Pediatric Heart Expert Forum
worried
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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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worried

hello i wanted to know if there is any chance of survival on my baby given that dr in uk have said this ,fetal echocardiography confirmed structual cardiac abnormality ,there are normal cardiac connections with left sided aortic arch .bright left ventricular endocardial echos suggesting fibrosis .no antegrade flow was seen across aortic valve. there was free mitral regurgitation which has peak velocity of 3.1m/second .left atrium was considerably enlarged. the atrial septum bowed left to right. there was a small foramen ovale .the pulmonary veins were dialeted bilaterally .there was good right ventricular function .no triscupid regurgitation .retrograde flow in the aortic arch. The letter then says this is incipient of hlhs with a dysplastic mitral valve and aortic atresia and corrective surgery is not possible. PLEASE can you tell me if this op has been done before i have read about the fontan type procedure but been told it can only be done theoreticaly in my case. thanks .
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773637_tn?1327450515
Dear Siobhan,

Unfortunately, what you are describing in your baby is not ideal for hypoplastic left heart syndrome (HLHS) treatment.  HLHS occurs when the left ventricle is small and the aorta is atretic, or so under-formed as to be unusable.  There are different types of HLHS, with the best outcomes occurring when the aortic AND the mitral valves are atretic.  In your baby’s case, you have mitral valve dysplasia, with blood easily entering and leaking back out of the left ventricle.  These are often associated with fistulas, or abnormal connections, between the coronary arteries and the left ventricle; these fistulas can jeopardize the heart for worsening dysfunction.  Already there is what sounds like endocardial fibroelastosis, which is formation of abnormal scar tissue on the inside of the left ventricle.  As well, you are describing what sounds like a restrictive atrial septum, in which the foramen ovale (the natural flap valve that all babies have in utero) is too small for blood to pass through.  This leads to abnormally high blood pressure across the lungs at birth, even after this obstruction is relieved.  This high blood pressure (pulmonary hypertension) makes the ability of the Fontan eventually to function normally very poor.

The interventional options for your child include the three-staged repair, with the last stage being a Fontan procedure, and cardiac transplantation.  Both staged repair as well as cardiac transplantation is quite frequently done for HLHS in numerous centers around the world.  Without evaluating your baby, though, I cannot say for sure, but what I am seeing here suggests that either of these options will be quite high-risk for your baby, with a much lower than normal rate of survival.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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