Tests

1st tests to order

ECG

Test
Result
Test

A standard 12-lead ECG is routinely performed to identify cardiac anomalies that may be associated with congenital heart disease or valve disease.[13][14][15]​

Result

mild PS: typically normal or mild right axis deviation; moderate PS: right axis deviation (abnormal for age) and RV conduction delay (abnormal for age); severe/critical PS: extreme right axis deviation, R wave large for age, right atrial enlargement with tall and peaked P wave in lead II and in precordial leads V1 to V3

chest x-ray

Test
Result
Test

Routinely performed in patients with known or suspected valve disease.[14][15]​​​

Result

usually normal; may show prominent main pulmonary artery shadow; marked cardiomegaly, right atrial and ventricular enlargement, and decreased pulmonary vascularity may be seen in severe disease

echocardiography with Doppler assessment

Test
Result
Test

Two-dimensional echocardiography with Doppler interrogation is the investigation of choice.[13][15]

It confirms diagnosis by visualizing pulmonary valve and stenosis and classifies severity by measuring transvalvular gradient.

A transthoracic echocardiogram is usually performed first-line; a transesophageal echocardiogram may provide complementary information in adult or adolescent patients.​[13]​[15]​[16]​

Result

abnormal morphology of valve; increased transvalvular gradient across pulmonary valve during systole (>10 mmHg)

Tests to consider

diagnostic cardiac catheterization

Test
Result
Test

May be required in some patients to more precisely confirm the extent, severity, and level of right ventricular outflow tract obstruction.[13][14][15]

Required to differentiate critical pulmonary stenosis from pulmonary atresia with intact ventricular septum.[9]

Result

thickened and doming of valve in fluoroscopy and contrast angiography; increased transvalvular gradient (10 mmHg) measured by direct pullback or simultaneous measurement with double lumen catheters

Hb and Hct

Test
Result
Test

Required if cyanosis is present.

Result

increased in cyanosis caused by right-to-left shunt, leading to erythrocytosis

pulse oximetry

Test
Result
Test

Required if cyanosis is present.

Result

low arterial oxygen saturation (SaO₂) in central cyanosis

arterial blood gas

Test
Result
Test

Required if cyanosis is present.

Result

low PaO₂ in central cyanosis

cardiac MRI

Test
Result
Test

Not a first-line investigation but can support procedural planning and may be requested by the cardiac interventionist and/or surgeon.[13][15]

Result

additional information relating to the level of right ventricular outflow tract obstruction, right ventricular volumes, pulmonary annulus, outflow tract and artery dimensions, and differential pulmonary blood flow

cardiac CT

Test
Result
Test

Not a first-line investigation but can support procedural planning and may be requested by the cardiac interventionist and/or surgeon.[13][15]

Result

may show reduced functional capacity

exercise stress testing

Test
Result
Test

May be used to objectively assess symptoms if considering intervention.[13][14]

Result

may show reduced functional capacity

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