A Case of Berry Syndrome Type 2B

Abstract

We present a case of a 34-day-old baby boy with congestive heart failure and differential cyanosis. Based on echocardiography and confirmed with computed tomography angiography, the following abnormalities were detected: aortopulmonary window, aortic origin of the right pulmonary artery, patent ductus arteriosus, and hypoplasia/coarctation of the aorta. No other congenital abnormalities were detected, and a diagnosis of Berry Syndrome type 2B was made. No preoperative cardiac catheterization or angiocardiography was needed. The patient underwent complete surgical repair of the aortopulmonary window and reconstruction of the hypoplastic aortic arch. This case highlights the value of cross-sectional imaging with computed tomography angiography in the evaluation of complex congenital heart disease.

Current outcomes of one-stage surgical correction for Berry syndrome

“…The literature consists of anecdotal case studies performed by different surgeons throughout the world (Table 2). [2][3][4][5][8][9][10][11][12][13][14][15][16][17] Most centers advocate one-stage surgery in the neonatal period, although Ghelani and colleagues 5 suggested that a staged repair should be considered in premature infants or small-forgestational-age infants. We also recommend surgical correction should be performed as early as possible.…”

Nuclear medicine and the emergency department patient: an illustrative case-based approach

“…Imaging confirmation of brain death is usually sought to accelerate this process. A scintigraphic brain death study is noninvasive, simple to perform even in the intensive care unit (ICU) setting (portable) and reliable, making it the modality of choice to establish imaging evidence supporting the clinical diagnosis of brain death [14][15][16][17]. From a clinical standpoint, this diagnosis requires the patient to be in a deep coma, with total absence of brainstem reflexes or spontaneous respiration.…”

Berry syndrome: One stage surgical repair in a neonate

“…Patients with Berry syndrome present with respiratory distress, metabolic acidosis, anuria, severe congestive heart failure or acute cardiovascular collapse with spontaneous closure of ductus arteriosus in the first few days of life. Early clinical diagnosis with detailed preoperative recognition of these unique anatomic features is therefore essential for good surgical outcomes [2,3,[5][6][7]. Long-term follow-up is required after surgical correction as stenosis at the site of anastomosis is a potential complication.…”

“…Aortopulmonary window has been classified based on the location of the septal defect [1,2]. Type I comprises proximal communication with normal origin of pulmonary arteries.…”