Very rare congenital heart malformation consisting of the variable association of 4 obstructive lesions of the left heart:

-        mitral supravalvular ring (2)

-        parachute-like mitral valve deformation (1), 

-        subaortic stenosis (3) (or bicuspid aortic valve)

-        hypoplasitic aortic arch or aortic coarctation (4)

The most common clinical presentation is aortic coarctation or hypoplastic aortic arch in the neonatal period.

Anesthetic implications:

exclude other lesions in case of coarctation of the aorta; management of a mitral and/or aortic stenosis. Major risk of postcapillary pulmonary hypertension

References : 

• Sachse K, Hannallah M. 
  The anesthetic management for cesarean delivery in a patient with Shone’s syndrome. 
  Anesth Analg 2008; 107: 1652-4.
• Naz A, Dasgupta S, Bandyopadhyay BK, Shirazee HH.
  Graded epidural anaesthesia for Caesarian section in a parturient with Shone’s syndrome: a case study.
  SAJAA 2016; 22: 41-4.
• Bhatia K, Eccles J, Meessala DK.
  Anesthetic management of a parturient with Shone’s syndrome : a case report with review of literature.
  Korean J Anesth 2021 ; 74 ;342-9.

Updated: December 2021