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Restrictive cardiomyopathies
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They account for about 2-5 % of children's cardiomyopathies.
The causes in children are:
- amyloidosis, chronic eosinophilia (Löffler's disease)
- cardiomyopathy following treatment with anthracyclines (or mediastinal radiation therapy) is a more common cause
- untreated Gaucher disease
- in Africa, fibroelastic endocarditis with eosinophilia
The increase in myocardial stiffness leads to a gradual decrease in the telediastolic volume of the LV or both ventricles and diastolic dysfunction. The restriction of ventricular filling leads to upstream stasis with dilatation of the atria and finally pulmonary hypertension. In addition to fatigue with dyspnea on exertion and hepatalgia, the signs are those of RV failure: hepatomegaly, lower limbs edema, ascites. Thromboembolic complications and conduction disorders are common. Survival is less than 50 %, 2 years after the diagnosis.
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diastolic function |
decreased compliance of ventricles diastolic dysfunction of the ventricles dilatation of the atria increased atrial pressure |
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systolic function |
at first, the contractility of both ventricles is preserved |
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pulmonary circulation |
increased pressure in LA leads to increased pulmonary pressure risk of reversible and then irreversible increase in pulmonary resistance |
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preload effect |
the decrease in compliance of the ventricles leads to a rapid increase in atrial pressures in case of filling |
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heart rate effect |
avoid bradycardia because the ejection volume is fixed avoid tachycardia because it decreases the filling of the LV and therefore the ejected volume |
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post-charge effect |
avoid a decrease in peripheral resistances because the ejected volume is fixed |
Hemodynamic consequences of restrictive cardiomyopathy
Anesthetic implications:
recent echocardiography. It is important to maintain the ventricular filling and inotropism: etomidate and titrated doses of opiates are the best choice. Avoid bradycardia because the volume of systolic ejection is fixed, as well as any increase in pulmonary vascular resistance. Ketamine, the cardiodepressant effect of which can no longer be compensated by an increase in sympathetic tone in this context, has to be used with caution. Risk of intraventricular thrombus.
References :
- Batisse A, Fermont L, Lévy M
Cardiopathie par dysfonction myocardique.
In Cardiologie pédiatrique pratique, 4ème éd. Doin, 2013, p 187-93.
- 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
- Williams GD, Hammer GB.
Cardiomyopathy in childhood.
Curr Opin Anaesthesiol 2011 ; 24 : 289-300.
- Kipps AK, Ramamoorthy C, Rosenthal DN, Williams GD.
Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia.
Pediatr Anesth 2007; 17:775-81.
Updated: July 2021