General responses to selected questions from Joel Braunstein, MD, of Johns Hopkins University and Joseph Toscano, MD.
Question:
My 2-week-old granddaughter was diagnosed with a heart murmur. We took her to a pediatric heart specialist and were told that she has an abnormal heart valve that closes completely, but that does not open completely. The doctor said we should watch her and make sure the valve develops properly as the rest of her heart and pulmonary artery grows. How fast does the heart grow? Should I be highly concerned about her condition? They performed an EKG that revealed blood is flowing out okay but is not coming back in. Wouldn't it take just a small amount of growth without the valve developing to cause congestive problems? She has a hard time sleeping on her back because of shortness of breath – last night she quit breathing and had to gasp and cough for air. She's also been getting dark rings around her eyes. Should I be concerned about these conditions as well?
Answer:
There are two issues here. The first is your granddaughter's heart. As far as the heart valve not opening fully, this is called "valvular stenosis" and it sounds like she may have pulmonary valvular stenosis, or PS, based on your reference to the pulmonary artery in your note. Overall, PS makes up about 10% of all congenital heart disease (heart disease at birth), so it is not that uncommon. Based on the tightness of the valve, PS is usually graded as mild, moderate, or severe. The valve can be seen using a test called an echocardiogram, a form of ultrasound similar to that used to look at gallbladders, kidneys, and pregnant women's babies. PS can occur by itself or in addition to a hole between the right and left lower chambers of the heart, called a ventricular septal defect or VSD. When PS and VSD occur together, it is a more complicated situation.
It sounds like your granddaughter has had an echocardiogram based on your comment about blood flow through the heart, and this should have shown how bad the PS is and whether anything else is wrong. (As you mentioned, she may have had an EKG as well, though this is an electrical tracing of the heart and doesn't show blood flow.) My exact advice would depend on this information, but it sounds like she had a full evaluation with a pediatric heart specialist, which is the first step. Sometimes this evaluation and a follow-up echocardiogram in 6-12 months is all that is needed. Because no treatment was recommended, she may just have mild or very mild PS.
