Pediatric Heart Expert Forum
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Questions in this forum are answered by pediatric cardiologists, cardiothoracic surgeons and anesthesiologists from The Children's Hospital of Philadelphia. This forum is for questions and support about pediatric heart problems, symptoms and topics such as heart murmurs, palpitations, fainting, chest pain, congenital heart defects (including management and intervention), fetal cardiology, adult congenital cardiology, arrhythmias and pre-participation athletic screening.

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What medication/treatments would you recommend for a 2 1/2 yr old patient with these sedated 2-D Doppler Echocardiogram results?:


MITRAL VALVE thickening of the mitral valve leaflets was present.
                         systolic anterior motion of the anterior leaflet of the mitral valve was noted.
LEFT VENTRICLE/LEFT VENTRICULAR OUTFLOW TRACT Moderate concentric left ventricular hypertrophy was present.
                                                                                    A fibromuscular ridge protruding in the left ventricular outflow was present.
AORTIC VALVE hypoplasia of the aortic valve was noted.



DOPPLER STUDY

Moderate to severe regurgitation of the mitral valve was present.
Trivial regurgitation of the tricuspid valve was present.
Doppler interrogation of the pulmonary valve was normal.
Trivial regurgitation of the aortic valve was present.
Severe subaortic stenosis was present.
Trivial left to right shunt was present across the ventricular septum.


CONCLUSION

Moderate to severe mitral valve insufficiency.  {424.0}

Mild aortic valve hypoplasia.  {747.22}

Discrete fibrous subaortic membrane.  {746.81}

Moderate left ventricular hypertrophy.  {429.3}

Qualitatively normal right ventricular function.

Qualitatively normal left ventricular function.

Multiple ventricular septal defects.  {745.4}

Related Discussions
773655_tn?1340656399
Has this child ever had any intervention?  what about the aortic arch?  there is no mention of whether there is a coarctation.
4 Comments
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Avatar_m_tn
He hasn't had any intervention.  He just takes propranolol 8mg q 6hrs.  Aortic arch is normal.
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Avatar_m_tn
Sorry, there was an additional comment i left out accidentally, it says "Aortic annulus measures 10mm" ; is this very abnormal or why was it even mentioned?
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773655_tn?1340656399
This child has severe obstruction to the blood flow from the left side of the heart to the body.  A 10 mm aortic valve annulus in a 2 year old is about the size of the aortic valve in a newborn baby.  It sounds like the thickening of the heart muscle (LVH) is due to this blockage, and the mitral valve leakage is due to either high pressure in the pumping chamber and/or an abnormal mitral valve.  Propranolol may help suppress extra heart beats but will not help the underlying situation.  This child needs surgery to relieve the outflow tract obstruction, which will likely need to be a very complex surgery called a Ross-Konno procedure.  In this surgery, the outflow tract of the left ventricle is opened and the small aortic valve is replaced by the patient's pulmonary valve;  also a conduit is placed from the right ventricle to the lung arteries.  If this is successful in relieving the outflow tract obstruction, then gradually the thickening of the pumping chamber would regress.  Without therapy of some type, heart failure will develop in this child, which will shorten the life span.  If the heart fails, then a heart transplant might be indicated.  Depending upon your location and access to medical care, these options may or may not be readily available to this child.
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