Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions

Figures

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  Figure 4. Main aetiologies of restrictive cardiomyopathy according to the age of disease presentation.

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  Figure 1. Suggested flow-chart for initial approach to patient with heart failure and suspected restrictive cardiomyopathy. CXR chest X-ray, ECG electrocardiogram; MR magnetic resonance, Nt-pro-BNP N-terminal pro B type Natriuretic Peptide, RCM-restrictive cardiomyopathy.

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  Figure 2. A 6-year-old boy with restrictive cardiomyopathy. Electrocardiogram shows abnormal P waves suggestive of atrial enlargement and abnormal ventricular repolarization suggestive of biventricular pressure overload.

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  Figure 3. (A) Chest X-ray, 8-year-old boy with hypertrophic-restrictive cardiomyopathy and congestive heart failure. (B) Echocardiography apical 4-chambers view, 11-year-old girl with restrictive cardiomyopathy, normal ventricular volumes, preserved ejection fraction and severe atrial dilatation; we can appreciate the presence of an implantable cardiac defibrillator lead. (C) Echocardiography parasternal long axis view, 6-year-old boy with restrictive cardiomyopathy associated to severe left atrial dilatation.

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  Figure 5. Flow-chart management of paediatric restrictive cardiomyopathy). Right heart catheterization is usually indicated for all patients referred for HT. However, it is useful to stratify prognosis in patients in NYHA class I to II since severe PH and low cardiac output can develop with mild or subtle symptoms. DPG, diastolic pulmonary gradient; HT, heart transplantation; PVR, pulmonary vascular resistance; RHC, right heart catheterization; TPG, transpulmonary gradient.