News & Perspective
Drugs & Diseases
CME & Education
Academy
Video
Decision Point
Edition: English

Medscape

English
Deutsch
Español
Français
Português
UKNew

Univadis

Sign Up It's Free!
English Edition

Medscape

Univadis

    X
    Univadis from Medscape

    No Results

      News & Perspective Drugs & Diseases CME & Education Academy Video Decision Point
      Drugs & Diseases > Pediatrics: Cardiac Disease and Critical Care Medicine

      Pediatric Restrictive Cardiomyopathy Workup

      Updated: Sep 18, 2019
      • Author: Kimberly Y Lin, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
      • Print
      Workup

      Approach Considerations

      Laboratory studies generally do not contribute to the diagnosis of restrictive cardiomyopathy (RCM). Echocardiography is often diagnostic and is very useful in distinguishing RCM from constrictive pericarditis (CP); cardiac computed tomography (CT) or magnetic resonance imaging (MRI) may also be a helpful adjunct in this respect. Cardiac catheterization is generally indicated to assess hemodynamics. Electrocardiography (ECG) usually reveals abnormalities. Endomyocardial biopsy may reveal a specific cause but appears to be much more helpful in adults than in children.

      Next:

      Echocardiography

      Echocardiography is often diagnostic of RCM. Results usually include marked atrial enlargement with normal left ventricular end-diastolic dimensions (see the image below). Ventricular hypertrophy and atrioventricular valve dysfunction may also be present. Atrial thrombi may be seen. Pulmonary vein atrial flow reversal duration that exceeds mitral a wave duration has also been described. Duration of pulmonary vein a wave appears to have positive correlation with degree of elevated left ventricular end-diastolic pressure; this measure seems to exhibit high degree of sensitivity and specificity. [31]

      Echocardiographic 4-chamber view of a child with r Echocardiographic 4-chamber view of a child with restrictive cardiomyopathy demonstrating characteristic marked enlargement of right atrium (RA) and left atrium (LA), which are larger than left ventricle (LV).

      Echocardiography can be very useful in distinguishing RCM from CP. [32, 33] For example, echocardiographic evidence of minimal respiratory variation in Doppler ventricular inflow signals is observed in patients with RCM; in comparison, significant respiratory variation is observed in patients with CP. [34] Prolongation of the systole-to-diastole ratio is observed in pediatric RCM, although this abnormality is also found in children with dilated cardiomyopathy. Mid-diastolic reversal of flow across mitral and tricuspid valves is also more common in RCM.

      Unfortunately, Doppler echocardiographic findings still show overlap between RCM and CP. Echocardiography may reveal a thickened pericardium in patients with CP.

      Previous
      Next:

      Electrocardiography

      ECG usually reveals evidence of atrial enlargement and ST-T wave changes. Infiltrative diseases can have low voltage changes. An arrhythmia (eg, atrial fibrillation) may be present. Familial RCM can be associated with atrioventricular block.

      Because of risk of sudden deterioration and death, some investigators recommend serial ECG and Holter monitoring to observe for evidence of ischemia and arrhythmia. [35]

      Previous
      Next:

      Cardiac Catheterization

      Pulmonary artery pressures are usually elevated. Right and left ventricular end-diastolic pressures are elevated, and the 2 ventricular end-diastolic pressures are generally discordant, with left ventricular end-diastolic pressure usually being significantly higher (>5 mm Hg) than right ventricular end-diastolic pressure.

      The systolic area index, which uses the ratio of right-to-left ventricular systolic pressure-time area during inspiration and expiration as measured during cardiac catheterization, has also been proposed as a means of differentiating between RCM and CP. [36]

      Previous
      Next:

      Other Studies

      On chest radiography, heart size is usually increased, often with evidence of right or left atrial enlargement. Pulmonary venous congestion is often evident.

      Computed tomography (CT) and magnetic resonance imaging (MRI) may be useful for assessing pericardial thickness in patients for whom CP is in the differential diagnosis.

      Previous
      Next:

      Tissue Analysis and Histologic Findings

      Endomyocardial biopsy may reveal a specific cause but appears to be much more helpful in adults than in children. Thus, indications must be individualized and balanced with the risks of the procedure. [37] Guidelines for the role of endomyocardial biopsy in the management of cardiovascular disease have been proposed. [38]

      Findings from biopsy or autopsy are usually abnormal but are not necessarily diagnostic. Varying degrees of myocyte hypertrophy, interstitial fibrosis, myocytolysis, and endocardial sclerosis have been found. In those patients (usually adults) with an infiltrative cause, such as amyloidosis, biopsy findings may be diagnostic.

      Previous
      Treatment & Management
       
       
      Media Gallery
      of 1
      Find Us On
      About
      About Medscape Privacy Policy Editorial Policy Cookies Terms of Use Advertising Policy Help Center
      Membership
      Become a Member About You Professional Information Newsletters & Alerts Market Research
      App
      Medscape
      WebMD Network
      Medscape Live Events WebMD MedicineNet eMedicineHealth RxList WebMD Corporate Medscape UK
      Editions
      English Deutsch Español Français Português UK
      All material on this website is protected by copyright, Copyright © 1994-2023 by WebMD LLC. This website also contains material copyrighted by 3rd parties.