Pediatric emergency medicine trisk 1743 1743
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HCM is a genetic disease in which cardiac hypertrophy and symptoms develop
over time. In this sense it is an acquired cardiomyopathy. Please see the section
on SCD earlier in this chapter for more discussion ( Table 86.10 ).
Dilated Cardiomyopathy (DCM) is the end result of various disease processes.
While the majority of DCM in pediatrics is idiopathic, some cases are familial or
due to inborn errors of metabolism (mitochondrial disorders, Barth syndrome,
carnitine deficiency). In older children the most commonly identified causes are
myocarditis and neuromuscular disease (Duchenne and Becker muscular
dystrophy). Left ventricular obstructive lesions and incessant tachycardia may
also produce reversible DCM when the obstruction or tachycardia is corrected.
KD, rheumatic fever, and Lyme disease are also recognizable causes of DCM.
Some patients who have received anthracyclines for chemotherapy will develop
DCM.
The incidence of DCM in children younger than 18 years old is 0.57
cases/100,000/year. A higher incidence has been noted in boys, Blacks compared
to Whites, and infants less than 1 year old. DCM may lead to CHF and is the
most common reason for cardiac transplantation in the pediatric population.
Patients with familial or idiopathic DCM that are older than 6 years of age, or
have CHF at diagnosis are at highest risk for heart transplantation.
DCM presents with shortness of breath and exercise intolerance. Young
children have vague symptoms such as tachypnea, dyspnea, irritability, and poor
feeding. Symptoms usually are insidious except in acute viral myocarditis.
Physical examination may reveal signs of CHF and a mitral regurgitation murmur
or S3 gallop. EKG usually shows sinus tachycardia, LVH, and nonspecific ST and
T wave changes. Incessant tachyarrhythmias such as SVT and VT are poorly
tolerated in patients with DCM and should thus be treated aggressively. Any
incessant tachycardia may be the cause of DCM. The goal of treatment of DCM
in the ED is early identification of CHF and supportive care. ECMO can be a
bridge to recovery or transplant and cardiac surgery should be involved early. For
management of CHF, see section on Acute Heart Failure Syndromes.
