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

mild disease

1st line – observation

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ACUTE
|
mild disease
1st line – 

observation

This form is asymptomatic and rarely progresses. It requires only sequential cardiology follow-up into adulthood.[13][17][18]

moderate disease

1st line – percutaneous balloon pulmonary valvuloplasty

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ACUTE
|
moderate disease
1st line – 

percutaneous balloon pulmonary valvuloplasty

In patients with moderate disease, intervention with percutaneous balloon pulmonary valvuloplasty (PBPV) is generally recommended.[13][15][18] Most experts agree that moderate gradients will eventually progress to severe obstruction and right heart failure and warrant invasive treatment independently of symptom status.[19] Nevertheless, the utility of invasive treatment in asymptomatic patients with moderate PS remains under debate, and its use varies by institution.

US guidelines recommend PBPV as the first-line therapy for moderate or severe PS and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance.[13] European guidelines recommend intervention in the presence of one or more of the following: symptoms related to PS; decreasing right ventricular function and/or progressive tricuspid regurgitation to at least moderate; and/or right-to-left shunting via an atrial septal defect or ventricular septal defect.[15]

The benefits of PBPV are that it is less invasive compared with surgical valvuloplasty, does not require cardiopulmonary bypass, and significantly decreases neonatal mortality.

2nd line – surgical valvuloplasty

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ACUTE
|
moderate disease
2nd line – 

surgical valvuloplasty

Surgical valvuloplasty (cutting of a constricted cardiac valve to relieve obstruction) is indicated if patients are ineligible for percutaneous balloon pulmonary valvuloplasty (PBPV), for example if they have a dysplastic pulmonary valve not amenable to balloon dilation (e.g., in Noonan syndrome) or if they have multiple levels of fixed obstruction (i.e., sub- and/or supravalvar), or if PBPV has previously failed.[13]

severe to critical disease

1st line – percutaneous balloon pulmonary valvuloplasty

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ACUTE
|
severe to critical disease
|
without respiratory distress or cyanosis
1st line – 

percutaneous balloon pulmonary valvuloplasty

Most experts agree that severe gradients will eventually progress to severe obstruction and right heart failure and warrant invasive treatment independently of symptom status.[19] Critical disease requires urgent treatment.

US guidelines recommend PBPV as the first-line therapy for moderate or severe PS and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance.[13] European guidelines recommend intervention in the presence of one or more of the following: symptoms related to PS; decreasing right ventricular function and/or progressive tricuspid regurgitation to at least moderate; and/or right-to-left shunting via an atrial septal defect or ventricular septal defect.[15]

The benefits of PBPV are that it is less invasive compared with surgical valvuloplasty, does not require cardiopulmonary bypass, and significantly decreases neonatal mortality.

2nd line – surgical valvuloplasty

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ACUTE
|
severe to critical disease
|
without respiratory distress or cyanosis
2nd line – 

surgical valvuloplasty

Surgical valvuloplasty (cutting of a constricted cardiac valve to relieve obstruction) is indicated if patients are ineligible for percutaneous balloon pulmonary valvuloplasty (PBPV), for example if they have a dysplastic pulmonary valve not amenable to balloon dilation (e.g., in Noonan syndrome) or if they have multiple levels of fixed obstruction (i.e., sub- and/or supravalvar), or if PBPV has previously failed.[13]

1st line – supplemental oxygen ± alprostadil

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ACUTE
|
severe to critical disease
|
with respiratory distress or cyanosis
1st line – 

supplemental oxygen ± alprostadil

Fraction of inspired oxygen (FiO₂) of 1 at a flow rate of 8 to 10 L/minute in infants, up to 15 L/minute in adults. Adjusted as necessary for pulse oximetry saturations of 92% to 96% in term infants/adults and 88% to 92% in preterm infants.

Cyanotic neonates who are unresponsive to oxygen can be treated with alprostadil (prostaglandin E1). This dilates arterioles and maintains patency of ductus arteriosus increasing blood flow to the lungs.[9] Maximal effect seen in 30 minutes. Doses above 0.03 micrograms/kg/min do not offer any clinical advantage once patency of the ductus arteriosus has been established. Alprostadil is usually continued until 24 hour post-balloon or surgical valvuloplasty.

Primary options

alprostadil: 0.05 to 0.1 micrograms/kg/minute intravenous infusion, maximum 0.4 micrograms/kg/minute

Plus – percutaneous balloon pulmonary valvuloplasty

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ACUTE
|
severe to critical disease
|
with respiratory distress or cyanosis
Plus – 

percutaneous balloon pulmonary valvuloplasty

Treatment recommended for ALL patients in selected patient group

Most experts agree that severe gradients will eventually progress to severe obstruction and right heart failure and warrant invasive treatment independently of symptom status.[13] Critical disease requires urgent treatment.

US guidelines recommend PBPV as the first-line therapy for moderate or severe PS and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance.[13] European guidelines recommend intervention in the presence of one or more of the following: symptoms related to PS; decreasing right ventricular function and/or progressive tricuspid regurgitation to at least moderate; and/or right-to-left shunting via an atrial septal defect or ventricular septal defect.[15]

The benefits of PBPV are that it is less invasive compared with surgical valvuloplasty, does not require cardiopulmonary bypass, and significantly decreases neonatal mortality.

2nd line – surgical valvuloplasty

Back
ACUTE
|
severe to critical disease
|
with respiratory distress or cyanosis
2nd line – 

surgical valvuloplasty

Surgical valvuloplasty (cutting of a constricted cardiac valve to relieve obstruction) is indicated if patients are ineligible for percutaneous balloon pulmonary valvuloplasty (PBPV), for example if they have a dysplastic pulmonary valve not amenable to balloon dilation (e.g., in Noonan syndrome) or if they have multiple levels of fixed obstruction (i.e., sub- and/or supravalvar), or if PBPV has previously failed.[13]

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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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