Ectopia cordis
Updates to Article Attributes
Ectopia cordis is an extremely rare congenital malformation where the heart is located partially or totally outside the thoracic cavity.
The four main ectopic positions includeare::
- adjacent to the thorax: ~60 %
- abdominal: 15-30%
- thoraco-abdominal: 7-18%
- cervical: ~3%
Epidemiology
The estimated prevalence is 1:126,000 births. There may be a slight female preponderance ref.
Pathology
It results from the failure of migration of lateral mesoderm into the midline.
Ectopia cordis may occur as an isolated malformation or it may be associated with a larger category of ventral body wall defects that affect the thorax, abdomen or both.
Associations
Individual associations
Ventricular septal defect and tetralogy of Fallot are the most common intracardiac defects.
Omphalocele and is the most common abdominal wall defect.omphalocele,
Syndromic associations
A well-known association is pentalogy of Cantrell 1 which comprises of:
- ectopia cordis
- omphalocele (typically supraumbilical)
- congenital diaphragmatic hernia
- sternal cleft
- congenital heart disease
Radiographic features
Antenatal ultrasound
When in isolation, the heart is seen in the amniotic cavity with a thoracic wall defect. If in association with pentalogy of Cantrell it may seen within an omphalocoele 2.
Plain radiograph
-
imagingImaging clues on frontal chest radiograph
- abnormal cardiac position and configuration
- air lucency may surround the ectopic heart
- sternal defect is often present
- wide separation of the sternal ends of the clavicles
- widening of the superior mediastinum
The lateral view may confirm the extrathoracic location of the heart
Treatment and prognosis
The prognosis is generally poor and depends on the severity of intracardiac malformations and the presence of associated abnormalities. Most infants are stillborn or die within the first hours or days of life.
-<p><strong>Ectopia cordis</strong> is an extremely rare congenital malformation where the <a href="/articles/heart">heart</a> is located partially or totally outside the <a href="/articles/thoracic-cavity">thoracic cavity</a>. </p><p>The four main ectopic positions include:</p><ul>- +<p><strong>Ectopia cordis</strong> is an extremely rare congenital malformation where the <a href="/articles/heart">heart</a> is located partially or totally outside the <a href="/articles/thoracic-cavity">thoracic cavity</a>. The four main ectopic positions are::</p><ul>
-</ul><h4>Epidemiology</h4><p>The estimated prevalence is 1:126,000 births. There may be a slight female preponderance <sup>ref</sup>.</p><h4>Pathology</h4><p>It results from the failure of migration of lateral mesoderm into the midline.</p><p>Ectopia cordis may occur as an isolated malformation or it may be associated with a larger category of ventral body wall defects that affect the thorax, abdomen or both.</p><h5>Associations</h5><h6>Individual associations</h6><p><a href="/articles/ventricular-septal-defect-1">Ventricular septal defect</a> and <a href="/articles/tetralogy-of-fallot">tetralogy of Fallot</a> are the most common <a href="/articles/intracardiac-defect">intracardiac defects</a> and <a href="/articles/omphalocoele">omphalocele</a>, the most common <a href="/articles/abdominal-wall-defect">abdominal wall defect</a>.</p><h6>Syndromic associations</h6><p>A well-known association is <a href="/articles/pentalogy-of-cantrell-3">pentalogy of Cantrell</a> <sup>1</sup> which comprises of:</p><ul>- +</ul><h4>Epidemiology</h4><p>The estimated prevalence is 1:126,000 births. There may be a slight female preponderance <sup>ref</sup>.</p><h4>Pathology</h4><p>It results from the failure of migration of lateral mesoderm into the midline.</p><p>Ectopia cordis may occur as an isolated malformation or it may be associated with a larger category of ventral body wall defects that affect the thorax, abdomen or both.</p><h5>Associations</h5><h6>Individual associations</h6><p><a href="/articles/ventricular-septal-defect-1">Ventricular septal defect</a> and <a href="/articles/tetralogy-of-fallot">tetralogy of Fallot</a> are the most common <a href="/articles/intracardiac-defect">intracardiac defects.</a></p><p><a href="/articles/omphalocoele">Omphalocele</a> is the most common <a href="/articles/abdominal-wall-defect">abdominal wall defect</a>.</p><h6>Syndromic associations</h6><p>A well-known association is <a href="/articles/pentalogy-of-cantrell-3">pentalogy of Cantrell</a> <sup>1</sup> which comprises of:</p><ul>
-</ul><h4>Radiographic features</h4><h5>Antenatal ultrasound</h5><p>When in isolation, the heart is seen in the amniotic cavity with a thoracic wall defect. If in association with pentalogy of Cantrell it may seen within an omphalocoele <sup>2</sup>.</p><h5>Plain radiograph</h5><ul>-<li>imaging clues on frontal chest radiograph<ul>- +</ul><h4>Radiographic features</h4><h5>Antenatal ultrasound</h5><p>When in isolation, the heart is seen in the amniotic cavity with a thoracic wall defect. If in association with pentalogy of Cantrell it may seen within an omphalocoele <sup>2</sup>.</p><h5>Plain radiograph</h5><p>Imaging clues on frontal chest radiograph</p><ul>
-</ul>-</li>-<li>lateral view may confirm the extrathoracic location of the heart</li>-</ul><h4>Treatment and prognosis</h4><p>The prognosis is generally poor and depends on the severity of intracardiac malformations and the presence of associated abnormalities. Most infants are stillborn or die within the first hours or days of life.</p>- +</ul><p>The lateral view may confirm the extrathoracic location of the heart.</p><h4>Treatment and prognosis</h4><p>The prognosis is generally poor and depends on the severity of intracardiac malformations and the presence of associated abnormalities. Most infants are stillborn or die within the first hours or days of life.</p>