Monday, January 2, 2012

Diagnosis- Hypertrophic Cardiomyopathy

Okay soo.... Micah was diagnosed with hypertrophic cardiomyopathy, a thickened heart muscle that makes his heart beat irregular.

Because of this I dont know how safe it is for him to go under general anesthesia to have any surgeries. He needs to get some work done below his belly so we are waiting for more results and so Micah gets to wear a heart monitor every so often and the docs will decide if he can go under general anesthesia and if he needs meds......

I can't even think of this and the risks it involves. I have to just enjoy each moment and not overthink this.  I have to be strong for Mikey...

Here is some Wikipedia info:

Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause.[1][2][3][4][5][6] It is perhaps best known as a leading cause of sudden cardiac death in young athletes.[7] The occurrence of hypertrophic cardiomyopathy is a significant cause of sudden unexpected cardiac death in any age group and as a cause of disabling cardiac symptoms. Younger people are likely to have a more severe form of hypertrophic cardiomyopathy.[citation needed]
HCM is frequently asymptomatic until sudden cardiac death, and for this reason some suggest routinely screening certain populations for this disease.[8]
A cardiomyopathy is a disease that affects the muscle of the heart. With HCM, the sarcomeres (contractile elements) in the heart increase in size, which results in the thickening of the heart muscle. In addition, the normal alignment of muscle cells is disrupted, a phenomenon known as myocardial disarray. HCM also causes disruptions of the electrical functions of the heart. HCM is most commonly due to a mutation in one of 9 sarcomeric genes that results in a mutated protein in the sarcomere, the primary component of the myocyte (the muscle cell of the heart).
While most literature so far focuses on European, American, and Japanese populations, HCM appears in all racial groups. The prevalence of HCM is about 0.2% to 0.5% of the general population

In children
Hypertrophic cardiomyopathy (HCM) is one of the most uncommon cardiac malformations encountered in pediatric cardiology. This attributed largely to the phenotypes is usually absent, incomplete, or delayed into adulthood. Most of current information pertaining HCM arises from studies in adult populations, and the implication of these observations for pediatric population is often uncertain.[71] Nonetheless, recent studies in pediatric cardiology have revealed that HCM accounts for 42% of childhood cardiomyopathies with an incidence report of 0.47/100,000 in children.[72] Further, in asymptomatic cases, sudden death is considered one of the most feared complications associated with the disease in select pediatric population. Consequently, the recommended practice is to start screening children of affected individuals throughout childhood to detect cardiac abnormalities at an early stage in hope to prevent further complication of the disease.[73]
Generally, the diagnosis of HCM in a pediatric population is made during assessment for murmur, congestive heart failure, physical exhaustion, and genetic testing of children of affected individuals.[71] Specifically, echocardiogram (ECHO) has been used as a definitive noninvasive diagnostic tool in nearly all children. ECHO assesses cardiac ventricular size, wall thickness, systolic and diastolic function, and outflow obstruction. Thus, ECHO has been chosen as an ideal means to detect excessive wall thickening of cardiac muscle in HCM.[71]
In HCM, treatment strategies aimed to reduce disease symptoms and lower the risk of sudden death.[74] Due to the heterogeneity of the disease, treatment is usually modified according to individual patients needs.[74] β-blockers improve left ventricular filling and relaxation and thereby improve symptoms. In some pediatric patients, β–blockers drugs (e.g. propranolol) were shown to effectively reduce the risk of sudden death.[74] Further, calcium channel blockers (verapamil) and antiarrhythmic drugs maybe used as an adjunct therapy to β-blockers in symptomatic children. Nonetheless, further testing is needed to determine their definitive benefits.[74]

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