my daughter is 5. small, 1-5th percentile weight, 7th% height, with suspected, yet unidentified connective tissue disorder with cardiac involvement. all heart valves affected, mitral most seriously. she also has supravalvular aortic stenosis. she is currently on enalapril 1.5 ml/twice daily. blood pressures typically 95/50, but have recently started to fall into low 40s. highlights of latest echo: abnormal, prolasping mitral and tricuspid vavles, upper moderate mitral regurg, dilated left ventrical, mild dilated left atrium, normal left ventricular systolic function, shortening fraction 31%, ejection fraction 59%, mild flow acceleration in the ascending aorta, (her aortic root diameter is at the minumum for her size)
leah's deterioration pattern has always been iratic and unpredictable. we have noticed a signifigant decline in her activity tolerance in the last couple months. she is always pale and gray around her mouth, hands and feet. she often 'grunts' when breathing. her heart rate is irregular most of the time.
finally, my question....from what i understand, the mitral valve will have to be replaced. although her overall function is still acceptable, we feel we are walking a fine line before heart failure begins. what would you advise, generally, for timing of this surgery? what are the most concerning risk/benefits? in a nutshell, should we wait for verifiable heart failure criteria before risking surgery, or would that be too late? can valves be replaced on a heart with muscle damage?
thanks for the opinion,
This is one of the more difficult questions that we face, in that we don't always know the exact timing or way to deal with an abnormal mitral valve. The options include surgical revision of the mitral valve and replacement of the mitral valve with another valve, typically a mechanical one. Both have their downsides. Revision of the mitral valve when there is a connective tissue disorder present suggests that the valve repair may not last particiularly long, or even at all. Placement of a mechanical valve means that the valve won't grow and will need to be replaced in the future to account for growth AND that Leah will need to be treated with anticoagulation medication (Coumadin). However, it sounds like the severity of her mitral regurgitation in conjunction with the symptoms that you mention confirm that she is already in heart failure. And, I would certainly expect her shortening fraction to be higher with the degree of regurgitation that she is experiencing. With a "normal" shortening fraction of 31%, her heart is already getting worn down by the extra volume load placed on it. Obviously, without evaluating her myself, I cannot say for sure. However, it sounds to me like these finidngs should be forcing the hand of cardiologist and/or surgeon to move toward intervention at this time. It is a big step, and I would definitely recommend a second opinion if you are not feeling like you are getting the answers you need to the questions that you have.
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