Pediatric Heart Expert Forum
Finding a cause for DCM
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Finding a cause for DCM

My son was diagnosed with DCM at 11 days of age (Feb 09.)  They initially thought that his condition was caused or triggered by a viral infection.  In May 09, at his checkup, he had gotten almost completely better.  Things had normalized.  But then in Aug 09, his checkup found his heart function almost back to where it had been in Feb 09.  I am wondering if an Inborn Error in Metabolism is really where our doctors should be investigating.  Our cardiologist said that he doesn't have typical DCM.  HIs heart walls are thin and usually they are thick?  He is seeing more non-conpaction in the LV.  His heart is enlarged and his EF is not more than 45-50%.  It is my understanding that DCM has many causes.  As a mother, what can you tell me about DCM?  Thank you.
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Dear Cool,

Dilated cardiomyopathy (DCM) is a description of the heart when the heart muscle is not squeezing as well as it should.  The muscle typically also does not relax normally, either.  Since these two issues occur, the heart becomes enlarged, or dilated.  There are many different potential reasons for dilated cardiomyopathy.  You have already mentioned two.  One is a  viral infection, which causes a disease called myocarditis secondary to both viral damage to the heart as well as damage due to the immune system’s attempt at fighting off the virus.  This can happen in utero as well as after birth.  The gold standard for diagnosis of this is by a biopsy, although there are newer techniques that use cardiac MRI as well as other viral testing to try to demonstrate this as the etiology.  Another is an inborn error of metabolism.  This is a genetic abnormality in which there is one or more problems in the way certain chemicals in the body are metabolized.  These abnormally metabolized chemicals can poison the heart, can be stored abnormally in the heart, or can cause the production of other abnormal chemicals that damage the heart.  This can often be diagnosed when there are abnormal levels of amino or organic acids in the blood or urine, or other abnormal levels of chemicals in the bloodstream.  However, DCM can also be caused by a genetic disorder of one of the proteins in the heart muscle itself.  The proteins within the muscles don’t work correctly and can cause the muscle function to decrease.  Although many of these gene mutations are known, genetic testing is not widely available for DCM, since there are SO many different gene mutations for it.  Without seeing your son, I can’t say exactly what is going on with him.  I can say that, if he truly has non-compaction of the left ventricle (LV), this may be due to a purely genetic abnormality of the muscle.  Non-compaction is also both a description as well as a type of cardiomyopathy.  The left ventricle, which is the pumping chamber on the left side of the heart, pumps out blood under high pressure.  Therefore, the muscle is normally somewhat thick and dense.  While the heart is forming, the muscle starts out thin and with many peaks and valleys.  Over time, though, this muscle tissue compacts and becomes normal thick muscle.  Non-compaction occurs when this step fails to happen.  There is some research that suggests that this may be a variant of hypertrophic cardiomyopathy (from a gene testing standpoint).  Of note, hypertrophic cardiomyopathy genetic testing is commercially available, as there are fewer overall gene mutations associated with this.

At this point, I would make sure that your son is evaluated by a pediatric cardiologist who has both experience managing cardiomyopathies as well as access to genetic and metabolic testing, as appropriate, to be able to help diagnose exactly what your son has, to give you a prognosis, and to be able to tailor his therapy to his needs.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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