Definition:

A congenital heart defect which causes babies to have insufficient oxygen throughout their body.  There are four distinct abnormalities associated with defect: a hole between the two lower chambers of the heart (ventricular septal defect), the narrowing of the tube that connects the heart to the lungs (pulmonary stenosis), the muscular thickening of the chamber that sends blood from the heart to the lungs (right ventricular hypertropy), and the displacement of the aorta (the vessel that sends blood from the heart to the body) over the ventricular septal defectt, causing mixing of oxygenated and unoxygenated blood.

Symptoms:

• Cyanosis (bluish skin) during agitation (crying/feeding)
• Shortness of breath during cyanotic episodes
• Fainting (syncope)

Diagnosis:

• Blood oxygen content - will show increase in red blood cell count
• Ultrasound - to see if blood is flowing in right direction
• X-Rays with Contrast - to see if blood is flowing in right direction
• Echocardiography - confirmation of disease
• EKG - visualization of right ventricle muscle thickening

Treatment:

• Surgery - ventricular septal defect closure, removal of muscle from right ventricle, pulmonic valve repair, pulmonary artery enlargement (surgery can be done temporarily in small infants, and then complete repair can follow when the baby is of age)
• Regulation of physical activity/exertion
• Regular visits to the cardiologist to ensure proper circulation