Older children, adolescents, or adults with an unrepaired ventricular septal defect or ventricular septal defect repaired after early childhood may present with symptoms of Eisenmenger syndrome: 2
      • Dyspnea
      • Fatigue
      • Palpitations
      • Chest pain
      • Headache
      • Disequilibrium
      • Syncope
      • Cyanosis
  • Adult presenting with an undiagnosed ventricular septal defect is rare

Physical examination

  • Auscultation findings include: 3
    • Holosystolic or pansystolic murmur
      • Grade depends on velocity of flow; smaller defects are loudest and thrill may be present
        • When ventricular septal defect is large, murmur is typically quieter (with a blowing sound) and without associated thrill
      • Murmur location is dependent on defect location
        • Muscular defects are heard over lower left sternal border
        • Supracristal defects are best heard at mid- to upper left sternal border
      • Ventricular septal defect murmur may be absent in Eisenmenger syndrome owing to reversal of shunt direction (do not mistake for evidence that ventricular septal defect is closing spontaneously)
    • Perimembranous defects may have associated systolic click of tricuspid aneurysm
    • Loud single S₂ is present over left superior sternal border in large ventricular septal defects 2
    • Loud pulmonary component of S₂ in the presence of pulmonary hypertension 2
    • When ventricular septal defect is large with low pulmonary vascular resistance, diastolic rumble is heard at apex as a result of increased mitral flow
  • In patients with small ventricular defects, other examination findings are typically normal
  • Signs suggestive of heart failure and/or left to right shunting associated with moderate to large ventricular septal defects
    • Low body weight for age
    • Tachypnea
    • Tachycardia
    • Hepatomegaly
    • Left precordium prominence
    • Palpable parasternal lift
    • Intercostal retractions
    • Nasal flare
  • Signs associated with Eisenmenger syndrome 3
    • Cyanosis
    • Clubbing
    • Tachypnea
    • Palpable right ventricular heave

Causes and Risk Factors

Causes

  • Caused by inadequate development of the interventricular septum. Thought to result from interactions between various genetic, epigenetic, maternal, and environmental factors 8

Risk factors and/or associations

Genetics
  • Ventricular septal defect may occur in conjunction with multiple congenital defects in the following chromosomal disorders: 9
    • Trisomy 21 (Down syndrome) 10
    • Monosomy 22q11 (DiGeorge syndrome) 11
    • TBX5 mutation (Holt-Oram syndrome) 12
    • Monosomy 11q (Jacobsen syndrome) 13
    • Trisomy 13 (Patau syndrome)
    • Trisomy 18 (Edwards syndrome)
    • Monosomy 10p
    • Trisomy 9
    • Monosomy 8p
    • Trisomy 8 mosaicism
    • Monosomy 4p (Wolf-Hirschhorn syndrome) 14
    • Monosomy 5p (cri du chat syndrome) 15
    • Chromosome 9q subtelomeric deletion (Kleefstra syndrome) 16 17
Ethnicity/race
  • Supracristal ventricular septal defect is more common among Asians (accounts for 21%-30% of ventricular septal defects in Asian countries compared with 5%-8% of cases in western countries) 5
Other risk factors/associations
  • Maternal illnesses and exposures to drugs and chemicals during pregnancy may be associated with increased risk of ventricular septal defect 18
    • Illness
      • Diabetes
      • Febrile illness
      • Influenza
      • Rubella
      • Untreated phenylketonuria
    • Exposure
      • Marijuana
      • Ibuprofen
      • Organic solvents
  • Smoking, obesity, and heavy alcohol consumption have also been implicated, but evidence is inconsistent

Diagnostic Procedures

Primary diagnostic tools

  • Suspect in presence of characteristic murmur with or without clinical manifestations of congestive heart failure; confirm diagnosis with echocardiography 3
    • Additional information may be obtained by chest radiography, MRI, and ECG; cardiac catheterization is rarely indicated 19

Imaging

  • Echocardiography (typically transthoracic or color Doppler 23
    • Confirms presence, size, and location of ventricular septal defect and provides information for planning surgical procedure
    • Allows hemodynamic evaluation of defect
    • Identifies elevated pulmonary artery pressure, obstruction of right ventricular outflow tract, aortic insufficiency, and distortion of valve apparatus
    • Transesophageal echocardiography is occasionally indicated if transthoracic echocardiography is inadequate
  • Chest radiography 3
    • Findings are usually normal in presence of small ventricular septal defects
    • Cardiomegaly and increased pulmonary vascularity are evident with larger ventricular septal defects
    • Right ventricular enlargement, prominent main pulmonary artery, and loss of pulmonary vasculature are seen with increasing pulmonary resistance or Eisenmenger syndrome
  • MRI
    • May be useful in patients with inadequate echocardiographic images 19
    • Can be useful to delineate anatomy in patients with ventricular septal defects in association with complex cardiac defects 3

Functional testing

  • ECG 3
    • Results are usually normal in patients with small ventricular septal defects
    • Increased voltage in V₅ and V₆, leads II and III, or aVF may suggest left ventricular hypertrophy in patients who have moderate to large ventricular septal defect with left to right shunting and volume overload
    • Tall R waves or upright T waves in V₄R and V₁ and narrow P waves may suggest right ventricular hypertrophy and right atrial enlargement in patients with increasing pulmonary resistance or Eisenmenger syndrome

Procedures

Cardiac catheterization
General explanation
  • Catheters are placed percutaneously in peripheral blood vessels and advanced into central circulation 20
  • Measures 20
    • Pressure within cardiac chambers and great vessels
    • Oxygen saturation in the heart and great vessels
    • Cardiac contractility and function
  • Angiography delineates anatomic structures and coronary artery patency 20
  • General anesthesia necessary when performed on infants 20
  • No longer routinely required in preoperative anatomic evaluation of ventricular septal defect; replaced by echocardiography and other noninvasive imaging modalities 21
Indication
  • Anatomic and physiologic assessment of congenital heart defects to determine risk for adverse outcome, need for intervention, and response to medical therapy 21
  • Indicated if anatomy cannot be adequately defined by noninvasive studies; 21 rarely required in patients with uncomplicated defects 19
  • May be undertaken in patients with pulmonary vascular disease to measure pulmonary vascular resistance, pulmonary reactivity, volume of shunting, and response to pulmonary vasodilators 3
Contraindications
  • No absolute contraindications
  • Relative contraindications
    • Contrast material allergy
    • Hemodynamic instability
    • Severe, uncontrolled hypertension
    • Severe anemia
    • Uncompensated congestive heart failure
    • Uncontrolled coagulopathy
Complications 22
  • Death
  • Myocardial infarction
  • Cerebrovascular accident
  • Arrhythmias
  • Perforation of the heart or great vessels
  • Allergic reaction to iodinated contrast medium
  • Acute renal injury due to iodinated contrast medium
  • Thrombosis
  • Bleeding
  • Pseudoaneurysm formation
  • Arteriovenous fistula formation
  • Embolization
  • Local vascular complications
  • Ionizing radiation exposure
  • Infection
Interpretation of results
  • Delineates anatomy of heart defect and provides information on hemodynamic implications

Differential Diagnosis

Most common

  • Atrioventricular septal defect 4
    • Septal defect involving both the atrial and ventricular septum
    • Frequently, presentation is similar to moderate to large ventricular septal defects, including tachypnea, feeding difficulties, poor weight gain, and respiratory infections in infancy
    • Typically associated with single S₁, holosystolic murmur at apex, and middiastolic rumble at lower left sternal border and apex
    • Definitive diagnosis is made based on echocardiographic findings
  • Patent ductus arteriosus 2
    • Persistent communication between the descending thoracic aorta and pulmonary artery due to failure of the fetal ductus arteriosus to close
    • May be asymptomatic or present with tachypnea, feeding difficulties, and poor weight gain, similar to ventricular septal defect
    • Left to right shunt results in pulmonary overcirculation and a continuous machinerylike murmur on auscultation
    • Precordial thrill may be present
    • Diagnosed based on echocardiographic findings
  • Atrial septal defect
    • Defect in atrial septum allowing communication between the right and left atria
    • As with ventricular septal defects, small defects are usually asymptomatic, whereas large defects may present with congestive heart failure in infancy
    • Soft systolic ejection murmur may be present; larger defect can result in middiastolic murmur
      • Wide fixed splitting of S₂ may be heard
    • Diagnosed based on echocardiographic findings
  • Double-chambered right ventricle 4
    • Right ventricle is separated into proximal high-pressure and distal low-pressure chambers by muscular bands
    • Often asymptomatic; may be associated with other syndromes (including ventricular septal defect) and may be found during evaluation of these
    • Can produce murmur very similar to that created by flow across ventricular septal defect; typically holosystolic murmur is loudest at mid- and upper left precordium
    • Diagnosed based on echocardiographic findings; some patients may require MRI or right ventricular pressure measurement via cardiac catheterization
  • Still murmur (innocent vibratory murmur) 23
    • Benign flow murmur that may be accentuated during high output states, such as fever
    • Asymptomatic
    • Associated with soft systolic murmur, loudest at lower left sternal border; may have vibratory or musical quality
    • Diagnosed clinically in older children; infants may require echocardiography to exclude pathologic murmur

Treatment

Goals 2

  • Relieve symptoms of congestive heart failure, if present
  • Prevent development of irreversible pulmonary hypertension or other long-term sequelae

Disposition

Admission criteria

  • Severe congestive heart failure
  • Need for inpatient monitoring during initiation of medical therapy for heart failure

Recommendations for specialist referral

  • Refer all infants and children with suspected or known ventricular septal defect to a pediatric cardiologist for evaluation and subsequent referral for surgical closure, if indicated 6

Treatment Options

Management depends on defect size, likelihood of spontaneous closure, symptom severity, and risk of long-term complications (eg, pulmonary hypertension, valve damage)

  • Manage children who have small ventricular septal defects conservatively with regular follow-up to monitor growth and development of symptoms 2
    • However, surgical repair is usually indicated for those with small supracristal ventricular septal defects owing to high risk of aortic valve insufficiency
  • Medically treat patients with moderate to large ventricular septal defects and congestive heart failure to alleviate symptoms before surgical closure; successful early treatment may allow surgical closure to be postponed or avoided in some cases 2
    • Medical therapy may consist of diuretics, ACE inhibitors, nutritional support, and supplemental oxygen; in severe cases, parenteral inotropic agents or digoxin may be indicated 24
    • Medical management of congestive heart failure in infants and children should be determined by a pediatric cardiologist with appropriate monitoring of clinical status and laboratory parameters
  • Surgical closure is indicated for patients with moderate to large ventricular septal defects in the following settings: 2
    • Patients of any age with clinical symptoms and failure to thrive that cannot be medically controlled
    • Infants aged 6 to 12 months with pulmonary hypertension, even if symptoms are medically controlled
    • Patients older than 2 years with pulmonary to systemic flow ratio greater than 2:1
    • Patients with supracristal ventricular septal defect of any size, even if asymptomatic
  • Open surgical closure is safe and effective; this treatment method usually prevents development of pulmonary vascular disease when performed before age 1 year 2
    • Transcatheter device closure is an alternative for some ventricular septal defects, particularly muscular, trabecular (Swiss cheese) ventricular septal defects. These defects are the most technically challenging to surgically repair and are associated with higher rates of postsurgical complications 21
    • In complex lesions or very unwell infants, closure may need to be approached as a 2-stage procedure with initial pulmonary artery banding and subsequent ventricular septal defect repair

Nondrug and supportive care

Supplemental oxygen 25

  • Controversial; consider in setting of pulmonary hypertension, including Eisenmenger syndrome
  • Can be given on a trial basis and continued only if it produces a consistent increase in arterial oxygen saturation

Nutritional support 24

  • Appropriate for patients with failure to thrive associated with ventricular septal defect
  • Increase daily caloric intake by giving high-calorie baby formulas, supplementing breastfeeding, or providing other nutritional support
  • Nasogastric feeding may be required if patient is unable to feed adequately
Procedures
Open ventricular septal defect repair 26

General explanation

  • Patch closure of ventricular septal defect under cardiopulmonary bypass
  • Single-stage procedure performed via sternotomy

Indication

  • Procedure of choice in most patients with hemodynamically significant ventricular septal defect

Contraindications

  • Patients with severe Eisenmenger syndrome who are unresponsive to pulmonary vasodilators 27
Transcatheter ventricular septal defect closure 28 29

General explanation

  • Deployment of a device (eg, ventricular septal defect occluder) to close defect; performed via percutaneous catheter with transesophageal echocardiographic or fluoroscopic guidance 3
    • May also be undertaken as hybrid periventricular approach with open surgical incision, avoiding both cardiopulmonary bypass and adverse effects associated with transcatheter placement in small infants 21

Indication

  • Hemodynamically significant muscular ventricular septal defect 21
    • A child who weighs 5 kg or more and has favorable anatomy is considered a candidate for percutaneous closure 21
    • For infants who weigh less than 5 kg or for patients with abnormal septal wall planes, surgery or an alternative method of device delivery (ie, hybrid periventricular approach) is typically considered 21

Contraindications 21

  • Defect that is too complex or anatomically inappropriate for transcatheter closure
  • Weight less than 3 kg (unless hybrid periventricular approach is used) 21
  • Sepsis
  • Aspirin use
Pulmonary artery banding 30

General explanation

  • A band is placed around aortopulmonary trunk to restrict flow into pulmonary arteries, thereby preventing pulmonary overflow and development of pulmonary hypertension
    • Subsequent debanding and repair of ventricular septal defect is done in a second operation

Indication

  • Palliation or stalling for staged repair in the following settings:
    • Specific types of ventricular septal defects (multiple muscular Swiss cheese defects, defects with complex anatomy, univentricular physiology)
    • Infant with severe marasmus or sepsis
    • Cardiopulmonary bypass contraindicated (eg, after intracranial bleed)

Contraindications

  • Defect amenable to single-stage repair

Comorbidities

  • Ventricular septal defect occurring as a component of complex cardiac defects (eg, tetralogy of Fallot, double outlet right ventricle, transposition of great vessels, functionally univentricular heart) is addressed during management of these defects

Special populations

  • Pregnant patients
    • No contraindications to pregnancy in patients with repaired ventricular septal defect or isolated small defects and no pulmonary hypertension; pregnancy is well tolerated
    • Pregnancy is contraindicated in patients with ventricular septal defect and Eisenmenger syndrome; high risk of premature delivery and maternal and fetal death 19
      • Counsel against pregnancy, ensure adequate contraception, and consider early termination if pregnancy occurs
      • Requires obstetric management at specialist center with access to intensive care if pregnancy proceeds
  • Adults
    • Rare for adult to present with an undiagnosed isolated ventricular septal defect
      • Management should be undertaken at specialized adult congenital heart disease center and may include cardiac catheterization to assess operability, medical therapy to treat pulmonary vascular disease, and closure of the defect
    • Adults with previously repaired defects associated with heart failure or hemodynamic abnormalities or small residual defects require follow-up at a specialized adult congenital heart disease center every 1 to 5 years, depending on clinical scenario
    • Adults with previously repaired isolated defects and no pulmonary hypertension do not require specific follow-up at a specialized center

Monitoring

  • For patients who have small to moderate ventricular septal defects that do not warrant surgical closure, monitor with periodic clinical examination and echocardiography until ventricular septal defect closes spontaneously 2

Complications and Prognosis

Complications

  • Eisenmenger syndrome
    • Multisystem disorder associated with irreversible pulmonary hypertension, bleeding complications, paradoxical emboli, renal dysfunction, heart failure, hypertrophic osteoarthropathy, and premature death 31
      • Most severe complication of large ventricular septal defects
    • Longstanding left to right shunt leads to increasing right heart pressures, pulmonary vascular changes, and ultimately shunt reversal (right to left) with hypoxemia and cyanosis 31
      • Physical signs (eg, murmurs, thrills) can consequently disappear; this should not be mistaken for evidence that ventricular septal defect is closing spontaneously
  • Infective endocarditis2
    • Uncommon; extremely rare in children younger than 2 years 32
    • Antibiotic prophylaxis is required for dental procedures in the initial 6 months after repair with prosthetic material or device; prophylaxis is also required when residual defects remain after repair 33
    • Data support the importance of a focus on maintenance of good oral hygiene, prevention of gingival and dental disease, and access to routine dental care for children at risk 34
  • Aortic regurgitation 2
    • Secondary to prolapse of valve into the defect
    • Risk is greatest in supracristal ventricular septal defects
    • Results in gradually progressive left ventricular overload
  • Subaortic stenosis 35
    • Rare; can occur either spontaneously or after surgical closure
  • Right ventricular outflow obstruction 32
    • Infundibular, orificial, or mixed stenosis may occur
    • Reduces left to right shunt, thereby reducing occurrence of pulmonary hypertension (to an extent)

Prognosis

  • Small ventricular septal defects
    • 30% to 50% of defects close spontaneously by age 2 years and almost all close by age 4 years 2
    • Long-term morbidity is low even if defect does not spontaneously close 2
  • Moderate ventricular septal defects
    • Without surgery, defect size may diminish over time and a small proportion close completely 2
    • Risk of congestive heart failure depends on degree of left to right shunting
    • Many cases can be managed with medical therapy and surgery may not be required
  • Large ventricular septal defects 2
    • Rarely close spontaneously; associated with recurrent episodes of respiratory infection and congestive heart failure despite optimal medical management
    • In the small number of patients in whom the defect is not repaired and persists into late childhood without intervention, Eisenmenger syndrome later develops
  • Prognosis is good when moderate to large ventricular septal defects are corrected surgically during infancy
    • After primary surgical repair, consequences of shunting tend to reverse and there is marked improvement in clinical status and catch-up in growth 36
    • Life expectancy is normal if there is no residual defect
    • Complications of surgical repair are rare 2
      • Conduction disorders
        • Right bundle branch block occurs in 20% to 50% of ventricular septal defect repairs; usually benign and temporary 26
        • Complete heart block occurs in 1% to 3% of ventricular septal defect repairs 26
      • Valvular insufficiency (tricuspid or pulmonary, depending on surgical approach) 26
      • Cognitive difficulty (rare complication of cardiopulmonary bypass and induced hypothermia) 37

Screening and Prevention

Screening

At-risk populations

  • Patients with Down syndrome 3

Screening tests

  • Routine echocardiographic screening is standard of care in all patients with Down syndrome 3

Sources

1 Bernier PL et al: The challenge of congenital heart disease worldwide: epidemiologic and demographic facts. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 13(1):26-34, 2010 Reference

Sharing is caring!

Diphenhydramine

Diphenhydramine Brand Names Aid to Sleep | Alka-Seltzer Plus Allergy | Aller-G-Time...

Dinutuximab

Dinutuximab Brand Name– Unituxin What is Dinutuximab Dinutuximab is a...

You cannot copy content of this page

shares
15585

Sign up to receive the trending updates and tons of Health Tips

Join SeekhealthZ and never miss the latest health information

15856
Scroll to Top