ABSTRACT
Objective To explore the value of transthoracic echocardiography in diagnosis of Berry syndrome and analyze the sonographic typing.Methods The echocardiographic results of 6 Berry patients were reviewed retrospectively.Their sonographic typing were also analyzed connected with the reports in the literature.Results The ultrasound diagnosis of 5cases were confirmed by surgery(3)or angiography(3) and the remain one infant was died 14 days after the echocardiographic procedure.The malformations of Berry syndrome consisted of the distal aorto-pulmonary septal defect(APSD),aortic origin of the right pulmonary artery(RPA),interruption of the aortic arch(5 cases,type A with patent ductus arteriosus)or coarctation of the aortic isthmus(1 case)with intact ventricular septum.In 6 cases,there were 2 sonographic types:(1)Type A(3 cases),the aorto-pulmonary septum was almost absent and the left and right pulmonary arteries remained widely separated,the blood of the RPA came almost from the aorta,the aortic arch was interrupted.(2)Type B(3 cases),the left and right pulmonary arteries remained adjacent and the bifurcation straddled the distal APSD,the blood of the RPA came from the aorta and the pulmonary trunk separately,the aortic arch was coarctated or interrupted. Conclusions The transthoracic echocardiography can be used to evaluate all containing malformations of Berry syndrome and it may be the first diagnostic choice.
ABSTRACT
Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.
Subject(s)
Humans , Infant, Newborn , Angioplasty , Aorta , Constriction, Pathologic , Ductus Arteriosus, Patent , Fruit , Pulmonary Artery , Stress, Psychological , Ventricular SeptumABSTRACT
Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.
Subject(s)
Humans , Infant, Newborn , Aorta, Thoracic , Fruit , Heart Defects, Congenital , Pulmonary ArteryABSTRACT
Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.
Subject(s)
Humans , Infant, Newborn , Aorta, Thoracic , Fruit , Heart Defects, Congenital , Pulmonary ArteryABSTRACT
Berry syndrome is a rare association of congenital heart anomalies which consists of a distal aortopulmonary window with aortic origin of the right pulmonary artery and hypoplasia or interruption of the aortic arch. This defect can be corrected only by immediate surgical intervention, so accurate preoperative diagnosis and detailed anatomic depiction of this syndrome are important in prognosis. We experienced a case of Berry syndrome in an 8-day-old male, who had presented with multiple malformation such as syndactyly, high arched palate and brain hemorrhage. The diagnosis was made by two-dimensional echocardiography. The case is presented with a brief review of related literatures.