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

pulmonary vascular resistance (PVR) <5 Wood units (WU) and Qp:Qs <1.5

1st line – observation

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ACUTE
|
pulmonary vascular resistance (PVR) <5 Wood units (WU) and Qp:Qs <1.5
1st line – 

observation

Young patients with small interatrial communications can be observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

PVR <5 WU and Qp:Qs ≥1.5, or evidence of right atrial enlargement

1st line – corrective closure

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ACUTE
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PVR <5 WU and Qp:Qs ≥1.5, or evidence of right atrial enlargement
1st line – 

corrective closure

If the ratio of pulmonary to systemic blood flow, Qp:Qs, is or remains ≥1.5, or there is evidence of right atrial enlargement, the defect requires closure to prevent heart failure, atrial arrhythmias, and pulmonary vascular obstructive disease.[28] This is usually performed at age 2 to 4 years. Patients who develop unremitting heart failure require earlier closure.

Ostium secundum atrial septal defect (ASD) or vestibular ASD: device closure is the preferred method if rims are adequate to secure a device. If the rims are inadequate or there are large septal aneurysms or multiple fenestrations of the atrial septum, surgical closure is required.

Ostium primum defect: surgical closure with repair of the left atrioventricular valve is the most commonly used procedure.

Coronary sinus defect: surgical closure can be performed by simply closing the orifice of the coronary sinus. More complex correction is needed in the presence of a persistent left superior vena cava.

Sinus venosus defect: anomalous right pulmonary veins are baffled via the defect to the left atrium. A Warden operation may also be performed, whereby the superior vena cava is transected and then reconnected to the right atrial appendage while a patch is placed to the orifice of the superior vena cava to route anomalous right pulmonary vein flow across the defect to the left atrium.

Sinus venosus defect with partial anomalous pulmonary drainage and unroofed coronary sinus (especially if associated with a persistent left superior vena cava) should be corrected irrespective of shunt volume.

Surgical correction can be performed with minimally invasive strategies through thoracotomies or mini-sternotomies, with equivalent results when compared with conventional median sternotomy procedures.[26]

Consider – prophylactic antibiotics

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ACUTE
|
PVR <5 WU and Qp:Qs ≥1.5, or evidence of right atrial enlargement
Consider – 

prophylactic antibiotics

Additional treatment recommended for SOME patients in selected patient group

Required for the first 6 months after device or surgical closure, to prevent endocarditis.[23][27]

The antibiotics are given before a procedure that may cause bacteraemia. Guidelines on which procedures require prophylaxis vary between countries, and local guidelines on endocarditis prophylaxis should be adhered to. For example, many guidelines no longer recommend prophylaxis for dental procedures, or for procedures involving the upper and lower gastrointestinal tract, genitourinary tract, or the upper and lower respiratory tract.

Clindamycin is an alternative to penicillin in patients with a penicillin allergy.

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

PVR ≥5 WU

1st line – pulmonary vasodilator

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ACUTE
|
PVR ≥5 WU
1st line – 

pulmonary vasodilator

Corrective closure is not recommended if PVR is ≥5 WU. Instead, pulmonary vasodilator therapy should be initiated in an attempt to lower PVR.[23][27]​​

Pulmonary vasodilators that have been shown to be of benefit include endothelin receptor antagonists (e.g., bosentan), the phosphodiesterase-5 inhibitors sildenafil or tadalafil, and, in limited studies, infusions of the prostanoid epoprostenol.[29]

Primary options

bosentan: body weight ≤40 kg: 62.5 mg orally twice daily; body weight >40 kg: 62.5 to 125 mg orally twice daily

OR

sildenafil: 20-80 mg orally three times daily; 10 mg intravenously every 8 hours

OR

tadalafil: 40 mg orally once daily

Secondary options

epoprostenol: 2 nanograms/kg/minute intravenously initially, increase by 2 nanograms/kg/minute every 15 minutes according to response

Plus – corrective closure

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ACUTE
|
PVR ≥5 WU
|
PVR drops to <5 WU after pulmonary vasodilator therapy (reversible)
Plus – 

corrective closure

Treatment recommended for ALL patients in selected patient group

If pulmonary vasodilator treatment lowers PVR to <5 WU, corrective closure may be considered (and vasodilator treatment discontinued).[23]

Ostium secundum atrial septal defect (ASD) or vestibular ASD: device closure is the preferred method if rims are adequate to secure a device. If the rims are inadequate or there are large septal aneurysms or multiple fenestrations of the atrial septum, surgical closure is required.

Ostium primum defect: surgical closure with repair of the left atrioventricular valve is the most commonly used procedure.

Coronary sinus defect: surgical closure can be performed by simply closing the orifice of the coronary sinus. More complex correction is needed in the presence of a persistent left superior vena cava.

Sinus venosus defect: anomalous right pulmonary veins are baffled via the defect to the left atrium. A Warden operation may also be performed, whereby the superior vena cava is transected and then reconnected to the right atrial appendage while a patch is placed to the orifice of the superior vena cava to route anomalous right pulmonary vein flow across the defect to the left atrium.

Sinus venosus defect with partial anomalous pulmonary drainage and unroofed coronary sinus (especially if associated with a persistent left superior vena cava) should be corrected irrespective of shunt volume.

Surgical correction can be performed with minimally invasive strategies through thoracotomies or mini-sternotomies, with equivalent results when compared with conventional median sternotomy procedures.[26]

Consider – prophylactic antibiotics

Back
ACUTE
|
PVR ≥5 WU
|
PVR drops to <5 WU after pulmonary vasodilator therapy (reversible)
Consider – 

prophylactic antibiotics

Additional treatment recommended for SOME patients in selected patient group

Required for the first 6 months after surgical closure, to prevent endocarditis.[23][27]

The antibiotics are given before a procedure that may cause bacteraemia. Guidelines on which procedures require prophylaxis vary between countries, and local guidelines on endocarditis prophylaxis should be adhered to. For example, many guidelines no longer recommend prophylaxis for dental procedures, or for procedures involving the upper and lower gastrointestinal tract, genitourinary tract, or the upper and lower respiratory tract.

Clindamycin is an alternative to penicillin in patients with penicillin allergy.

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

Plus – continue pulmonary vasodilator

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ACUTE
|
PVR ≥5 WU
|
PVR remains ≥5 WU after pulmonary vasodilator therapy (irreversible)
Plus – 

continue pulmonary vasodilator

Treatment recommended for ALL patients in selected patient group

Corrective closure is generally not recommended if the PVR remains ≥5 WU despite adequate pulmonary vasodilator therapy. Supportive medical therapy with pulmonary vasodilators is the mainstay of treatment, and should be continued.

Note that in some patients with a left-to-right shunt and PVR ≥5WU, corrective closure may still be considered by some clinicians on a case-by-case basis, weighing risks and benefits.

Consider – monitoring and treatment of hyperviscosity

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ACUTE
|
PVR ≥5 WU
|
PVR remains ≥5 WU after pulmonary vasodilator therapy (irreversible)
Consider – 

monitoring and treatment of hyperviscosity

Additional treatment recommended for SOME patients in selected patient group

Fixed pulmonary hypertension with shunt reversal as a result of pulmonary overcirculation is also referred to as Eisenmenger's syndrome and is associated with various degrees of desaturation. Patients with Eisenmenger's syndrome frequently develop erythrocytosis to compensate for the hypoxaemia, which can lead to hyperviscosity.

Hyperviscosity can produce symptoms of headache, fatigue, and sometimes mental status changes. Treatment of symptomatic patients involves phlebotomy and intravenous infusion of saline.

Routine phlebotomy should be avoided.

Consider – pulmonary-to-systemic shunt (reversed Potts shunt)

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ACUTE
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PVR ≥5 WU
|
PVR remains ≥5 WU after pulmonary vasodilator therapy (irreversible)
Consider – 

pulmonary-to-systemic shunt (reversed Potts shunt)

Additional treatment recommended for SOME patients in selected patient group

Last resort option for patients with pulmonary arterial hypertension who have undergone surgical correction of congenital heart disease and are unresponsive to maximal medical therapy. May improve exercise capacity and facilitate easier medical management.[10]

Consider – consider for heart-lung or double lung transplantation

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ACUTE
|
PVR ≥5 WU
|
PVR remains ≥5 WU after pulmonary vasodilator therapy (irreversible)
Consider – 

consider for heart-lung or double lung transplantation

Additional treatment recommended for SOME patients in selected patient group

Heart-lung transplantation may be considered for some patients. When feasible, surgical correction of the interatrial communication together with double lung transplantation may also be considered.[10]

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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

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