Pediatric Heart Expert Forum
Tetrology of fallot with absent pulmonary syndrome.
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Tetrology of fallot with absent pulmonary syndrome.

If a child age 4 years is suffering from Tetrology of fallot with absent pulmonary syndrome, after surgery, he/she will spent his/her life normally?
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Tetralogy of Fallot (TOF) with absent pulmonary valve leaflets is one of the less common forms of this congenital heart anomaly.  In many cases, the large pulmonary arteries associated with this condition compress the airway tubes and cause respiratory distress that leads to early surgery.  If there is a child who reaches 4 years without a surgical intervention, it means that they don't have as much of that airway compression and their oxygen levels (hopefully) have been reasonable.  After surgical repair, the patient would be treated like any other TOF patient:  looking for signs of right heart chamber dilation from a leaking pulmonary valve, watching for narrowings of the lung artery branches and making sure the lung artery pressure is normal.  We do not know the life expectancy for all patients with serious congenital heart disease, but the hope for most TOF patients is that they will lead productive adult lives, knowing that other interventions like a pulmonary valve replacement, may be needed somewhere along the line.
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Thanks for your comprehensive answer........
Regards,
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
Philadelphia, PA
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Marie M Gleason, M.D.Blank
The Children’s Hospital of Philadelphia
Philadelphia, PA
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