Just curious if you know of any advancements being made in dealing with Ectopia Cordis cases? It interested me so I did a little looking and it all looks pretty bleak. The prognosis is generally not good due to infection/cardiac failure etc very shortly after birth. Just wondering if being more plugged in to the medical world if you knew more. :) Have a good one!
So I read a 2014 review article (http://www.ncbi.nlm.nih.gov/pubmed/24753313)on ectopia cordis after you sent me this question. It confirms that theprognosis is generally quite bleak but it does depend on the type of ectopiacordis (defined by location of the heart), and the severity of associateddefects.
Cervical EC, in which the heart is located at the base ofthe neck above the sternum is almost always fatal in utero. There have been 91cases of thoracic EC, with the heart emerging through split or absent sternum,in the literature and only one patient has survived. A better prognosis is seenwith thoracoabdominal EC, which often accompanies Pentalogy of Cantrell. Theother findings in Pentalogy of Cantrell are supraumbilical abdominal walldefect, ventral diaphragmatic hernia, sternal cleft and congenital intracardiacdefects.
Intracardiacdefects frequently occur with EC and their severity also affects the prognosis.Ventricular septal defects occur in 100% of cases, atrial septal defects in53%. Tetralogy of Fallot and left ventricular diverticulum occur in 20%. Othergenetic anomalies can coexist and worsen the prognosis.
EC can bedetected on ultrasound early in pregnancy and if the parents choose to continuethe pregnancy, caesarean delivery needs to be planned. They can then choose nonaggressivetreatment or surgical repair.
Multistagesurgery is the usual strategy for EC beginning with covering the heart withsoft tissue to prevent fluid loss, cardiac desiccation and trauma. Next, theheart needs to be reduced into the thoracic cavity and any intracardiacanomalies repaired. Some think that delaying the reduction a year or two instable patients allows growth of the thoracic cavity. Thoracic hypoplasia oftenpresents a difficulty in this repair – you can’t just squish the heart back inthere, the heart and vessels would become compressed. To accommodate the heartsome techniques will remove part of the lung, liver, or thymus. Finally, thesternal defect needs to be repaired. Autogenous bone grafts (costal cartilage,ribs and ilium) or alloplastic prosthetics (titanium, plastics) can be used,then covered with muscle and skin flaps. Single stage surgery usually haspoorer prognosis but there are some successful cases reported. Additionally,any other midline defects that commonly occur with EC need to be repaired.Heart transplantation is not done because immunosuppressive therapy iscontraindicated in the extensive surgery usually required for the accompanyingdefects.
So, ectopia cordis is a serious defect usually complicatedby other defects. It sounds like it is so rare that it would be hard to work onimproving techniques. From the little readingthat I did, I had a hard time getting a sense of what a “good” prognosis evenis. How long do they live if they make it though surgery? What is the long termprognosis? I don’t know. It probably depends again on the severity and theother defects they are dealing with.
To end on a positive note, this little girl born withectopia cordis is doing so well she has a Twitter account!
