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PubMedDate not supplied

Fetal echocardiography in ectopia cordis.

Ectopia cordis is an extremely rare congenital abnormality occurring in 5.5 to 7.9 per 1 million live births with high lethality. Between January 1995 and October 1997 eight cases of ectopia cordis were diagnosed at our institute before birth. On the basis of echocardiography the fetal heart anatomy was categorized as either normal heart anatomy (NHA; n = 3) or congenital heart defect (CHD; n = 5). In the majority of cases (seven of eight) other abnormalities were present. Some reports have described ectopia cordis being diagnosed in the first trimester of pregnancy. In our study group the average gestational age at diagnosis was 26 weeks. The prenatal diagnosis of isolated ectopia cordis is easy; counseling the patient, the perinatal management including term, place, and method of delivery, and optimal care of the newborn are more difficult. Ectopia cordis is a malformation that pediatricians rarely encounter, even at pediatric cardiology centers. Much more frequently it is a problem for sonographers and obstetricians; however, pediatric cardiologists should be aware of diagnostic algorithm for such cases, especially when additional abnormalities are present.

Abnormalities, Multiple↗
PubMed2005

Prenatal 2-dimensional and 3-dimensional ultrasonography diagnosis and autoptic findings of isolated ectopia cordis.

Ectopia cordis is a very rare congenital malformation, commonly associated with intracardiac anomalies. It is due to a defect in fusion of the anterior chest wall resulting in an extrathoracic location of the heart. We report prenatal 2-dimensional (2D) and 3D ultrasonography diagnosis and postnatal autoptic findings of an isolated ectopia cordis with tricuspid atresia. Ectopia cordis prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. 3D ultrasonography may add more detailed visualization of the heart anomaly even if the 2D ultrasonography alone permits the prenatal diagnosis. Obstetrical management should include a careful search for associated anomalies, especially cardiac, and the assessment of fetal karyotype. As this is considered a sporadic anomaly, the recurrence risk is low and no genetic origin is known.

Adult↗
PubMed2001

Successful staged-Fontan operation in a patient with ectopia cordis.

Ectopia cordis is a very rare and often fatal disease. We report a successful surgery case of thoracoabdominal ectopia cordis with univentricular heart. This patient underwent a three-stage Fontan procedure, a right-modified Blalock-Taussig shunt at the age of 1 month, bidirectional Glenn shunt and pulmonary arterioplasty at 2 years 8 months, and finally a total cavopulmonary connection at 4 years. This patient was discharged from the hospital in good condition and has been doing well since. Thus, ectopia cordis is not a contraindication for a Fontan operation.

Child, Preschool↗
PubMed1991

Prenatal sonographic diagnosis and perinatal management of ectopia cordis.

Ectopia cordis is a rare malformation presenting as an isolated lesion or as part of the Cantrell's pentology syndrome. Prenatal ultrasonographic diagnosis of ectopia cordis must be followed by a careful search for associated anomalies, since the prognosis may vary accordingly. A case of prenatal diagnosis of ectopia cordis in the early second trimester is presented. Routine sonographic examination at 18 weeks of gestation revealed protrusion of the heart into the amniotic fluid, ventricular septal defect and omphalocele. The perinatal evaluation and management are discussed.

Journal Article↗
PubMed1981

Ectopia cordis.

Ectopia cordis is a rare congenital anomaly with an unknown incidence. The heart lies totally or partially outside the thoracic cage. Sternal cleft without cardiac protrusion outside the thorax is not included. The condition is often associated with various cardiac and extracardiac anomalies. During the period 1968-1978 three cases of thoracic total ectopia cordis were encountered. Surgical correction was unsuccessful. This has been the outcome in all but one case of thoracic forms of total ectopia cordis reported earlier in the literature.

Heart Defects, Congenital↗
PubMed2000

Ectopia cordis.

Ectopia cordis is a rare congenital defect in which the heart is placed externally on the surface of the chest. This article describes the embrylogic events that lead to the various classifications of the defect and how they possibly explain the process of its unusual occurrence in children. While survival in some cases is possible, the ultimate repair is difficult and survival is rare. The most extreme forms of ectopia cordis, especially those with intracardiac defects, have a poor prognosis. Several cases of this rare anomaly with primary repair and staged repair are discussed.

Cardiac Surgical Procedures↗
PubMed1982

Staged repair of ectopia cordis.

Ectopia cordis is a challenging congenital anomaly that generally has defied repair because of the magnitude of the deformity and the associated defects, particularly the intracardiac anomalies. We were fortunate to be presented with a boy who had successfully undergone an operation on the day of his birth to provide skin coverage over his bare ectopic heart. Subsequent studies disclosed no intracardiac anomalies. This paper describes the second stage of his repair designed to place the heart within the thorax and construct a bony shield anterior to it.

Follow-Up Studies↗
PubMed1999

Presentation and outcomes of ectopia cordis.

BACKGROUND: Ectopia cordis, defined as complete or partial displacement of the heart outside the thoracic cavity, is rare. The cause and pathogenesis are unknown. The anatomy of the heart may be normal, but the presence of structural heart disease as well as noncardiac malformations is common. Patients present incidentally after birth or, more recently, the condition has been diagnosed by prenatal echocardiography. OBJECTIVE: To review the presentation, diagnostic imaging, management and outcomes of infants with ectopia cordis at The Hospital for Sick Children, Toronto, Ontario. METHODS: The medical records of the database of the Division of Cardiology, The Hospital for Sick Children, were searched; echocardiograms, and surgical and autopsy reports were further reviewed. RESULTS: Between 1978 and 1998, 10 patients with ectopia cordis presented to the authors' centre. Normal cardiac anatomy was noted in three, and mild to complex structural cardiac anomalies were noted in the remainder. Associated noncardiac malformations were found in six cases. Six were diagnosed by prenatal ultrasound between 19 and 37 weeks of gestation. Pregnancies were electively terminated or the infants died shortly after birth. Four other neonates with ectopia cordis were referred to the authors' centre. All of these infants subsequently died either at the time of surgery for cardiac and noncardiac anomalies or in the immediate postoperative period. CONCLUSIONS: Despite modern treatment options, the mortality remains high for patients with ectopia cordis, especially in the presence of structural heart disease or other malformations.

Abnormalities, Multiple↗
PubMed2003

Complete thoracic ectopia cordis.

Thoracic ectopia cordis is a rare congenital defect with very few reported survivors after surgical correction. We report a case of complete thoracic ectopia cordis with double outlet right ventricle. The diagnosis was established antenatally and a repair was undertaken soon after birth. The child remained stable and was extubated on the fifth post-operative day. Forty-eight hours later the child succumbed to an unexplained respiratory arrest. Also presented is a review of the different surgical strategies for this unusual condition.

Cardiac Surgical Procedures↗
PubMed1995

Single-stage repair of thoracic ectopia cordis.

Thoracic ectopia cordis is a rare congenital defect most often seen in association with sternal and congenital heart defects. Surgical correction of these defects is complex and generally requires a staged closure including (1) coverage of the "naked heart," (2) placement of the heart into the thoracic cavity, and (3) sternal or thoracic reconstruction. Survival past the perioperative period is rare, with only 2 reported cases in the English-language literature. As with our case, neither had any discernable intracardiac defect. We present a case report of a patient with thoracic ectopia cordis repaired in a single stage using polytetrafluoroethylene membrane and skin for coverage and closure of the heart and thoracic defect.

Female↗
PubMed2000

Ectopia cordis and other midline defects.

BACKGROUND: Thoracic ectopia cordis and other midline defects are rare congenital anomalies that often occur with other intracardiac defects. Despite significant improvements in neonatal and infant cardiac surgeries, operations for thoracoabdominal ectopia cordis carry an extremely high mortality with only a few reported survivors of thoracic ectopia cordis. METHODS: The clinical charts of 4 patients with ectopia cordis over a 6-year period were reviewed. Three of the patients showed varying degrees of Cantrell's Pentalogy; thoracic ectopia cordis was found in 1. We have reviewed our surgical strategies and reported the patients' clinical outcomes. RESULTS: All 4 patients are alive at follow-up. Two infants with double-outlet right ventricle have been fully corrected, and extracorporeal membrane oxygenation was necessary in 1 infant for cardiac failure following the cardiac repair. A newborn with thoracoabdominal ectopia cordis underwent primary repair of his diaphragmatic defect, and a silo was used to progressively reduce the omphalocele. He is currently awaiting elective repair of tetralogy of Fallot. Lastly, the patient with thoracic ectopia cordis underwent successful soft tissue coverage, and she is being followed in the clinic with restrictive muscular ventricular septal defects and a left ventricular diverticulum. CONCLUSIONS: Our experience along with other reports in the literature demonstrates that patients with thoracic and thoracoabdominal ectopia cordis can undergo and survive full cardiac, neurologic, and abdominal repair during infancy. Furthermore we advocate different approaches determined by the severity of the presentation and the presence of other complicating factors.

Female↗
PubMed2002

[Ectopia cordis and cardiac anomalies].

Ectopia cordis is a rare disease that occurs in 5.5 to 7.9 per million live births. Only 267 cases had been reported as of 2001, most (95%) associated with other cardiac anomalies. We studied the cardiac malformations associated in 6 patients with ectopia cordis. Depending on where the defect was located, the cases of ectopia were classified into four groups: cervical, thoracic, thoraco-abdominal, and abdominal. All 6 patients died before the third day of life, 4 during delivery. Three of the patients were included in the thoracic group, whereas the other 3 belonged to the thoraco-abdominal group. All the patients had associated ventricular septal defects, 3 double-outlet right ventricle (50%) and the rest (50%) tetralogy of Fallot-pulmonary atresia. Two patients with double-outlet right ventricle presented mitral-valve pathology, a parachute valve and an atresic mitral valve. None of these cardiac anomalies have been reported to date.

Abnormalities, Multiple↗
PubMed1997

Successful repair of ectopia cordis using alloplastic materials.

Ectopia cordis is a very rare congenital anomaly associated with a high mortality rate. A successful repair of ectopia cordis with complete absence of sternum was achieved in a two-stage procedure. Initial management consisted of coverage of skin over the malpositioned heart using bilateral pectoral skin flaps. A second more definitive repair was undertaken at age 14 months. Four methyl methacrylate struts were used to reconstruct the anterior chest wall and were then covered with bilateral pectoralis major muscle flaps. At the 2.5-year follow-up there is no evidence of cardiopulmonary compromise and the development of the thorax appears normal. We advise that use of alloplastic materials is a valid option in managing this difficult congenital anomaly.

Bone Cements↗
PubMed2006

[Ectopia cordis: multidisciplinary approach with successful result].

Ectopia cordis is a pathology in which the heart is complete or partially outside of the thoracic cavity. It represents a challenge for the diagnosis and handling because of its high perinatal mortality. We present the first case of thoracic ectopia cordis with prenatal diagnostic (27th week), its multidisciplinary handling at Hospital de Ginecopediatria núm. 71 of the Centro Medico Nacional Adolfo Ruiz Cortines, IMSS, in Veracruz, Mexico. We communicate the case of a 24 year-old woman referred by her correspondent medical unit with the diagnosis of 23.6 weeks pregnancy with suspicion of heart malformation by ultrasound. The pregnancy was followed-up until the 38th week, when it was programmed a caesarean section. The newborn had ectopia cordis, which was treated at the moment of birth. In this article, we present pictures of the heart defect as well as its evolution. The up-to-date bibliography is revised about the medical profile and treatment. The handling of the ectopia cordis includes an appropriate prenatal diagnosis through echocardiograph, a multidisciplinary perinatal team, to program the caesarean operation, aseptic handling of the newborn, immediate correction of the wall defect with skin torn piece without trying to correct the costal grill, specific hemodynamic cares, to reprogramme a correction of associated defects.

Adult↗
PubMed1999

Prenatal sonographic diagnosis of ectopia cordis.

PURPOSE: We present a small series of prenatally diagnosed cases of ectopia cordis. METHODS: Four fetuses with prenatally diagnosed ectopia cordis were sonographically evaluated and followed up. RESULTS: The fetuses were diagnosed with ectopia cordis at 9, 13, 21, and 29 weeks' menstrual age. The case diagnosed at 9 weeks is the earliest prenatal diagnosis reported, to our knowledge. The diagnoses were based on the demonstration of a fetal heart outside the thoracic cavity with Doppler waveforms typical of intracardiac flow. One fetus had isolated ectopia cordis, and 3 had other associated anolmalies. The diagnosis was postnatally confirmed in all cases. Therapeutic abortion was done in 2 cases. One infant survived, and the fourth died shortly after birth. Chromosome study was successfully performed in 2 cases and was normal in both of them. CONCLUSIONS: This small series suggests that ectopia cordis can be readily diagnosed in utero as early as the first trimester. Later in pregnancy, sonography provides important information for planning surgical correction.

Adult↗
PubMed2004

Ectopia cordis: report of two cases.

Ectopia Cordis is a dramatic but rare cardiac anomaly with poor prognosis in most centers worldwide. This rare malformation occurs in 5.5-7.9 per million live births in the USA(1). This cardiac malformation is defined as a heart that is in an extra- thoracic position. In this article, we report two newborn infants admitted to our newborn unit with the heart beating outside the thoracic cavity. One child succumbed due to prematurity and severe cardiac malformations, while the other child successfully underwent surgical reconstruction of the ectopia cordis. She succumbed due to overwhelming sepsis one week after the surgery. Such cases have neither been reported nor treated locally.

Fatal Outcome↗
PubMed1999

Morphological aspects of ectopia cordis: four case reports and a review of the literature.

Ectopia cordis is a rare congenital anomaly. We present 4 cases of ectopia cordis, 1 of which is the first report of an affected fetus in a triplet pregnancy. The morphological relationship between the types of ectopia cordis and their outcomes were investigated in all 4 cases. In addition, the literature on ectopia cordis in Japan was reviewed and discussed.

Adult↗
PubMed1990

Ectopia cordis in the chick embryo heart: an experimental study.

Ectopia cordis was observed during a study on the effects of a calcium antagonist (verapamil) on chicken embryo heart development. Experimental procedures, carried out at 60 hours of incubation, included placement of windows in eggs, injection of verapamil or saline, and the removal of ovalbumen from eggs. Fluid removal caused a downward displacement of the embryo and helped separate the embryo and its membranes from the shell membranes. Ectopia cordis was only observed in experiments involving fluid removal. One exception, the appearance of one ectopic heart in a window-only experiment (no fluid removed), remains unexplained. The movements of fluids, brought about by the withdrawal of ovalbumen from eggs, and the subsequent effects of such movements on the positioning of embryos seemed to be the most important factor in the genesis of ectopia cordis. Also observed in fluid removal experiments were asymmetric circulations, abnormal embryonic flexion, and several embryos whose positions were rotated. These abnormalities were probably related to fluid removal and/or ectopia cordis.

Animals↗