3|DISCUSSION
Berry syndrome is an extremely rare congenital condition, accounting for only 0.046% of reported cases of congenital heart structural abnormalities[6]. Patients are often diagnosed prenatally or in infancy, and may present with cyanosis, respiratory distress, respiratory tract infections, cardiac murmurs, heart failure, and other systemic symptoms[2]. Hu et al. suggested that patients should undergo immediate surgical intervention upon detection, even in the neonatal period[5], in order to avoid irreversible pulmonary hypertension and progressive right heart failure, which could have detrimental effects. Additionally, early surgical correction could restore normal blood supply to the lower body.
The currently widely accepted theory regarding the formation of Berry syndrome suggests that a significant shunt between the aorta and pulmonary artery leads to reduced blood flow in the aortic isthmus during fetal development[7]. Failure of the aortic septum results in an aortopulmonary septal defect, which can be located at the proximal or distal end of the septum. Failure of posterior truncal septation may disrupt normal blood flow and cause the pulmonary bifurcation to attach incorrectly to the unseptated truncus arteriosus instead of connecting to the main pulmonary artery. As a result, the RPA becomes connected to the aorta, while the LPA becomes connected to the pulmonary trunk[8]. According to research by Bi et al., the majority of Berry syndrome patients have type II APW and type A IAA[2].
Echocardiography is a commonly used method for diagnosing complex congenital heart diseases. As mentioned earlier, some patients with Berry syndrome can be diagnosed prenatally through echocardiography. However, these patients are often missed because they usually have a normal four-chamber heart and outflow tract appearance. The use of the three-vessel view (3VV) can improve the prenatal detection rate of Berry syndrome. Additionally, APW is typically easily detected by echocardiography, therefore, Zhang et al. suggested that when APW is detected, it is necessary to determine the origin of the RPA and the morphology of the aortic arch[6]. For postnatal infants, as these patients often exhibit easily noticeable symptoms, TTE has become the primary tool for evaluating Berry syndrome [4]. Computed tomography angiography (CTA) with three-dimensional reconstruction and cardiac magnetic resonance imaging (MRI) can provide comprehensive anatomical information in patients with Berry syndrome[9, 10].
Surgical treatment of Berry syndrome primarily focuses on repairing the anatomical abnormalities of APW and IAA. Currently, multiple surgical approaches have been discussed in various centers[3, 5, 11]. In general, the surgical process should aim to preserve the growth potential of the aorta and pulmonary arteries while achieving unobstructed left ventricular outflow and continuity of the aortic arch, ensuring no shunting between the main pulmonary arteries.