Universidad Médica Pinareña (Jan 2019)
Congenital heart disease and inverse situs as a prenatal expression of Ivemark Syndrome
Abstract
Introduction: Ivemark Syndrome is a rare congenital condition that affects multiple organs of the body; it is classified as a disorder of heterotaxiaor laterally disorder. It is characterized by anomalies of the spleen and other abdominal organs as well as malformations of the heart. Case report: a 15-month-old boy who underwent fetal echocardiography in the 28th week of his fetal development, in the left heart cavity an axis of more than 650 with increase of right cavities, at the aortic valve level a slight decrease in its caliber was observed. Diagnostic impressions are presented as inverse situs, interruption of inferior vena cava and continuation with azygos vein, aortic valvular stenosis and left isomerism. Cesarean delivery was performed at 39 weeks of pregnancy, weight 5080 gr, apgar scoring 9-9. In the abdominal ultrasound the spleen was not defined, the right kidney was malrotated observing also intestinal malrotation. Ivemark Syndrome was the definitive diagnosis. The infant is periodically monitored by a multidisciplinary medical team. Conclusions: at present, it is possible to formulate an early diagnosis of the cardinal signs that involve internal organs such as cardiovascular system, liver, and spleen during prenatal stage to confirm the Ivemark Syndrome, which contributes to the genetic counseling and the multidisciplinary approach in the neonatal stage, avoiding fatal complications and therefore to increase in quality and life expectancy.
