Canadian Journal of Cardiology
Clinical Research| Volume 33, ISSUE 2, P253-259, February 2017

Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood

Published:September 29, 2016DOI:



      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.


      We reviewed records of adults with complete or incomplete Shone complex followed at the Montreal Heart Institute between 1982 and 2014.


      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.


      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.



      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.


      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.


      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 %).


      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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