Quadricuspid pulmonary valve associated with atrial septal defects and pulmonary stenosis

Abstract

A 1-month-old Hispanic female was referred to the cardiology service. During physical examination, a systolic ejection murmur at the pulmonic area was auscultated. The echocardiography evaluation of the pulmonary valve demonstrated a quadricuspid pulmonary valve (QPV) with slightly thickened leaflets, associated atrial septal defects and mild pulmonary stenosis. QPV is a rare congenital anomaly variant that can remain asymptomatic with few or non-hemodynamic alterations. Associations with structural or functional alterations have been reported. This is the first case of QPV that was diagnosed in a Hispanic newborn.

Multimodality imaging of quadricuspid pulmonary valve associated with pulmonary artery aneurysm

“…Quadricuspid pulmonary valve (QPV) is a rare abnormality of the pulmonary valve with an incidence reported from 1 in 1000 to 1 in 20 000 necropsies and is reported to be more common in men . It has been described as two small and two large cusps; four cusps of equal size; or a rudimentary and deformed/shrunken accessory cusp accompanied by three cusps of normal size .…”