Isolated pulmonary valvular stenosis with equilateral triangle orifice

Area of Science:

• Cardiology
• Congenital Heart Disease
• Medical Imaging

Background:

• Isolated congenital pulmonary valvular stenosis is a common congenital heart defect.
• Pulmonary valvular stenosis can present asymptomatically into adulthood.
• Echocardiography and CT are key diagnostic tools.

Observation:

• A 52-year-old woman presented with an incidental cardiac murmur.
• Transthoracic echocardiography revealed mild pulmonary valvular stenosis (peak gradient 25 mmHg).
• Cardiac CT showed a dome-shaped pulmonary valve with a unique equilateral triangle orifice, lacking commissural fusion.

Findings:

• This is the first reported case of pulmonary valvular stenosis with an equilateral triangle orifice.
• The unique orifice morphology is hypothesized to be an embryonic remnant from semilunar leaflet formation.
• The patient's condition was classified as mild and asymptomatic.

Implications:

• This case expands the understanding of pulmonary valvular stenosis morphology.
• Careful follow-up is recommended for patients with this rare anatomical variation.
• Further research may elucidate the embryological origins of such variations.