Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

congenital: small

1st line – observation

Back
ACUTE
|
congenital: small
1st line – 

observation

In cases of small restrictive ventricular septal defects with a pulmonary-systemic blood flow ratio (Qp:Qs) <1.5:1, observation and follow-up is all that is generally indicated.[2]

Small restrictive defects detected at birth without symptoms close spontaneously in most cases, and the prognosis of those that fail to close is excellent.[2]

Consider – prophylactic antibiotics

Back
ACUTE
|
congenital: small
Consider – 

prophylactic antibiotics

Treatment recommended for SOME patients in selected patient group

Endocarditis is a complication of ventricular septal defects (VSDs), and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Prophylactic antibiotics are now only indicated in patients at particularly high risk of developing endocarditis.[2] In patients with a small VSD, this includes those with a previous history of infective endocarditis. Prophylaxis is no longer recommended for routine gastrointestinal procedures.

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More

congenital: medium or large

1st line – corrective closure

Back
ACUTE
|
congenital: medium or large
|
asymptomatic
1st line – 

corrective closure

Medium or large ventricular septal defects (VSDs) should be closed to prevent progression to severe pulmonary hypertension, heart failure, and Eisenmenger syndrome.[2]

Surgical closure is recommended in adults with evidence of left ventricular volume overload and hemodynamically significant shunts (a pulmonary-systemic blood flow ratio [Qp:Qs] ≥1.5:1) if: pulmonary artery (PA) systolic pressure is <50% systemic and pulmonary vascular resistance is less than one third systemic. Surgical closure may be considered: when a perimembranous or supracristal VSD causes worsening aortic regurgitation; with a history of infective endocarditis; or when PA systolic pressure is ≥50% systemic and/or pulmonary vascular resistance is greater than one third systemic with a left-to-right shunt (Qp:Qs ≥1.5:1).[2] 

The usual procedure is open surgery in which a patch (bovine pericardium or synthetic material) is used to close the VSD.[2] One study found that surgical repair and transcatheter device closure of perimembranous VSDs in children were equally effective, with transcatheter closure being associated with shorter hospital stay, less blood transfused, and lower cost.[28]

Percutaneous device closure is an option for type 4 (muscular) and some forms of type 2 (perimembranous) defects, especially if the defect is away from the tricuspid valve and the aorta.[29][30][31][32]

Consider – prophylactic antibiotics

Back
ACUTE
|
congenital: medium or large
|
asymptomatic
Consider – 

prophylactic antibiotics

Treatment recommended for SOME patients in selected patient group

Endocarditis is a complication of ventricular septal defects, and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Prophylactic antibiotics are now only indicated in patients at particularly high risk of developing endocarditis.[2] This includes patients with a previous history of infective endocarditis, patients within 6 months following patch repair or percutaneous device closure, or patients with a residual defect following closure. Prophylaxis is no longer recommended for routine gastrointestinal procedures.

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More

1st line – preoperative medical therapy

Back
ACUTE
|
congenital: medium or large
|
symptomatic with left-to-right shunt
1st line – 

preoperative medical therapy

Medical therapy is not curative and is used to control heart failure symptoms prior to surgery. This is mainly required in infants to delay closure until the infant has grown enough and surgery can be performed; these therapies are almost never required in adults.

Medications include diuretics, and in some cases ACE inhibitors and digoxin.[2][33]

Anemia, if present, should be corrected by red blood cell transfusion, or by iron therapy in case of iron-deficiency anemia.

Primary options

furosemide: children: consult specialist for guidance on dose

Secondary options

furosemide: children: consult specialist for guidance on dose

-- AND --

captopril: children: consult specialist for guidance on dose

or

enalapril: children: consult specialist for guidance on dose

-- AND --

digoxin: children: consult specialist for guidance on dose

Plus – corrective closure

Back
ACUTE
|
congenital: medium or large
|
symptomatic with left-to-right shunt
Plus – 

corrective closure

Treatment recommended for ALL patients in selected patient group

The development of heart failure symptoms is an indication for surgery. Surgical closure should be considered with: a pulmonary-systemic blood flow ratio (Qp:Qs) ≥1.5:1; a perimembranous or supracristal ventricular septal defect (VSD) causing worsening aortic regurgitation; or a history of endocarditis.[2] Surgery can be performed once heart failure symptoms have been controlled with therapy.

The usual procedure is open surgery in which a patch (bovine pericardium or synthetic material) is used to close the VSD.[2] One study found that surgical repair and transcatheter device closure of perimembranous VSDs in children were equally effective, with transcatheter closure being associated with shorter hospital stay, less blood transfused, and lower cost.[28]

Percutaneous device closure is an option for type 4 (muscular) and some forms of type 2 (perimembranous) defects, especially if they are away from the tricuspid valve or the aorta.[29][30][31][32]

Consider – prophylactic antibiotics

Back
ACUTE
|
congenital: medium or large
|
symptomatic with left-to-right shunt
Consider – 

prophylactic antibiotics

Treatment recommended for SOME patients in selected patient group

Endocarditis is a complication of ventricular septal defects, and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Prophylactic antibiotics are now only indicated in patients at particularly high risk of developing endocarditis.[2] This includes patients with a previous history of infective endocarditis, patients within 6 months following patch repair or percutaneous device closure, or patients with a residual defect following closure. Prophylaxis is no longer recommended for routine gastrointestinal procedures.

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More

1st line – supportive medical therapy with pulmonary vasodilators

Back
ACUTE
|
congenital: medium or large
|
symptomatic with right-to-left shunt (Eisenmenger syndrome)
1st line – 

supportive medical therapy with pulmonary vasodilators

If patients progress to shunt reversal with Eisenmenger syndrome, the ventricular septal defect is inoperable and treatment is supportive with pharmacotherapy.[5]

Drugs used to treat pulmonary hypertension include phosphodiesterase-5 (PDE-5) inhibitors (e.g., sildenafil, tadalafil), endothelin receptor antagonists (e.g., bosentan, ambrisentan), and prostacyclins (e.g., epoprostenol).[24]

Sildenafil and tadalafil have been shown to improve exercise capacity and hemodynamics in patients with Eisenmenger syndrome.[24][34] However, the US Food and Drug Administration (FDA) does not recommend the use of these agents for this indication in pediatric patients due to an increased risk of mortality with higher doses in one trial, unless the medical team consider that the benefits of treatment with the drug are likely to outweigh its potential risks.[35] Revatio (sildenafil): drug safety communication - FDA clarifies warning about pediatric use for pulmonary arterial hypertension Opens in new window

Bosentan and ambrisentan have also been shown to improve hemodynamics in Eisenmenger syndrome, but larger studies are needed.[24][36]​​​

Epoprostenol may be an alternative option to these agents.[37][38][39]

Monotherapy or combination therapy with these agents may be recommended, and the choice of regimen should be decided in consult with a specialist.

Plus – prophylactic antibiotics

Back
ACUTE
|
congenital: medium or large
|
symptomatic with right-to-left shunt (Eisenmenger syndrome)
Plus – 

prophylactic antibiotics

Treatment recommended for ALL patients in selected patient group

Endocarditis is a complication of ventricular septal defects, and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Antibiotic prophylaxis is indicated in all patients with Eisenmenger syndrome.[2]

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More

Consider – monitoring and treatment of hyperviscosity

Back
ACUTE
|
congenital: medium or large
|
symptomatic with right-to-left shunt (Eisenmenger syndrome)
Consider – 

monitoring and treatment of hyperviscosity

Treatment recommended for SOME patients in selected patient group

Patients with Eisenmenger syndrome frequently develop erythrocytosis to compensate for the hypoxemia, and some of them develop hyperviscosity.[5] Hyperviscosity manifests with headache, fatigue, dyspnea, and dizziness. Phlebotomy and intravenous infusion of saline may be performed in select patients if symptoms of hyperviscosity are severe.[5] Routine phlebotomy for asymptomatic erythrocytosis is not indicated. Anemia and volume depletion can cause similar symptoms and should be excluded before starting this type of therapy.[5]

2nd line – heart-lung transplantation

Back
ACUTE
|
congenital: medium or large
|
symptomatic with right-to-left shunt (Eisenmenger syndrome)
2nd line – 

heart-lung transplantation

In some patients who are severely symptomatic, heart-lung transplant may be considered and, when feasible, surgical correction of the ventricular septal defect together with lung transplant may be considered.[5][24]​​​ The survival of patients with Eisenmenger syndrome who receive a heart-lung transplant is similar to that of other heart-lung transplant recipients, or better, despite a more difficult operative course.​[24][44]​​​​

acquired

1st line – corrective closure following intra-aortic balloon pump insertion ± coronary artery bypass graft

Back
ACUTE
|
acquired
|
due to myocardial infarction
1st line – 

corrective closure following intra-aortic balloon pump insertion ± coronary artery bypass graft

Patients generally present with acute left-heart failure, and mortality without surgery is extremely high.[6] Mortality is much lower with urgent surgery.

Generally, patients undergo coronary angiography and intra-aortic balloon pump insertion prior to surgery in order to define coronary anatomy for possible coronary artery bypass graft (CABG) and to stabilize the patient with the intra-aortic balloon pump. CABG is carried out at the same time as the corrective closure.[6] [ Cochrane Clinical Answers logo ] Concomitant coronary revascularization appears to improve outcomes.[40]

Percutaneous device closure is an option if the risks of open surgical closure are too high.​[41][42][45]​​ These patients still receive an angiogram and intra-aortic balloon insertion.

Consider – prophylactic antibiotics

Back
ACUTE
|
acquired
|
due to myocardial infarction
Consider – 

prophylactic antibiotics

Treatment recommended for SOME patients in selected patient group

Endocarditis is a complication of ventricular septal defects (VSDs), and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Prophylactic antibiotics are now only indicated in patients at particularly high risk of developing endocarditis: patients with Eisenmenger syndrome; patients with a previous history of infective endocarditis; patients within 6 months following patch repair or percutaneous device closure of VSD; or patients with a residual defect following closure. Prophylaxis is no longer recommended for routine gastrointestinal procedures.[2]

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More

1st line – corrective closure

Back
ACUTE
|
acquired
|
traumatic
1st line – 

corrective closure

A traumatic ventricular septal defect is generally treated with surgical repair of the defect in patients with significant-sized defects. Small defects with insignificant shunts may be managed conservatively.

Consider – prophylactic antibiotics

Back
ACUTE
|
acquired
|
traumatic
Consider – 

prophylactic antibiotics

Treatment recommended for SOME patients in selected patient group

Endocarditis is a complication of ventricular septal defects (VSDs), and a high index of suspicion must be maintained.

Infective endocarditis often presents nonspecifically, and most commonly involves fever with possible physical signs of peripheral emboli (Osler nodes, Roth spots, or Janeway lesions).

Prophylactic antibiotics are now only indicated in patients at particularly high risk of developing endocarditis: patients with Eisenmenger syndrome; patients with a previous history of infective endocarditis; patients within 6 months following patch repair or percutaneous device closure of VSD; or patients with a residual defect following closure. Prophylaxis is no longer recommended for routine gastrointestinal procedures.[2]

Primary options

amoxicillin: children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure

OR

clindamycin: children: 20 mg/ kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

More
back arrow

Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

Use of this content is subject to our disclaimer