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.