Ectopia cordis

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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.

Often other birth defects are also present. This condition is usually fatal in the first days of life. In some cases surgical treatment is possible. 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 defects, double outlet right ventricle. Other non cardiac malformations may be present such as cleft palates, spine malformations that can cause kyphosis.

Occurrence: 7.9 per million births. Ectopia cordis is a very rare congenital heart malformation.

Etiology: No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome and Trisomy 18; however so far there is no evidence that it is a genetically transmissible disease.

Prognosis: Most cases result in stillbirth or death shortly after birth. Some cases of ectopia cordis can be treated surgically but in general involve lengthy and very complicated pediatric cardiothoracic surgery.

Depending on the position of the heart from birth ectopia cordis can be classified into four different categories: Cervical. 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.

The malfunction happens when the sternum forms during the gestation period and the heart begins to grow in the wrong place.

Christopher Wall

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Christopher Wall is known as a miracle of life. Wall was born with severe ectopia cordis, with his heart outside of his chest. As all other infants that had this condition lived no longer than 48 hours, Wall defied the odds by living more than twenty years. Surgeons were able to sew Wall's heart into the skin, however Wall was hospitalized for 3 years before he even went home. After recovery, Wall was able to live a normal life with a passion for basketball. Wall always had to wear a chest guard, for if his heart suffered even a mild blow, it would be fatal. Wall has set a Guinness World Record of the longest time lived with the heart outside of the body.

• • NASEEM HASNI**

born October 31,2006 in Jackson Memorial Hospital, Miami, Florida with Ectopia Cordis. Naseem stayed in Jackson for 15 weeks and had 4 surgery's before he was 3 months of age, and he suffered from hydrosyphilis and had a shunt placed in july of 2007. Naseem just celebrated his first birthday and major milestone for him. http://naseem-hasni.celebration-of.com

References

• Amato J, Douglas W, Desai U, Burke S (2000). "Ectopia cordis.". Chest Surg Clin N Am 10 (2): 297-316, vii. PMID 10803335.

• Ellis-Christensen, Tricia. "Ectopia cordis.". Theswisegeek.com.

• [1] Video of Christopher Wall on Guinness World Records Primetime.

[2]

See also

• Ectopia cordis on www.thefetus.net
• Doctors repair heart born outside baby's body NASEEM HASNI

Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .