Pediatric Heart Expert Forum
asymptomatic 2 year old girl with AV canal with large ASD and small pm ...
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asymptomatic 2 year old girl with AV canal with large ASD and small pm inlet vsd ,

Dear Doctor,
My 2 year old daughter was diagnosed with a heart murmur in her routine 2 yr old checkup. She has no other physical symptom. She was seen by a cardiologist and diagnosed with Complete AV canal. Here is the findings.
weight ls 12.5 kg, helght ie 84 cm, hcart rate le 132, respitory rate 32, blood pressure ot 90/50, fn general, she is in no acute distress. Her HEENT exam is unremarkable. Her lungs are clear to auscultation bilaterally. Cardiac exam reveals a regular race and rhythrm with a 3/6 harsh holosystolic murrnur appreciated along her left sternal border. No diastolic mtrrnur, gallops No rubs or are appreciated. Her abdomen is soft without distention There is no
orgrnomegaly or tenderness to palpation. Extremitties are walm and well perfused with 2+ femorel pulses, no brachial-femoral delay. EKG was obtained which shows normal sinus rhythm wlth right atrial enlargement, Ieft axis deviation, and evidence of an incornplete right, bundle branch block.Her QRS duratlon is 90 mscc.Echocardiography wae performed today, Tha echocardiogram shows her to have a complece AV canal. Complcte Av canal defect w/large primum ASD w/L-to-R shunt. small inlet vsd w/perimembrenous extension => covered w/aneurysmal tissue;LV-RV grad pk 60. Cleft ln L-sided (mitral) AV valve w/mlld to moderate regurgitation.Mlld to moderate R-sided regurgitation w/two distinct jets. Increased velocity across pulm valve => pk 2.5m/s likely fLow related 2-D Echo Doppler demonstrate normal left ventrlcular size and systollc function.

Her cardiologist thinks it is more like a transitional AV canal. Could you be kind enough to provide your opinion and the timeframe for the surgery (i.e should we delay the surgery till she is 3-4 years old) ? Also, do you think the VSD is also a concern and should that be fixed too? Should the valve be repaired or left as is ? also, what are the long term outlook with children with this kind of defect ? do they require more surgery in future ?
773655_tn?1340656399
Without actually seeing the images, I cannot comment accurately about what needs to be done.  But the description fits the diagnosis of a transitional AV canal.  From a practical perspective, this type of heart problem behaves like a hole between the upper heart chambers (ASD) and is not usually associated with any clinical symtpoms.  However it is always associated with an abnormal mitral valve that can have varying degrees of leakage through a "cleft" in the valve.  Surgery is indicated for this heart problem if the ASD is large enough and the right sided heart chambers become enlarged.  Surgery is usually performed between the ages of 2 and 4 years as it will not get better by itself.  The ASD is closed and generally the surgeon will put sutures in the cleft mitral valve to try to decrease the valve leakage if it is more than a mild amount.  they also usually put sutures into the VSD to try to close that, even though it is small.  So it sounds like the surgery could be done at any time in the next year or so, or even now.  Whatever you work out with your doctors.
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