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

hypercyanotic spells

1st line – maneuvers to increase systemic venous return

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ACUTE
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hypercyanotic spells
1st line – 

maneuvers to increase systemic venous return

Infant should be kept calm and maneuvers tried to increase the amount of blood exiting the right ventricle through the pulmonary vasculature instead of to the aorta.

Infant should be held in the parent's arms and positioned with the knees to the chest.

Consider – supportive care

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ACUTE
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hypercyanotic spells
Consider – 

supportive care

Treatment recommended for SOME patients in selected patient group

Supportive medical therapy may include volume/blood administration as needed, intravenous morphine to calm the child as needed, and bicarbonate to reverse acidosis as needed. Oxygen should be given, but care should be taken not to increase the infant's stimulation.

2nd line – beta-blocker

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ACUTE
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hypercyanotic spells
2nd line – 

beta-blocker

Thought to help resolve hypercyanotic spells, but the mechanism of action is not completely clear. It is believed that it helps to decrease the infundibular obstruction by decreasing the heart rate, prolonging diastolic filling, and decreasing contractility.[17]

May be initiated in patients with TOF with pulmonary stenosis if maneuvers such as knee-to-chest positioning or fluid bolus have not resolved the hypercyanotic spell.

Propranolol has been used in the past in the outpatient setting of an infant with spells to delay surgery. An infant with a single spell is considered an indication for urgent surgical repair, but propranolol may be used until surgery can be arranged.[29]

Primary options

esmolol: 100-500 micrograms/kg intravenous bolus initially, followed by 50-500 micrograms/kg/min infusion

More

OR

propranolol hydrochloride: 0.15 to 0.25 mg/kg intravenous bolus initially, may repeat in 15 min; 2-8 mg/kg/day orally given in divided doses every 6 hours

Consider – supportive care

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ACUTE
|
hypercyanotic spells
Consider – 

supportive care

Treatment recommended for SOME patients in selected patient group

Supportive medical therapy may include volume/blood administration as needed, intravenous morphine to calm the child as needed, and bicarbonate to reverse acidosis as needed. Oxygen should be given, but care should be taken not to increase the infant's stimulation.

3rd line – phenylephrine

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ACUTE
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hypercyanotic spells
3rd line – 

phenylephrine

Used to increase systemic venous resistance by direct simulation of peripheral alpha-adrenergic receptors.

The goal is to increase the systemic vascular resistance above the resistance of the pulmonary outflow tract so that blood in the right ventricle preferentially flows through the pulmonary circulation, rather than across the ventricular septal defect and into the systemic circulation.

Used in the intensive care unit setting as the last medical option for management of hypercyanotic spells. It is initiated when positioning and beta-blocker agents have not increased pulmonary blood flow.

Primary options

phenylephrine injection: 5-20 micrograms/kg intravenous bolus, followed by 0.1 to 0.5 micrograms/kg/min infusion

Consider – supportive care

Back
ACUTE
|
hypercyanotic spells
Consider – 

supportive care

Treatment recommended for SOME patients in selected patient group

Supportive medical therapy may include volume/blood administration as needed, intravenous morphine to calm the child as needed, and bicarbonate to reverse acidosis as needed. Oxygen should be given, but care should be taken not to increase the infant's stimulation.

neonate with profound cyanosis and severely limited pulmonary blood flow

1st line – alprostadil

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ACUTE
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neonate with profound cyanosis and severely limited pulmonary blood flow
1st line – 

alprostadil

May benefit from alprostadil (prostaglandin E1) to maintain the patency of the ductus arteriosus. This provides an alternative source of pulmonary blood flow while the infant awaits urgent intervention.

A potential adverse effect is apnea, and mechanical ventilation may be required. Monitor for apnea.

Primary options

alprostadil: 0.02 to 0.1 micrograms/kg/min intravenously

Consider – supportive care

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ACUTE
|
neonate with profound cyanosis and severely limited pulmonary blood flow
Consider – 

supportive care

Treatment recommended for SOME patients in selected patient group

Supportive medical therapy may include volume/blood administration as needed, intravenous morphine to calm the child as needed, and bicarbonate to reverse acidosis as needed. Oxygen should be given, but care should be taken not to increase the infant's stimulation.

neonate or infant with nonremitting severe cyanosis

1st line – surgical shunt, transcatheter stent/balloon, or immediate complete repair

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ACUTE
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neonate or infant with nonremitting severe cyanosis
1st line – 

surgical shunt, transcatheter stent/balloon, or immediate complete repair

For neonates or infants with TOF and severe pulmonary obstruction in whom the pulmonary blood flow is significantly restricted, an alternative source of pulmonary blood flow may be necessary.

If all medical therapies fail to resolve a spell and the patient remains severely cyanotic, urgent intervention will be needed.

Options include catheter-based right ventricular outflow tract ballooning or stenting, stenting of a patent ductus arteriosus, Blalock-Taussig shunt placement (a small GORE-TEX tube placed from a systemic artery to the pulmonary arteries to increase pulmonary blood flow), or immediate complete repair.​[22][27][28]

Consider – extracorporeal membrane oxygenation (ECMO)

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ACUTE
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neonate or infant with nonremitting severe cyanosis
Consider – 

extracorporeal membrane oxygenation (ECMO)

Treatment recommended for SOME patients in selected patient group

Emergent ECMO may be initiated to reverse cyanosis and acidosis before surgical intervention.

ONGOING

all patients

1st line – complete surgical repair

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ONGOING
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all patients
1st line – 

complete surgical repair

Specific treatment guidelines for surgical management of TOF do not exist, largely due to the complexity of the anatomic diversity among patients.

The anatomic variations in TOF, as well as its association with other cardiovascular anomalies, greatly influence the physiologic consequences.

Additionally, surgical technique varies by surgical program. Thus, the management of each patient is determined in consultation with cardiology and cardiovascular surgery.

Plus – monitoring with possible pulmonary valve replacement

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ONGOING
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all patients
Plus – 

monitoring with possible pulmonary valve replacement

Treatment recommended for ALL patients in selected patient group

Patients who undergo repair in childhood should be counseled about the possible need for future surgical or transcatheter interventions.There is an increasing awareness of the need to monitor patients for progressive dilation of the right ventricle secondary to longstanding pulmonary regurgitation, and the need for pulmonary valve replacement in this setting.

Typically, magnetic resonance imaging measurements are used to quantify the pulmonary regurgitation and right ventricular size. While there is no consensus on the exact criteria for the timing of replacement of the pulmonary valve, one review suggests using the following criteria:[34]

Repaired TOF or similar physiology with moderate or severe pulmonary regurgitation (regurgitation fraction ≥25% measured by cardiovascular magnetic resonance imaging) and 2 or more of the following criteria: right ventricular end-diastolic volume index ≥160 mL/m² (Z score >5); right ventricular end-systolic volume index ≥70 mL/m²; left ventricular end-diastolic volume index ≤65 mL/m²; right ventricular ejection fraction ≤45%; right ventricular outflow tract aneurysm; or clinical criteria (exercise intolerance, symptoms and signs of heart failure, cardiac medications, syncope, or sustained ventricular tachycardia).

Other hemodynamically significant lesions such as moderate or severe tricuspid regurgitation, residual atrial or ventricular septal defects, and severe aortic regurgitation may trigger referral for surgery in patients with moderate or severe pulmonary regurgitation. In the absence of the 6 criteria above, pulmonary valve replacement should be considered on a case-by-case basis.

Due to a higher risk of adverse clinical outcomes in patients who underwent TOF repair at age ≥3 years, pulmonary valve replacement may be indicated sooner and in the presence of less severe right ventricular dilation and dysfunction.

Consider – infective endocarditis prophylaxis

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ONGOING
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all patients
Consider – 

infective endocarditis prophylaxis

Treatment recommended for SOME patients in selected patient group

Cardiac surgery: directed primarily against staphylococci. First-generation cephalosporin (e.g., cephalexin) is most commonly used, but antibiotic choice depends on susceptibility patterns at each hospital.[35]

Infective endocarditis prophylaxis before dental procedures or for procedures on the respiratory tract or infected skin, skin structures, or musculoskeletal tissue is required for unrepaired cyanotic congenital heart disease (CHD), including palliative shunts and repaired CHD with residual defects at or adjacent to the site of a prosthetic patch or device: directed primarily against enterococci. Amoxicillin or ampicillin is the preferred agent.[35]

Primary options

cephalexin: 50 mg/kg orally as a single dose given 60 minutes before procedure, maximum 2000 mg/dose

OR

amoxicillin: 50 mg/kg orally as a single dose given 60 minutes before procedure, maximum 2000 mg/dose

OR

ampicillin: 50 mg/kg intravenously/intramuscularly as a single dose given 30 minutes before fore procedure, maximum 2000 mg/dose

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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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