Successful surgical repair of berry syndrome with severe biventricular dysfunction

Abstract

Background - Absence of the aortopulmonary septum with the presence of two separate semilunar valves, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus is a rarely reported association known as Berry syndrome. This abnormal right pulmonary arterial origin may lead to "steal" from the aortic flow during embryogenesis and cause hypoplasia of the aortic arch. Most patients present in infancy or earlier with symptoms of cardiac failure. Scattered reports in the literature confirm the possibility of surgical correction of this complex anomaly. Case Report – We report a case of 7 week old cyanotic child who presented in emergency with features of congestive cardiac failure. Two dimensional echocardiography and CT scan confirmed the diagnosis of interrupted aortic arch with large aorto-pulmonary window. Single stage repair of the defect was done with the use of PTFE patch and autologous pericardium. Conclusion – Berry Syndrome is a rare congenital cardiac anomaly with patients presenting early in infancy with congestive cardiac failure. Early single stage correction of the defect is indicated. Different surgical options have been tried but we used an intra aortic PTFE patch to baffle the RPA towards MPA without detaching the RPA and an autologous pericardium to augment the aorta and it translated into a shorter clamp time.