Double Jeopardy in the Echocardiography Laboratory: Coexistence of Two Distinct Cardiomyopathies?

Background In our Hypertrophic Cardiomyopathy (HCM) Center, we identified 6 patients each with what appeared to be the occurrence of 2 rare diseases that prompted investigation for a common derivative. Methods We reviewed our database, searching for all patients with a diagnosis of HCM, amyloid hear...

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Published inEchocardiography (Mount Kisco, N.Y.) Vol. 31; no. 8; pp. 931 - 935
Main Authors Paterick, Timothy E., Tercius, Alix J., Agarwal, Anushree, Treiber, Shannon C., Khandheria, Bijoy K., Tajik, A. Jamil
Format Journal Article
LanguageEnglish
Published United States Blackwell Publishing Ltd 01.09.2014
Subjects
Adult
amyloid heart disease
Amyloidosis - complications
Amyloidosis - diagnostic imaging
Cardiomyopathy, Hypertrophic - complications
Cardiomyopathy, Hypertrophic - diagnostic imaging
Diagnosis, Differential
Echocardiography - methods
Female
Heart Defects, Congenital - complications
Heart Defects, Congenital - diagnostic imaging
Humans
hypertrophic cardiomyopathy
left ventricular noncompaction
Male
Middle Aged
phenotypic variability
amyloid heart disease
hypertrophic cardiomyopathy
phenotypic variability
left ventricular noncompaction
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Summary:Background In our Hypertrophic Cardiomyopathy (HCM) Center, we identified 6 patients each with what appeared to be the occurrence of 2 rare diseases that prompted investigation for a common derivative. Methods We reviewed our database, searching for all patients with a diagnosis of HCM, amyloid heart disease and left ventricular noncompaction (LVNC). Results Using transthoracic echocardiography and magnetic resonance imaging, we identified 6 patients with apical hypertrabeculations and myocardium suggestive of LVNC; 4 of the patients had HCM and 2 of the patients had cardiac amyloidosis. Conclusions The significance of these possible concomitant cardiomyopathies is not presently well understood. We did not identify a common derivative when looking for a genetic link, but it is most likely hidden in the genetic substrate, yet to be identified.
Bibliography:ark:/67375/WNG-CH7FWZ6F-R
ArticleID:ECHO12502
istex:3EDD8D11FA961F7B05BBE09CE8A84F7C6CB48110
Movie clip S1. Two-dimensional echocardiography in the apical short-axis view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient A.Movie clip S2. Two-dimensional echocardiography in the apical short-axis view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient B.Movie clip S3. Two-dimensional echocardiography in the apical short-axis view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient C.Movie clip S4. Two-dimensional echocardiography in the apical four-chamber view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient D.Movie clip S5. Two-dimensional echocardiography in the apical short-axis view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient E.Movie clip S6. Two-dimensional echocardiography in the apical short-axis view demonstrating bilayered myocardium (noncompacted/compacted > 2) consistent with left ventricular noncompaction in Patient F.
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ISSN:0742-2822
1540-8175
DOI:10.1111/echo.12502