Dilated or congestive cardiomyopathies

These are the most common cardiomyopathies (60 %) in children. Annual incidence: 0.58/100.000. The symptoms are variable and depend on the severity of the myocardial involvement: fatigue on exertion, feeding difficulties, syncope, chest pain, cardiac failure. The diagnosis is sonographic: dilatation of the LV or both ventricles which leads to systolic dysfunction (ejection fraction < 45 %) and telediastolic dilatation of the ventricles, hypokinesia, sometimes mitral and / or tricuspid regurgitation by dilatation of the valve ring. It happens that cardiac dilation causes extrinsic compression of the left main bronchus. Mortality of 14 % within 2 years of diagnosis.

Treatment includes cardiotonic drugs (digoxin) often associated with a diuretic and sometimes a vasodilator (angiotensin-converting enzyme inhibitor) that must be continued until the day of the surgery even if it increases the risk of intraoperative hypotension.

Ventricular dilatation is associated with impaired systolic function: it is therefore necessary to maintain optimal ventricular filling while avoiding overload and pulmonary edema.

Etiologies	concerned pathologies
decompensated systolic overload	aortic stenosis, coarctation, high blood pressure
decompensated diastolic overload	long-lasting left-right shunt, mitral or aortic regurgitation, systemic arteriovenous fistula
myocardial ischemia	left coronary neonatal abnormality (ALCAPA), Kawasaki disease, neonatal ischemia
chronic rhythm disorder
infection	viral myocarditis, trypanosomiasis
collagenosis
toxic	chemotherapy (anthracyclines at the beginning of treatment); mediastinal radiatiotherapy
metabolic	primary or secondary carnitine deficiency some mitochondrial cytopathies abnormalities in the β-oxidation of fatty acids Danon's disease (glycogenosis) dystrophic epidermolysis bullosa (selenium and/or carnitine deficiency)
familial	see this term
muscular dystrophies	as exemple: - Duchenne myopathies, Becker - X-linked cardiomyopathy, laminopathy, desminopathy - dystroglycanopathy (limb-girdle dystrophic myopathy): usually early after the appearance of muscular signs but sometimes isolated with high CPK level
cystic fibrosis
sickle cell anemia, β-thalassemia
heavily burned (> 70 %)	transient, usually begins > 100 days after the burn

Causes of dilated cardiomyopathies in children

diastolic function	decreased compliance of ventricles with diastolic dysfunction dilatation of the atria increased atrial pressures
systolic function	decreased contractility: good response to milrinone unless very fibrous myocardium no filling reserve
pulmonary circulation	increased pressure in LA leads to increased pulmonary pressure risk of reversible and then irreversible increase in pulmonary resistance
preload effect	poor tolerance of hypovolemia
heart rate effect	avoid bradycardia because the ejection volume is fixed avoid tachycardia because it decreases subendocardial perfusion
post-charge effect	avoid any increase in peripheral resistances

Hemodynamic consequences of dilated cardiomyopathies

Anesthetic implications:

recent echocardiography. Invasive monitoring of BP (+ transthoracic or esophageal echo). Avoid a decrease in venous return that can lead to a significant drop in cardiac output. Circulation time is lengthened. Etomidate combined with low doses of an opiate is the induction agent of choice. Ketamine,the intrinsic cardiodepressant effects of which are not compensated by an increase in sympathetic tone in this context, hass to be used with caution. The initiation of positive pressure ventilation should be careful because its effect on ventricular filling is poorly predictable in these patients.

References :

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Cardiopathie par dysfonction myocardique.
In Cardiologie pédiatrique pratique, 4ème éd. Doin, 2013, p 187-93.

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Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia.
Pediatr Anesth 2007; 17:775-81.

- Ing RJ, Ames WA, Chambers NA.
Paediatric cardiomyopathy and anaesthesia.
Br J Anaesth 2012 ; 108 : 4-12

- Lynch J, Pehora C, Holtby H, Schwarz SM, Taylor K.
Cardiac arrest upon induction of anesthesia in children with cardiomyopathy : an analysis of incidence and risk factors.
Pediatr Anesth 2011 ; 21 : 951-7

- Simbre VC, Duffy SA, Dadlani GH, Miller TL, Lipshultz SE.
Cardiotoxicity of cancer chemotherapy : implications for children.
Pediatric Drugs 2005; 7:187-202.

- Huettemann E, Junker T, Chatzinikolaou KP, Petrat G, Sakka SG, Vogt L, Reinhart K.
The influence of anthracycline therapy on cardiac function during anesthesia.
Anesth Analg 2004; 98:941-7

- Williams GD, Hammer GB.
Cardiomyopathy in childhood.
Curr Opin Anaesthesiol 2011 ; 24 : 289-300.

Updated: July 2021