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Heart Disease  (Expert Forum)
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Assessment for CHD in older children
This forum is for questions and support regarding heart issues such as: Angina, Angioplasty, Arrhythmia, Bypass Surgery, Cardiomyopathy, Coronary Artery Disease, Defibrillator, Heart Attack, Heart Disease, High Blood Pressure, Mitral Valve, Pacemaker, PAD, Stenosis, Stress Tests

Assessment for CHD in older children

by Mickey7, May 26, 2007 12:00AM
Can vascular anomalies of the heart be detected by echocardiogram or high-resolution chest CT? We have just found out that our children, who have a disorder called primary ciliary dyskinesia (PCD), are at risk for cardiac malformations that might not be picked up in infancy. The children's father has been diagnosed with a transposition issue with his right main coronary artery  during a cardiac cath and is scheduled for surgery. Their uncle died suddenly from the same defect at age 44. We are being told it is not necessary to do additional assessment on the children because they have had normal echocardiograms and no vascular anomalies showed up on chest HRCT. Should we be pushing for additional assessment? My daughter had an echo several years ago due to episodes of passing out (echo was normal).  She has left-isomerism with polysplenia.  My son (age 17) is currently being treated for hypertension (requiring 3 medications) and chest pain. He has had a chest CT and an EKG, which were okay. His problems are assumed to be lingering issues from a bad pneumonia he had last November.  He has normal situs.  We carry a DNAH5 mutation in the family.  I would be grateful for any insight.  
Reference article; 1: Circulation. 2007 May 21; [Epub ahead of print]
Congenital Heart Disease and Other Heterotaxic Defects in a Large Cohort of
Patients With Primary Ciliary Dyskinesia.

by Forum-M.D.-bkj, May 26, 2007 12:00AM
Mickey,

Thanks for the post.

Unfortunately, I'm not familiar with the condition so I can't specifically advise you on what particular diagnostic course you should take.

Current cardiac CT is able to coronary vessels in adults. I'm not sure of the resolution in children. Given your history, I would make sure I was seen by a pediatric cardiologist or genetic counselor in a tertiary referral center in order to define your children's risk more thoroughly.

good luck
Member Comments (2)

by francie12, May 26, 2007 12:00AM
My son has aortic insufficiency and a dilated asc. aorta associated with a congenital bicuspid aortic valve.  In addition to monitoring with annual echos it was recommended that he have an MRA (an MRI with contrast) to image the area of the aorta that is impossible to be seen by an echo.  Thankfully in our case the dilation was not found to be extended into the arch, but it was important to be able to see the whole length of the aorta, top to bottom.
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