Prenatal Diagnosis and Management of Berry Syndrome, a Rare Conotruncal Anatomy

Abstract

Figure. A, Prenatal echocardiogram (Echo).Top, The aorta (Ao) and the main pulmonary artery (MPA) in the long-axis view with a large defect between them. Middle, Short-axis image showing again that the aorta and MPA are normally related to each other and that there is a large communication between the 2 great vessels. The left pulmonary artery (LPA) arises normally from the MPA, but the right pulmonary artery (RPA) arises from the aorta. Bottom, The MPA gives rise to the ductal arch and the LPA underneath. B, Postnatal echocardiogram images confirm the fetal diagnosis. Images are arranged side-by-side and in an orientation similar to that of the prenatal images. C, Part of the surgical repair included closure of the aortopulmonary window (APW) to permit communication between the MPA and RPA (longer dashed line) and aortic root enlargement with a patch (short dashed line). D, Postsurgery discharge echocardiogram. Top, Absence of residual aortopulmonary communication and mild RPA stenosis. Bottom, Unobstructed aortic arch (Arch). Duct indicates ductus arteriosus; and SVC, superior vena cava.

Prenatal diagnosis of Berry syndrome by fetal echocardiography: a case report

“…Since Matsubara et al . diagnosed a fetal Berry syndrome by echocardiography in 2010 for the first time, only a few reports about prenatal diagnosis of Berry syndrome have been published …”

“…Berry syndrome is an extremely rare disease of which the diagnosis at the fetal stage is also rare and complex. To date, only a few reports about prenatal diagnosis of Berry syndrome have been published . The difficulty in the prenatal diagnosis of Berry syndrome may be partly due to the normal four‐chamber view and normal ventricular outlets.…”

“…As described in our study, we found the distal aortopulmonary window and the origins of the left and right pulmonary arteries were separated apart in 3VT. The findings were similar to that of some previous studies . Because the branches of the pulmonary arteries were separated apart, the pulmonary artery pressure should be higher than the aortic pressure in fetus theoretically and a perfusion from MPA to AAO was supposed to be detected by color Doppler.…”

Berry syndrome: One stage surgical repair in a neonate

“…Two-stage repair involves reconstruction of aorta with ligation of patent ductus arteriosus followed by complete closure of aortopulmonary window in the next stage [11]. Ghelani et al advocate the utilization of two staged surgical repair in case of premature infants and small for gestational age infants [4]. However, one stage surgical repair is considered superior, the rate of reoperation varies with development of complications.…”

“…Prenatal diagnosis of this rare complex congenital anomaly is related with improved outcomes due to prompt initiation of prostaglandin infusion [4]. Detailed anatomic depiction using echocardiogram and other diagnostic modalities with early surgical repair is essential, thereby avoiding pulmonary hypertension associated with pulmonary over circulation and subsequent heart failure [5][6][7].…”