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A Rare Case Report of Thoracic Ectopia Cordis: An Obstetrician's Point of View in Multidisciplinary Approach

View Article: PubMed Central - PubMed

ABSTRACT

Ectopia cordis is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. It can be associated with other congenital abnormalities. Overall, the prognosis for infants with ectopia cordis is very poor but depends greatly on the type and severity of ectopia cordis and intracardiac and associated malformations. We present one case of a fetus with prenatally diagnosed thoracic ectopia cordis with intracardiac defects and omphalocele, all the abnormalities seen in pentalogy of Cantrell except a diaphragmatic defect. Considering poor prognosis for fetus, conservative management of prenatal care has been chosen. At the 42nd gestational week, during the active stage of labor, due to fetal distress, cesarean section was performed at a tertiary level hospital. The condition of the infant was impairing rapidly and the newborn succumbed within 24 hours. We discuss the perinatal care concerning this rare anomaly.

No MeSH data available.


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The newborn 5 minutes after birth. C: the ectopic heart positioned outside the thoracic cavity; F: supraumbilical omphalocele.
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fig3: The newborn 5 minutes after birth. C: the ectopic heart positioned outside the thoracic cavity; F: supraumbilical omphalocele.

Mentions: The woman was admitted to the tertiary level obstetrics and gynecology clinic during active stage of labor, at gestational age of 42 weeks. Diagnosis of congenital heart anomaly was heart ectopia, and septal ventricular defect was confirmed by ultrasound. Multiple anomalies were observed: wide anterior thoracic defect with extrathoracic four-chamber heart, rounded apex of the heart, high ventricular septal defect, the major blood vessels transposition, narrow pulmonary artery, and pericardium covering only ventricles (Figures 1 and 2). Despite unfavorable prognosis to the fetus, mother had chosen intrapartum fetal heart monitoring. Due to fetal distress at a cervical dilation of 6 cm, cesarean section was performed. The newborn was a female of 3300 g weight and 44 cm height who scored 8 (1 min) and 8 (5 min) on Apgar scale (Figure 3, Supporting Information Video 1 in Supplementary Material available online at http://dx.doi.org/10.1155/2016/5097059). At birth, the infant had hypotonia, weak cry, and generalized facial cyanosis. The physical examination revealed split sternum with complete thoracic ectopia cordis, the defect followed by anterior abdominal wall defect, supraumbilical omphalocele. Ectopic heart with partial absence of the pericardium was beating outside the thoracic cavity, at a rate of 130/min with remittent bradycardia. After birth, the infant's heart was covered with warm saline-soaked sterile dressing. The newborn girl was transferred to the specialized cardiac surgery centre, children's intensive care unit. She died within 24 hours. The parents declined postmortem newborn's autopsy.


A Rare Case Report of Thoracic Ectopia Cordis: An Obstetrician's Point of View in Multidisciplinary Approach
The newborn 5 minutes after birth. C: the ectopic heart positioned outside the thoracic cavity; F: supraumbilical omphalocele.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC5120199&req=5

fig3: The newborn 5 minutes after birth. C: the ectopic heart positioned outside the thoracic cavity; F: supraumbilical omphalocele.
Mentions: The woman was admitted to the tertiary level obstetrics and gynecology clinic during active stage of labor, at gestational age of 42 weeks. Diagnosis of congenital heart anomaly was heart ectopia, and septal ventricular defect was confirmed by ultrasound. Multiple anomalies were observed: wide anterior thoracic defect with extrathoracic four-chamber heart, rounded apex of the heart, high ventricular septal defect, the major blood vessels transposition, narrow pulmonary artery, and pericardium covering only ventricles (Figures 1 and 2). Despite unfavorable prognosis to the fetus, mother had chosen intrapartum fetal heart monitoring. Due to fetal distress at a cervical dilation of 6 cm, cesarean section was performed. The newborn was a female of 3300 g weight and 44 cm height who scored 8 (1 min) and 8 (5 min) on Apgar scale (Figure 3, Supporting Information Video 1 in Supplementary Material available online at http://dx.doi.org/10.1155/2016/5097059). At birth, the infant had hypotonia, weak cry, and generalized facial cyanosis. The physical examination revealed split sternum with complete thoracic ectopia cordis, the defect followed by anterior abdominal wall defect, supraumbilical omphalocele. Ectopic heart with partial absence of the pericardium was beating outside the thoracic cavity, at a rate of 130/min with remittent bradycardia. After birth, the infant's heart was covered with warm saline-soaked sterile dressing. The newborn girl was transferred to the specialized cardiac surgery centre, children's intensive care unit. She died within 24 hours. The parents declined postmortem newborn's autopsy.

View Article: PubMed Central - PubMed

ABSTRACT

Ectopia cordis is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. It can be associated with other congenital abnormalities. Overall, the prognosis for infants with ectopia cordis is very poor but depends greatly on the type and severity of ectopia cordis and intracardiac and associated malformations. We present one case of a fetus with prenatally diagnosed thoracic ectopia cordis with intracardiac defects and omphalocele, all the abnormalities seen in pentalogy of Cantrell except a diaphragmatic defect. Considering poor prognosis for fetus, conservative management of prenatal care has been chosen. At the 42nd gestational week, during the active stage of labor, due to fetal distress, cesarean section was performed at a tertiary level hospital. The condition of the infant was impairing rapidly and the newborn succumbed within 24 hours. We discuss the perinatal care concerning this rare anomaly.

No MeSH data available.


Related in: MedlinePlus