EAP 2019 Congress and MasterCourse

A Case of Congenital Annular Pericardial Fibrous Band Causing Ventricular Strangulation in a Neonate

Hyo Ju Yang Juae Shin Ji Hong Yoon Jae Young Lee
Department of Pediatrics, Seoul St. Mary’s Hospital, College of Medicine, the Catholic University of Korea, South Korea

Congenital pericardial anomalies are very rare, and manifested as pericardial cyst, diverticulum and absence of pericardium. As an acquired and localized pericarditis manifested as a constrictive pericardial band causing strangulation and hourglass shaping of ventricles has been rarely reported in adolescents and adults, but this type of congenital pericardial anomaly has not been reported in neonates. We report a case of congenital pericardial fibrous band causing strangulation of both ventricles, diagnosed at fetal life and confirmed after birth. At 22 weeks of gestation, a fetal echocardiography showed an unusual ring-like constriction of both mid-ventricular cavities and multiple mass-like lesions in the both ventricular cavities, suggesting cardiac tumor. Both ventricular systolic and diastolic functions were within normal limits. After an uneventful pregnancy, the baby was born at 38+3 weeks of gestation with a birth weight of 3.3 kg. Echocardiography showed a pericardial fibrous band causing strangulation of both ventricles, extending from mid left ventricular (LV) lateral wall, diaphragmatic surface of both ventricles, lateral wall of right ventricle (RV) and into the RV outflow track. A three-dimensional cardiac computed tomography also showed the same findings. There was no definite cardiac tumor. The baby was asymptomatic and discharged home 4 days after birth. At 6 months of age, the baby was still asymptomatic, but echocardiography showed a merged pattern of mitral inflow Doppler and mitral annular tissue Doppler imaging, along with mild dilatation of both atria, suggesting an early stage of ventricular diastolic dysfunction. Close follow up is needed to assess ventricular functions and to decide necessity of any surgical intervention timely.









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