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  • 10 1093@eurheartj@ehw390

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    Alma Medrano
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    1) A 51-year-old woman was referred for a heart murmur and found to have an incomplete Shone's complex - a rare congenital heart condition involving multiple left-sided obstructive lesions. 2) Echocardiogram and cardiac MRI showed she had a parachute mitral valve with anomalous chordae, a rudimentary papillary muscle, and dynamic left ventricular outflow tract obstruction. She also had fixed LVOT obstruction from a subaortic membrane. 3) Imaging findings were consistent with an incomplete Shone's complex, the oldest documented case of this rare congenital heart condition presenting asymptomatically into late adulthood. Multimodality imaging is useful for evaluating

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    1) A 51-year-old woman was referred for a heart murmur and found to have an incomplete Shone's complex - a rare congenital heart condition involving multiple left-sided obstructive lesions. 2) Echocardiogram and cardiac MRI showed she had a parachute mitral valve with anomalous chordae, a rudimentary papillary muscle, and dynamic left ventricular outflow tract obstruction. She also had fixed LVOT obstruction from a subaortic membrane. 3) Imaging findings were consistent with an incomplete Shone's complex, the oldest documented case of this rare congenital heart condition presenting asymptomatically into late adulthood. Multimodality imaging is useful for evaluating

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    31 vues1 page

    10 1093@eurheartj@ehw390

    Transféré par

    Alma Medrano
    1) A 51-year-old woman was referred for a heart murmur and found to have an incomplete Shone's complex - a rare congenital heart condition involving multiple left-sided obstructive lesions. 2) Echocardiogram and cardiac MRI showed she had a parachute mitral valve with anomalous chordae, a rudimentary papillary muscle, and dynamic left ventricular outflow tract obstruction. She also had fixed LVOT obstruction from a subaortic membrane. 3) Imaging findings were consistent with an incomplete Shone's complex, the oldest documented case of this rare congenital heart condition presenting asymptomatically into late adulthood. Multimodality imaging is useful for evaluating

    Droits d'auteur :

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    European Heart Journal Advance Access published September 11, 2016

    Cardiovascular Flashlight 1

    doi:10.1093/eurheartj/ehw390
    CARDIOVASCULAR FLASHLIGHT
    ....................................................................................................................................................
    Incomplete Shone’s complex in the sixth decade of life: echo and cardiac
    magnetic resonance imaging assessment
    Natalia Lorenzo1, Eduard Claver2, Josefina Ali
    o2, and Rio Aguilar3*
    1
    Department of Cardiology, Hospital Universitario Infanta Cristina, Avenida 9 de Junio 2, 28981 Parla, Madrid, Spain; 2Department of Cardiology, Hospital Universitario
    de Bellvitge, Feixa Llarga s/n, 08907 L’Hospitalet de Llobregat, Barcelona, Spain; and 3Department of Cardiology, Hospital Universitario de La Princesa, Diego Leon 62,
    28006 Madrid, Spain
    * Corresponding author. Tel: 134 915202272, Fax: 134 915202201, Email: rioaguilartorres@gmail.com

    A 51-year-old woman was referred to the Cardiology outpatient clinic for a murmur. She was asympthomatic. Transthoracic echocardio-
    gram showed a parachute mitral valve (Panels 1A–C and 2A), with anomalous and elongated chordae converging into a major papillary
    muscle (posteromedial) with two heads (Panels 1B and 2B, see Supplementary material online, Videos S1–S3). Transmitral gradient was nor-
    mal, and non-significant mitral regurgitation was detected. There was a rudimental anterolateral papillary muscle connected with anomalous
    ventricular bands. The elongated subvalvular apparatus protruded into the left ventricular outflow tract (LVOT) generating dynamic LVOT

    Downloaded from http://eurheartj.oxfordjournals.org/ by guest on September 25, 2016


    obstruction (Panels 1B and 2A, see Supplementary material online, Videos S1 and S4). Fibrous subaortic membrane generated fixed LVOT
    obstruction (peak instant gradient was 72 mmHg) (Panels 1D–E, see Supplementary material online, Video S1).
    Cardiac magnetic resonance images supported the echo findings (Panel 2A–B). Magnetic resonance angiogram showed focal narrowing of the
    aorta distal to the left subclavian artery, without significant compromise of the lumen (Panel 2C, see Supplementary material online, Video S5).

    All these abnormalities were consistent with incomplete Shone’s complex.


    Shone’s anomaly is a rare congenital entity, first described in 1963, consisting of left-sided obstructive lesions at multiple cardiovascular
    levels. The classic developmental complex consists of parachute mitral valve, supravalvular ring of the left atrium, subaortic stenosis, and
    aorta coarctation. Since this time, most of the literature comes from isolated reports, and even incomplete forms are still infrequent.
    This congenital complex is mostly detected in childhood. The severity of mitral valve obstruction is the main predictor of prognosis. Our
    case represents the oldest documented patient with a Shone’s anomaly. It illustrates that cases of mitral valve competence may be asympto-
    matic through to late adulthood.
    Shone’s complex must be diagnosed or suspected by echo; however, multimodality imaging allows better evaluation of intra and extra-
    cardiac obstructive lesions.

    Supplementary material is available at European Heart Journal online.

    Published on behalf of the European Society of Cardiology. All rights reserved. V


    C The Author 2016. For Permissions, please email: journals.permissions@oup.com.

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