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.