Approach

Treatment is guided by the disease severity determined by echocardiogram. See Diagnostic criteria.

Mild disease typically requires no intervention, whereas percutaneous balloon pulmonary valvuloplasty or surgical valvuloplasty may be warranted in moderate or severe/critical disease.[13][17][18]

In individuals with pulmonary stenosis (PS), those undergoing cardiac surgery with cardiopulmonary bypass during infancy are at a heightened risk of developing developmental delays or disorders, necessitating vigilant follow-up. Patients presenting with chronic cyanosis without cardiopulmonary bypass in infancy also warrant close monitoring because they have an increased risk of neurodevelopmental challenges. Additionally, a subset of patients may not display conventional risk factors but still possess an elevated risk of neurodevelopmental issues due to interventions or hospitalisations related to their condition from infancy through to adolescence.[7]

Mild disease

This form of PS rarely progresses, and although requiring sequential cardiology follow-up into adulthood, requires no medical or surgical therapy.[13][18][19] Patients are asymptomatic and symptoms, if they appear, should not be attributed to PS but should be investigated further to elucidate the cause.

Moderate disease

In patients with moderate disease, intervention with percutaneous balloon pulmonary valvuloplasty (PBPV) is generally recommended.[13][15][18] Although the Second Natural History Study, which followed patients with moderate PS over 20 years, reported excellent outcomes with and without invasive treatment, 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]

PBPV is determined by invasive cardiac catheterisation. It relieves right ventricular outflow tract obstruction by dilating the valve.[20] The benefits of this procedure are that it is less invasive compared with surgical valvuloplasty, does not require cardiopulmonary bypass, and significantly decreases neonatal mortality.

Surgical valvuloplasty (cutting of a constricted cardiac valve to relieve obstruction) is indicated if patients are ineligible for 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 or critical disease

Prior to echocardiographic assessment, oxygen may be started in patients with cyanosis or respiratory distress. The flow should be set to a fraction of inspired oxygen (FiO₂) of 1 with a flow rate of 8 to 10 L/minute in infants. A maximum of 15 L/minute can be given in adults. Cyanotic neonates who are unresponsive to oxygen can be treated with alprostadil (prostaglandin E1). This dilates arterioles and maintains the patency of the ductus arteriosus, increasing blood flow to the lungs.[9]

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] All patients therefore require urgent PBPV or surgical valvuloplasty; the indications and approach are the same as for moderate disease.[9][13][15][18]

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