1) Right Dominant Unbalanced Atrioventricular Canal Defect or Atriventricular Septal Defect (AVSD)
+"...large hole in the center of the heart. It exists where the wall between the upper chambers joins the wall between the lower chambers. This septal defect involves both upper and lower chambers. Also, the tricuspid and mitral valves that normally separate the heart's upper and lower chambers aren't formed as individual valves. Instead, a single large valve forms that crosses the defect."
(See website
http://www.americanheart.org/presenter.jhtml?identifier=132 for picture of normal heart and additional information)
2) Malposed Great Arteries
+"...In transposition of the great arteries, the aorta and pulmonary artery are reversed. The aorta receives the oxygen-poor blood from the right ventricle, but it's carried back to the body without receiving more oxygen. Likewise, the pulmonary artery receives the oxygen-rich blood from the left ventricle but carries it back to the lungs."
(See website
http://www.americanheart.org/presenter.jhtml?identifier=132 for picture of normal heart and additional information)
+In the case of Jevesh's heart, the pulmonary artery is absent (see below)
3) Pulmonary Atresia
+"In pulmonary atresia, no pulmonary valve exists. Consequently, blood can't flow from the right ventricle into the pulmonary artery and on to the lungs. ... The infant appears blue (cyanotic) because there's less oxygen in the blood circulating through the arteries."
(This is what originally alerted Deepika that there was a problem)...
"The only source of lung blood flow is the patent ductus arteriosus (PDA), an open passageway between the pulmonary artery and the aorta. ....Early treatment often includes using a drug to keep the PDA from closing."
(This was the Prostaglandin that they had Jevesh on prior to his surgery for the shunt which replaced the PDA)
"A surgeon can create a shunt between the aorta and the pulmonary artery that may help increase blood flow to the lungs. A more complete repair depends on the size of the pulmonary artery and right ventricle. If the pulmonary artery and right ventricle are very small, it may not be possible to correct the defect with surgery. In some cases, where the pulmonary artery and right ventricle are more normal in size, open-heart surgery may produce a good improvement in how the heart works."
(See website
http://www.americanheart.org/presenter.jhtml?identifier=132 for picture of normal heart and additional information)
4) Single Coronary Artery
+"The coronary arteries are the only branches of the ascending aorta, and they supply blood to all structures within the pericardial cavity. Usually, the 2 coronary artery ostia are located in the center of the left and right (anterior) sinuses of the aortic valve."
(See website
http://emedicine.medscape.com/article/153512-overview for more information)
+"Single coronary artery is a rare congenital anomaly of the coronary arteries where only one coronary artery arises from the aortic trunk by a single coronary ostium, supplying the entire heart"
Ostium = Opening
(See website
http://eurheartj.oxfordjournals.org/cgi/content/abstract/13/12/1637 for more information)
5) Left Pulmonary Artery (LPA) Stenosis
+A narrowing of the pulmoary artery, specifically for Jevesh, his left pulmonary artery is narrowed.
Stenosis = a narrowing
Atresia = Abnormally closed or absent
Malposed = Wrong position
AO (in the picture) = Aorta
IVE (in the picture) = Inferior Vena Cava
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Additionally Jevesh has:
Asplenia = He has no spleen (which equals higher risk of infection)
Heterotaxy or Situs ambiguous = internal organs are backwards from where they should be...Essentially, it is the Situs ambiguous that has resulted in all the problems...from the heart defects, to the asplenia, to the malrotation of the intestines... (See
http://en.wikipedia.org/wiki/Heterotaxy for more information)
Malrotation of the intestines (this is still being discussed by the doctors)
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Jevesh has had the first of what will be a minimum of three surgeries (likely more). The doctors placed the shunt to replace the Patent Ductus Arteriosus (PDA) and take him off the Prostoglandin.
Jevesh may have to have surgery for malrotation of his intestines...we are still waiting for the doctors to complete their evaluation and testing.
All of the defects are interrelated...they are developmental (congenital) defects which likely occurred during the early stages of pregnancy...There is no known cause, only theories...what the doctors know is that sometimes during gestational development, the cells get the message wrong...
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