Ectopia cordis
Encyclopedia
Ectopia cordis is a birth defect in which the heart
is abnormally located. In the most common form, the heart protrudes outside the chest through a split sternum. Less often, the heart may be situated in the abdominal cavity
or neck
.
No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome
and Trisomy 18; however it does not appear to be an inherited disease.
The ectopic heart is not protected by the skin or sternum. Other organs may also have formed outside the skin. Often the heart is not formed properly and many other heart defects are associated with this condition including: Tetralogy of Fallot
, pulmonary atresia
, atrial and ventricular septal defect
s, double outlet right ventricle
. Other non-cardiac malformations may be present such as cleft palates, and spine malformations that can cause kyphosis
.
or death shortly after birth. Some cases of ectopia cordis can be treated surgically. Christopher Wall (19 August 1975) holds the Guinness World Record for the oldest living person with condition.
Doctors at AIIMS, New Delhi, India successfully operated on a 10-day-old male, who was born with this abnormality, on 2 September 2009. The doctors created a record by completing the surgery without suspending his bodily functions. However, the boy died at 22 days.
Heart
The heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
is abnormally located. In the most common form, the heart protrudes outside the chest through a split sternum. Less often, the heart may be situated in the abdominal cavity
Abdominal cavity
The abdominal cavity is the body cavity of the human body that holds the bulk of the viscera. It is located below the thoracic cavity, and above the pelvic cavity. Its dome-shaped roof is the thoracic diaphragm , and its oblique floor is the pelvic inlet...
or neck
Neck
The neck is the part of the body, on many terrestrial or secondarily aquatic vertebrates, that distinguishes the head from the torso or trunk. The adjective signifying "of the neck" is cervical .-Boner anatomy: The cervical spine:The cervical portion of the human spine comprises seven boney...
.
Classification
Depending on the position of the heart, ectopia cordis can be classified into four categories:- Cervical - in the neck
- Thoracic - where the heart would lie within the thoracic cavity.
- Thoracoabdominal - where the heart would lie somewhere between the thoracic and abdominal cavities.
- Abdominal - where the heart would lie in the abdominal cavity.
Cause
The occurrence of ectopic cordis is about 7.9 per million births. Ectopia cordis is a very rare congenital heart malformation. It is caused by a failure of the ventral body wall to close properly during embryonic development.No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome
Turner syndrome
Turner syndrome or Ullrich-Turner syndrome encompasses several conditions in human females, of which monosomy X is most common. It is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent...
and Trisomy 18; however it does not appear to be an inherited disease.
The ectopic heart is not protected by the skin or sternum. Other organs may also have formed outside the skin. Often the heart is not formed properly and many other heart defects are associated with this condition including: Tetralogy of Fallot
Tetralogy of Fallot
Tetralogy of Fallot is a congenital heart defect which is classically understood to involve four anatomical abnormalities...
, pulmonary atresia
Pulmonary atresia
Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right...
, atrial and ventricular septal defect
Ventricular septal defect
A ventricular septal defect is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.The membranous...
s, double outlet right ventricle
Double outlet right ventricle
Double outlet right ventricle is a form of congenital heart disease where both of the great arteries connect to the right ventricle...
. Other non-cardiac malformations may be present such as cleft palates, and spine malformations that can cause kyphosis
Kyphosis
Kyphosis , also called roundback or Kelso's hunchback, is a condition of over-curvature of the thoracic vertebrae...
.
Prognosis
Most cases result in stillbirthStillbirth
A stillbirth occurs when a fetus has died in the uterus. The Australian definition specifies that fetal death is termed a stillbirth after 20 weeks gestation or the fetus weighs more than . Once the fetus has died the mother still has contractions and remains undelivered. The term is often used in...
or death shortly after birth. Some cases of ectopia cordis can be treated surgically. Christopher Wall (19 August 1975) holds the Guinness World Record for the oldest living person with condition.
Treatment
Surgical treatment is possible, but there are frequently other birth defects present.Doctors at AIIMS, New Delhi, India successfully operated on a 10-day-old male, who was born with this abnormality, on 2 September 2009. The doctors created a record by completing the surgery without suspending his bodily functions. However, the boy died at 22 days.