My son who is now 9 weeks old has an ASD measuring 4 mm and several small VSD that are close together causing a shunt (both directions) like a moderately sized VSD. The pressure in both ventricles are the same. He does not have Down Syndrome... pediatric cardiologist is having us wait 3 more weeks to check on the pressures again before ordering more tests to find out what is causing the pulmonary hypertension. I'm confused b/c I thought the VSD was the cause? Shouldn't the pressure in RV decreased at birth? Not 9 weeks or longer? Should we look at lungs? Sooner than later?
You are correct--the resistance to blood flow in the lungs (pulmonary vascular resistance) typically falls to adult levels, which is 1/4 to 1/5 of the systemic blood pressure, by about 2 months. Certainly, patients with moderate or large left to right shunts, like with the VSDs, can cause persistently increased pulmonary vascular resistance. Some kids maintain elevated pressures because of that. There can be other reasons, as well, including pulmonary vein stenosis or obstructive abnormalities of the mitral valve, that can maintain that elevation. However, there can also be intrinsic abnormalities that can lead to pulmonary hypertension. Sometimes the addition of a diuretic can help to decrease the amount of volume overload and cause a fall in resistance. Some folks will do a hyperoxygenation test to see if their resistance falls. Also, sometimes these kids need a bit more time to drop their resistance. Finally, some of them require cardiac catheterization to ensure that there are no other abnormalities that may have been missed by echocardiogram (as above) and to make sure that their pulmonary blood vessels respond appropriately to oxygen. Actually "looking" at the lungs is not necessarily helpful, outside of a biopsy, which may not be necessary this early in the process, unless there are other birth defects.
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