Ectopia cordis
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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 include:
- adjacent to the thorax:
~ 60 - 64~60 % - abdominal:
~15- 30-30% - thoraco-abdominal:
~7- 18-18% - cervical:
~ 3~3%
Epidemiology
The estimated prevalence is 1 : 126:126,000 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 and omphalocele, the most common abdominal wall defect.
Syndromic associations
A well known association is a 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.
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" title="Heart">heart</a> is located partially or totally outside the <a href="/articles/thoracic-cavity" title="thoracic cavity">thoracic cavity</a>. </p><p>The four main ectopic positions include</p><ul>-<li>adjacent to the thorax : ~ 60 - 64 %</li>-<li>abdominal : ~ 15 - 30 %</li>-<li>thoraco-abdominal : ~ 7 - 18 % </li>-<li>cervical : ~ 3 %</li>-</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" title="Ventricular septal defect">Ventricular septal defect</a> and <a href="/articles/tetralogy-of-fallot" title="Tetralogy of Fallot">tetralogy of Fallot</a> are the most common <a href="/articles/intracardiac-defect" title="intracardiac defect">intracardiac defects</a> and <a href="/articles/omphalocoele" title="Omphalocele">omphalocele</a>, the most common <a href="/articles/abdominal-wall-defect" title="abdominal wall defect">abdominal wall defect</a>.</p><h6>Syndromic associations</h6><p>A well known association is a <a href="/articles/pentalogy-of-cantrell-3" title="Pentalogy of Cantrell" style="color: rgb(63, 117, 216); text-decoration: none; ">pentalogy of Cantrell</a> <sup>1</sup> which comprises of </p><ul>-<li><strong>ectopia cordis</strong></li>-<li>-<a href="/articles/omphalocoele" title="Omphalocele" style="color: rgb(63, 117, 216); text-decoration: none; ">omphalocele</a> (typically supraumbilical)</li>-<li><a href="/articles/congenital_diaphragmatic_hernia" title="Congenital diaphragmatic hernia" style="color: rgb(63, 117, 216); text-decoration: none; ">congenital diaphragmatic hernia</a></li>-<li>-<a href="/articles/congenital_diaphragmatic_hernia" title="Congenital diaphragmatic hernia" style="color: rgb(63, 117, 216); text-decoration: none; "></a><a href="/articles/sternal-cleft" title="sternal cleft" style="color: rgb(63, 117, 216); text-decoration: none; ">sternal cleft</a>-</li>-<li>-<a href="/articles/sternal-cleft" title="sternal cleft" style="color: rgb(63, 117, 216); text-decoration: none; "></a><a href="/articles/congenital-cardiovascular-anomalies" title="Congenital heart disease" style="color: rgb(63, 117, 216); text-decoration: none; ">congenital heart disease</a>-</li>-</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><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><br>- +<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>
- +<li>adjacent to the thorax: ~60 %</li>
- +<li>abdominal: 15-30%</li>
- +<li>thoraco-abdominal: 7-18% </li>
- +<li>cervical: ~3%</li>
- +</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>
- +<li>ectopia cordis</li>
- +<li>
- +<a href="/articles/omphalocoele">omphalocele</a> (typically supraumbilical)</li>
- +<li><a href="/articles/congenital-diaphragmatic-hernia-1">congenital diaphragmatic hernia</a></li>
- +<li><a href="/articles/sternal-cleft">sternal cleft</a></li>
- +<li><a href="/articles/congenital-cardiovascular-anomalies">congenital heart disease</a></li>
- +</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><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>
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