Shone's syndrome
| Shone's syndrome | |
|---|---|
| Other names | Shone's complex, Shone's anomaly, or Shone's disease |
| Specialty | Cardiology |
Shone's syndrome is a rare congenital heart defect described by Shone in 1963. In the complete form, four left-sided defects are present:[1]
- Supravalvular mitral membrane (SVMM)
- Parachute mitral valve
- Subaortic stenosis (membranous or muscular)
- Coarctation of the aorta
Of these four defects, supravalvular mitral membrane (SVMM) is the first to occur and triggers the development of the other three defects. Partial complexes, or forme fruste, have also been described.[1] The definition is often expanded to include lesions of the left side of the heart not originally ascribed to Shone's syndrome, including mitral and aortic valvular lesions and supravalvular aortic stenosis.[2]
The term parachute mitral valve stems from the morphological appearance of the valve; that is to say, the mitral valve leaflets appear as the canopy of the parachute, the chordae as the strings and the papillary muscle as the harness.
- ^ a b Shone JD, Sellers RD, Anderson RC, Adams P, Lillehei CW, Edwards, JE (1963). "The Developmental Complex of "Parachute Mitral Valve," Supravalvular Ring of Left Atrium, Subaortic Stenosis and Coarctation of Aorta". Am J Cardiol. 11 (6): 714–25. doi:10.1016/0002-9149(63)90098-5. PMID 13988650.
- ^ Moustafa, S.E.; Lesperance J.; Rouleau J.L.; Gosselin G. (2008). "A forme frust of Shone's anomaly in a 65 year-old patient". McGill Journal of Medicine. 1. 11 (1): 19–21. PMC 2322915. PMID 18523519.