Pediatric Heart Expert Forum
pulmonary aneurysm - information
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pulmonary aneurysm - information

my daughter will be giving birth in November and her unborn child has been diagnosed with, among pulmonary stenosis and vertrical septal defect, a pulmonary aneurysm which is described as "huge."  I cannot find any information on this kind of aneurysm and need help locating information about it.
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773637_tn?1327450515
Dear Krismichelle,

For our other readers, an aneurysm is an abnormal enlargement and thinning of the blood vessel wall; in this case, I am presuming that this is in the pulmonary artery and not in the pulmonary vein.  I am somewhat concerned that this “pulmonary aneurysm” in the face of pulmonary stenosis and a ventricular septal defect may, in fact, be tetralogy of Fallot with absent pulmonary valve, though I can’t tell completely from your description.  Tetralogy of Fallot is a combination of 4 descriptive findings in the heart caused by anterior and upward displacement of the upper part of the septum (wall) between the ventricles.  The 4 findings include:  a ventricular septal defect, or a hole between the two lower chambers of the heart; an “overriding” aorta, in which the artery that leads out of the left side of the heart to the body is not lined up with the ventricular septum as it should be and overlaps the septum; pulmonary stenosis, in which there is obstruction at the pulmonary valve (the valve leading from the right side of the heart out to the lungs) and/or below the level of the valve in the outflow tract; and, right ventricular hypertrophy, in which there is thickening of the pumping chamber on the right side of the heart since it is seeing much higher blood pressures than it normally would.  These are the components of classic tetralogy.

Absent pulmonary valve syndrome occurs when the pulmonary valve leaflets don’t form normally, and lead to a combination of severe leakage and stenosis at the pulmonary valve.  This back-and-forth flow of the blood causes huge dilation of the main and branch pulmonary arteries, as well as the smaller pulmonary arteries inside the lungs.  This can lead to compression of the large and medium-sized airways in the lungs and give a picture similar to asthma.  These infants can have significant breathing difficulty when they are born.  Absent pulmonary valve syndrome can occur with an intact ventricular septum or in the face of tetralogy of Fallot.  Often, these babies need to go to the operating room to have their pulmonary arteries reduced in size, if they are having significant airway problems.  And, this doesn’t always fix the problem, as the smaller arteries in the lungs, which can’t be reached by surgery, can remain dilated and still compress the airways.  If they don’t have symptoms, they can be observed till they get bigger.  Either way, with tetralogy of Fallot, the heart will definitely require surgical repair in the future, usually before age 6 months.  If it is not tetralogy of Fallot, the need for and timing of repair depends on how large the ventricular septal defect is, how bad the pulmonary stenosis is, and how bad the airway compression is with by the pulmonary arteries.

Having said all of that, this could also truly be a large pulmonary artery aneurysm due to a genetic abnormality.  Another possibility is an aneurysm of the ductus arteriosus, which is a blood vessel between the aorta and the pulmonary arteries that all babies have in utero; this vessel closes after they are born.  Finally, I would definitely recommend that your granddaughter be evaluated and followed by a pediatric cardiologist, if she hasn’t already, and have a Genetics evaluation, as well.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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