2| CASE REPORT
The patient is a 6-year-old girl from a farming family in a
non-high-altitude area. She presented with mild cyanosis of the lips at
birth and was initially diagnosed with congenital heart disease. Because
of progressive cyanosis and dyspnea, the patient came to our outpatient
clinic for further diagnosis and treatment. She had normal growth and
development without significant physical or intellectual delays. The
family reported that she was prone to pneumonia and frequently coughed
since birth. Physical examination revealed significant cardiac murmurs,
clubbing of the fingers, and cyanosis of the lips. Electrocardiography
showed sinus rhythm with biventricular hypertrophy and right axis
deviation. Transthoracic echocardiography revealed significant
hypertrophy of both ventricles and the left atrium, with an absent echo
between the left wall of the ascending aorta and the right wall of the
main pulmonary artery (MPA), which measured approximately 18mm. There
was a narrow segment in the isthmus of the aortic arch, measured
approximately 4mm, and interruption of the aortic arch distal to the
left subclavian artery (LSA). The right pulmonary artery (RPA) arose
from the ascending aorta, while the left pulmonary artery (LPA)
originated from the pulmonary trunk. Between the descending aorta and
the left pulmonary artery, there was a prominent PDA with a diameter of
approximately 6mm. The atrial and ventricular septa were intact, valve
function was normal, and the coronary artery origins were normal.
Subsequently, the patient underwent CT angiography, which confirmed the
findings of the echocardiography. Three-dimensional reconstruction
clearly showed a type I APW, type A IAA, and PDA. Four branch vessels
were also visualized, namely the right common carotid artery (RCCA),
left common carotid artery (LCCA), anomalous right subclavian artery
(ARSA), and LSA, which all arose from the proximal end of the
interrupted aortic arch.
Based on the patient’s medical history and examinations, a definitive
diagnosis of Berry syndrome was established, and surgical intervention
was indicated. The patient’s family requested surgery. A standard
mid-sternotomy approach was performed, revealing abundant collateral
vessels from the main pulmonary artery. During the repair of interrupted
aortic arch, conventional deep hypothermic circulatory arrest and
selective cerebral perfusion were employed. Firstly, the ductus
arteriosus was dissected distally and ligated, followed by excision of
the ductal tissue. The aortic arch and descending aorta were carefully
mobilized, and an end-to-side anastomosis was performed between the
descending aorta and the aortic arch. The anterior wall was reinforced
using a pericardial patch. After the correction of IAA, the RPA was
disconnected. The APW was observed, and the defect was approximately 1cm
from the aortic valve annulus. The origin of the coronary arteries was
normal. Along the edge of the APW, aorta and MPA were separated, while
the RPA was end-to-side anastomosed to the MPA. A bovine pericardial
patch was used, and the incision in the aorta was closed with 6-0
prolene sutures in a continuous manner. Intraoperative transesophageal
echocardiography confirmed continuous flow through the aortic arch and
no shunting between the MPA and the aorta. The patient received further
treatment in the ICU postoperatively and was transferred to a regular
ward after 5 days. A successful evaluation was conducted, and the
patient was discharged 7 days later.