What Is Prune Belly Syndrome?
9. Cardiovascular Abnormalities
Cardiovascular abnormalities can also occur in Prune Belly syndrome. They are reported in roughly ten percent of patients; the most common being tetralogy of Fallot and ventriculoseptal defects.
Tetralogy of Fallot (TOF) is a congenital condition that affects the structure of the heart. It is caused by a combination of four heart defects that include pulmonary valve stenosis (narrowing of the pulmonary valve), ventricular septal defect (“hole” in the wall that separates the ventricles), overriding aorta (aorta is shifted to the right and receives blood from both ventricles), and right ventricular hypertrophy (thickened muscular wall of the right ventricle). The result of these cardiac defects, is that blood that is low in oxygen flows out of the heart and to the rest of the body. Thus, children with this abnormality can have blue-tinged skin (cyanosis).
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