Abstract
Background
Shone complex consists of a constellation of left-sided, usually obstructive, cardiac
lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis,
and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly
associated with lesions involving the subaortic region, aortic valve, or thoracic
aorta. There is a paucity of data regarding long-term outcomes in adults with Shone
complex.
Methods
We reviewed records of adults with complete or incomplete Shone complex followed at
the Montreal Heart Institute between 1982 and 2014.
Results
Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35
± 11 years; 50% women) had complete or incomplete Shone complex and were followed
for a median of 8 years. Only 39% were previously diagnosed as having Shone complex.
The most common defects were congenital mitral stenosis (93%), aortic coarctation
(75%), and bicuspid aortic valve (71%). Heart transplantation was required in 2 patients
(7.1%) at age 22 and 28 years, respectively. Overall, 48% had cardiovascular hospitalizations
during adulthood, predominantly for arrhythmias or heart failure. Freedom from cardiovascular
intervention was 55%, 18%, and 8% at 10, 20, and 30 years of age, respectively. Although
aortic coarctation was the most common indication for initial intervention (61%),
adult interventions occurred predominantly for aortic valve/left ventricular outflow
tract (60%) and mitral valve (33%) lesions.
Conclusions
Shone complex is an under-recognized entity associated with relatively low mortality
in adulthood but substantial morbidity related to arrhythmias, heart failure, and
interventions. Increased awareness of this condition and associated complications
may allow for more tailored follow-up.
Résumé
Introduction
Le complexe de Shone consiste en une constellation de lésions généralement obstructives
du cœur gauche, dont l’anneau supravalvulaire mitral, la valve mitrale parachute,
la sténose sous-aortique et la coarctation de l’aorte. La forme partielle du complexe
de Shone consiste en une anomalie de la valve mitrale associée à des lésions touchant
la région sous-aortique, la valve aortique ou l’aorte thoracique. Les données sur
les résultats à long terme chez les adultes atteints du complexe de Shone sont insuffisantes.
Méthodes
Nous avons revu les dossiers d’adultes atteints de la forme partielle ou complète
du complexe de Shone qui avaient été suivis à l’Institut de cardiologie de Montréal
entre 1982 et 2014.
Résultats
Parmi 4189 adultes atteints d’une cardiopathie congénitale, 28 (0,67 %) patients (âge
moyen, 35 ± 11 ans ; 50 % de femmes) étaient atteints de la forme partielle ou complète
du complexe de Shone et avaient été suivis durant une période médiane de 8 ans. Seuls
39 % des patients avaient auparavant reçu un diagnostic de complexe de Shone. Les
anomalies les plus fréquentes étaient la sténose mitrale congénitale (93 %), la coarctation
de l’aorte (75 %) et la bicuspidie aortique (71 %). Une transplantation cardiaque
avait été nécessaire chez 2 patients (7,1 %), à 22 ans et à 28 ans. Dans l’ensemble,
48 % des patients avaient été hospitalisés en raison d’une maladie cardiovasculaire
à l’âge adulte, principalement pour des arythmies ou une insuffisance cardiaque. L’absence
d’intervention cardiovasculaire avait respectivement été de 55 %, de 18 % et de 8
% chez les patients de 10, 20 et 30 ans. Bien que la coarctation de l’aorte fût l’indication
la plus fréquente pour une première intervention (61 %), les interventions chez les
adultes étaient principalement survenues en raison de lésions de la chambre de chasse
du ventricule gaucheet de la valve aortique (60 %) et de la valve mitrale (33 %).
Conclusions
Le complexe de Shone est une entité peu reconnue qui est associée à une mortalité
relativement faible à l’âge adulte, mais à une morbidité substantielle liée aux arythmies,
à l’insuffisance cardiaque et aux interventions. Une sensibilisation accrue à cette
affection et à ses complications associées peut permettre un suivi plus adapté.
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Article info
Publication history
Published online: September 29, 2016
Accepted:
September 26,
2016
Received:
July 7,
2016
Footnotes
See editorial by Opotowsky and Webb, pages 214–215 of this issue.
See page 258 for disclosure information.
Identification
Copyright
© 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
