How is pediatric dilated cardiomyopathy treated?

  Pediatric dilated cardiomyopathy (DCM), which has been on the rise in recent years, is a serious cardiac disorder that endangers pediatric health. The disease progresses rapidly, and most of them are diagnosed with cardiac insufficiency, with poor prognosis and high mortality, often resulting in death due to refractory heart failure or fatal arrhythmias. The diagnostic criteria are described in [1]. Echocardiography is an important tool for the diagnosis of DCM, which is characterized by “large chambers, thin walls, small mouths, reduced amplitude of motion, and reduced ejection fraction”. The characteristics of DCM echocardiography are “large chambers, thin walls, small mouth, reduced motion, and reduced ejection fraction.  DCM in children often presents as chronic systolic heart failure, which can present with arrhythmias and sudden death. Its treatment regimen has shifted from short-term hemodynamic/pharmacological measures to a long-term, restorative strategy aimed at altering the biology of the failing heart, improving symptoms while improving quality of life and preventing and delaying the progression of myocardial remodeling, which has resulted in a 46% reduction in mortality.  General treatment Control of respiratory infections Rest Can slow down the heart rate, reduce the heart load, prolong the diastolic period, increase cardiac output, and protect the myocardium. After the heart failure is controlled, it is still necessary to limit the activity until the heart size returns to normal.  Progress of comprehensive treatment of heart failure Pharmacotherapy (1) Basic drugs digitalis, diuretics and vasodilators are commonly used.  ①Digoxin oral dose: >2 years old 0.03-0.04 mg/kg; <2 years old 0.05-0.06 mg/kg, 1/4-1/5 of the dose, divided into 2 doses in the morning and evening every day, long-term maintenance for 6 months to several years, until the heart shrinks close to normal. Diuretic application: For chronic heart failure and edema, it is advisable to combine potassium preservation with potassium excreting diuretics for long-term users. In acute heart failure, furosemide can be used, 1~2mg/time, intravenous or intramuscular injection, repeated use needs to pay attention to water-electrolyte imbalance. The former, such as phentolamine, l-2 μ g / (kg.min) intravenous drip, for pulmonary hypertension, the latter, such as isosorbide nitrate, 0.5 ~ 1.0 mg / (kg.d), divided into three doses, for pulmonary stasis, severe cases of arterial and venous dilators such as sodium nitroprusside 0.5 ~ 8.0 μ g/(kg.min).  (2) β a receptor blocker: With the deepening of understanding of the pathogenesis of heart failure in recent years, attention to chronic heart failure, excessive neuroendocrine activation, catecholamine concentration is too high, the myocardium has toxic effects, damage to the myocardial cell membrane conduction system (β a receptor adenylate cyclase system), damage to myocardial contractile function, so that chronic heart failure is difficult to control, β a receptor blocker can block the above vicious circle, has confirmed that chronic β-1 receptor blockade can prevent the development of cardiomyopathy, and at the same time can exert anti-apoptotic, anti-remodeling and antioxidant stress responses, which can improve the biological effects of myocardium and enhance the anti-cardiac failure efficacy. The improvement in cardiac function and heart shrinkage after treatment with the selective β1-monoceptor blocker metoprolol were both statistically significant, with significant improvement in the prognosis of cardiomyopathy, and even cure. The initial dose of metoprolol is 0.2-0.5 mg/(kg.d), divided into 2 doses, and gradually increased in 2-3 weeks, the maximum dose is 2.0 mg/(kg.d), divided into 2 doses, and the course of treatment is 8 weeks-6 months or more, and even several years, starting with a small dose, gradually increasing, reaching the maximum maintenance amount, and a long course of treatment is appropriate.  For the application in pediatric patients, experts remind that the treatment should be started with small doses and gradually increased, and attention must be paid to individualized doses and long courses of treatment. Blood pressure and heart rate should be closely monitored during the course of treatment, and should not be used in patients with bradycardia or conduction block. The combination of carvedilol on the basis of conventional treatment can improve the patient's cardiac function and symptoms, and the treatment compliance is good, but the progress of chronic heart failure and sudden asthma need to be closely monitored.  (3) The initial dose of angiotensin-converting enzyme inhibitor Benazepril is 0.1 mg/(kg.d) once/d, gradually increased to 0.3 mg/(kg.d) in a week or so, the course of treatment is 4-12 weeks or more, Enalapril 0.08-0.10 mg/(kg.d) once/d, or Captopril 0.2-4.0 mg/(kg.d) in 2 doses, the course of treatment is the same as above.  Wang Cheng et al. applied hydrochlorothiazide 1~2 mg/(kg.d), enalapril 0.08~0.10 mg/(kg.d), spironolactone 1~2 mg/(kg.d) orally, and small dose digoxin was added for class IV cardiac function, which was given orally twice a day at 1/5 of the saturated dose for 3~6 months, and dobutamine and dobutamine were added for children with recalcitrant heart failure, which was given by infusion pump at 2~5ug/(kg.d). ~The dobutamine and dobutamine should be administered intravenously by infusion pump at the rate of 2 to 5ug/(kg.min) for 3 to 5 days. When cardiac function improves to grade II-III, metoprolol 1.0-1.5 mg/(kg.d) is added orally. Combined application of hydrochlorothiazide, enalapril, metoprolol and spironolactone for pediatric DCM can significantly delay or prevent the progression of myocardial remodeling, improve cardiac function and improve the quality of survival.  (4) Immunosuppressive therapy The application of immunosuppressive agents is subject to different opinions. For early patients with short onset, or those with complicated cardiogenic shock, severe heart failure or severe arrhythmias, prednisone therapy can be added, starting with 2 mg/kg.d, divided into 3 doses, maintained for 1 to 2 weeks and gradually reduced to 0.3 mg/kg.d for about 8 weeks, and maintained until 16 to 20 weeks, and then gradually reduced to discontinuation for more than half a year. If prednisone alone is not effective, azathioprine 2mg/kg.d can be used in combination, divided into 2 doses, and the white blood cells should be monitored and maintained at 4×109/L or more.