Introduction: Tetralogy of Fallot (TOF) is considered the most common cyanotic congenital heart disease. However, for it to be associated with absent pulmonary valve syndrome (with pulmonary artery aneurysm) is a rarity and the treatment becomes complicated.
Objective: To present a rare case of Tetralogy of Fallot associated with Pulmonary Artery Aneurysm.
Clinical Features: When the patient was born, he was already observed with cyanosis and was already diagnosed with congenital heart disease. Patient was then admitted when he was a month old since he was observed to be tachypneic and more cyanotic. CT scan was done which showed dilated left pulmonary artery, to consider aneurysm compressing the left mainstem bronchus.
Intervention and Outcome: Patient underwent TOF correction and pulmonary artery arterioplasty. Patient was on critical watch but expired on the 24th postoperative day.
Conclusion: This is a rare case which makes standardization of practice very difficult so that a team effort involving different specialties is needed