Sudden Demise in a 37-Week-Old Male Infant Due to D-Transposition of Great Vessels with a Closed Foramen Ovale
WA Kanner, MB Lopes, RD LeGallo. University of Virginia, Charlottesville, VA
Background: Congenital heart defects cause significant morbidity and mortality in the pediatric population. D- transposition of the great arteries (D-TGA) accounts for about 6% of all congenital heart diseases. By definition, the aorta originates from the right ventricle (RV) and the pulmonary artery (PA) arises from the left ventricle (LV). The systemic and pulmonic circulations run in parallel requiring atrial or ventricular communication in order for mixing of the blood. D-TGA is compatible with fetal life but may be life threatening after birth when the physiologic shunts (foramen ovale, ductus arteriosus) close. We report a case of D-TGA with an intact ventricular septum and a closed foramen ovale, presenting as cardiorespiratory failure shortly after birth.
Design: This male infant was born at 37 weeks gestation to a 31-year-old Caucasian female (G6 P4 A1) by elective Cesarean section. The prenatal history was unremarkable and a mid-trimester ultrasound failed to detect structural abnormalities. The infant initially cried, however APGAR was 2 at 5 minutes. The heart rate was below 100 and the baby was not pink. All attempts at resuscitation were unsuccessful. A chest x-ray showed complete opacity of the lungs. The baby was pronounced dead and permission was granted for a full autopsy.
Results: At autopsy the body weight and measurements were appropriate for gestational age and there were no dysmorphic features. The larynx and trachea were unobstructed. The lungs (91 grams) demonstrated diffuse pulmonary hemorrhage. Examination of the heart (26.12 grams) revealed that the aorta arose anterior and to the right of the PA. There was normal atrial situs and systemic venous return. The atrial septum showed a closed foramen ovale with ballooning of the septum into the left atrium. The unremarkable RV gave rise to the aorta via a muscular outflow tract. The coronary arteries arose in the usual anatomy. The pulmonary venous return was into the left atrium. The unremarkable LV gave rise to the PA through a membranous outflow tract. The ductus arteriosus was narrowly patent (2mm). All valves were normal appearing. Microscopic exam was unremarkable. Cytogenetics studies revealed a normal karyotype.
Conclusions: We present a case of D-TGA with an intact ventricular septum and closed foramen ovale, which is exceedingly rare with only three case reports identified in the literature. This is a lethal combination, and should be considered, among other congenital abnormalities in any sudden demise after birth.
Wednesday, March 24, 2010 9:30 AM
Poster Session V # 7, Wednesday Morning