Absent pulmonary valve syndrome with ascending aortic aneurysm

Absent pulmonary valve syndrome is rare. We report the case of a child with absent pulmonary valve syndrome noted after birth. Progressive dilatation of the main pulmonary artery and ascending aorta were noted by echocardiography and confirmed by heart computed tomography. He underwent ascending aorta graft replacement and pulmonary artery reduction angioplasty at the age of 6. Good outcome was achieved at 18-month follow-up where subsequent computed tomography revealed a normal size ascending aorta and main pulmonary artery.