Pediatric Heart Expert Forum
Tetralogy of Fallot with dextrocardia
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Tetralogy of Fallot with dextrocardia

Greetings,

I am a nursing student who was looking for a video about Tetralogy of fallot and I came across your video, I find it interesting. Is that really possible to have a TOF to live until in their 40's? if so is that with surgery or without? I asked because I came across a patient with TOF who never had any surgery. she has all three signs of TOF except for pulmonary stenosis, her's is Pulmonary and Mitrial Atresia also Dextrocardia. Dr. say that she will never live pass her eight birthday but she is now fifteen years of age. I wonder if she can still be a candidate for the Blalock-taussig Shunt or any surgery for that matter?
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Dear Nursing Student,

As you probably know, tetralogy of Fallot is essentially a combination of a ventricular septal defect and pulmonary outflow obstruction.  Given the right amount of balanced pulmonary obstruction, it is certainly possible to have unoperated ToF survive out of infancy and even into adulthood.  The patient about whom you are enquiring is definitely more complicated.  With mitral atresia, we are now talking about all of the body's blood flow going through the right atrium and right ventricle, then dividing into pulmonary and aortic flow.  This can last a while, but it definitely is life shortening for many reasons.  These kids are at risk for endocarditis and brain abscesses.  They are also at risk for Eisenmenger syndrome, with irreversible pulmonary hypertension.

I wouldn't necessarily recommend a B-T shunt at this point, because it seems like she probably has enough pulmonary blood flow without the extra added flow of the shunt.  In fact, deciding what to do with this patient wouldn't really be appropriate without a cardiac catheterization to assess pulmonary hemodynamics and to see what could be tolerated.  Eventually, she may require a shunt to augment pulmonary blood flow, but much more information would be needed.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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