Thoracic ectopia cordis (naked heart).

To the editor: We read with interest the case report and its successful surgical treatment in the Annals of Saudi Medicine by Mohammed S. Kabbani on thoracic ectopia cordis (EC).1 We report another case of EC or the so-called naked heart with another severe congenital heart anomaly other than its position.

An indicated abortion in the 21st gestation week was done for a unipara woman, because of detection by ultrasonography of a naked heart and another nonspecific congenital vitium cordis in her 18th gestation week. A 340-g and 27-cm male presented with the thoracic type of EC, the heart was completely extrathoracic and penduculated by the heart vessels with an orientation of the heart apex toward the chin. The parietal pericardium was completely absent. A defect of the distal sternum with an epigastric omphalocele was present. The diaphragm was intact.

The heart was 28 × 16 × 13 mm with an anatomical insertion of the heart vessels. The right atrium was dilated with an atrioventricular congenital sulcus connection to the rudimentary right ventricle; a real atrioventricular connection was absent. At the side of the sulcus a droplet dimple was completely covered by muscles and directed toward the posterior part of the intraventricular septum. The dominant left ventricle with macroscopic architecture communicated to a rudimentary right ventricle via a 2-mm defect throughout the muscular part of the intraventricular septum. An infundibular stenosis, a small pulmonary orifice with pulmonary trunk stenosis, an atrial septal defect (fossa ovalis type) and agenesis of ductus arteriosus were further anomalies. Body organs were without macroscopic or histo-pathological findings, except for lung hypoplasia. Genetic examination was of normal karyotype (46, XY). The placenta was 11 × 10 × 2 cm with eccentric insertion of the umbilical cord. Amniotic membranes and amniotic fluid were without pathological findings.

EC is a rare congenital malformation of the heart position in which the heart is partially or totally outside the thorax. EC was first reported in 1671 by Stensen.2 The thoracic and thoracoabdominal types are the most common.1 Etiopathogenesis of the EC is not completely understood.3,4 Thoracic EC can be classified as a) a separated EC without another cardiac anomaly, b) as an intracardiac malformation (as interatrial or interventricular spetal defects, cor triloculare, cor biloculare, tricuspidal atresia, Fallot tetralogy and others), or c) EC with another somatic malformation.4–6 The ultrasonographic examination is the procedure of choice in the prenatal period.3,8 The naked heart in the majority of cases can be diagnosed in the 2nd trimester, but can also be diagnosed in the first trimester.2,9,7 In our case diagnosis of EC with another severe intracardiac anomaly was done in the 18th of gestation week, so the pregnancy was interrupted artificially. The prenatal diagnosis of EC is easy, but the decision to interrupt artificially or to continue in pregnancy is challenging and depends on the relatives’ decision and the regional cultural and religious roles. We believe that if EC is present without another severe cardiac congenital anomaly, it can be successfully treated surgically. However the prognosis of EC is also challenging without surgical intervention because such a naked heart can be a risk for serious infection.