Objectives Berry syndrome is a very rare congenital cardiovascular anomaly. We report two infant cases in 2011.12–2012.1.

Methods Both the two cases were diagnosed by echocardiography, and were validated by CT scan and surgery. The anatomical structure and hemodynamic condition of the two cases were evaluated by echo after repair.

Results Case 1, male, 4-month-old, who referred to our hospital as pneumonia and 3/6 cardiac systolic murmur; Case 2, male, 2-month-old, who referred to our hospital as paroxysmal cough, choking one time accompanied by cyanosis and 3/6 cardiac systolic murmur; Echocardiography and CT scan revealed the two cases suffered with left heart over volume and with the anatomic characteristics of Berry syndrome: (1) An AP window(diameter 16 mm and 14 mm), (2) a type A interrupted aortic arch, (3) An RPA arising from ascending aorta, (4) A PDA. Two cases received surgical repair. Postoperative echo demonstrated satisfactory arch reconstruction and normal relationship of the two great arteries. The peak velocities of blood flow in aortic arch and descending aorta were increased, the measurement were 2.3 m/s/24 mm Hg and 2.6 m/s/26 mm Hg respectively.

Conclusions For Berry syndrome early clinical recognition, prompt echocardiography and CT scan, early surgical operation can lead good result. Postoperative echocardiography is mandatory because stenosis at the site of the aortic reconstruction and the RPA is a potential problem. Echocardiography could provide accurate preoperative diagnosis and postoperative evaluation.