Shone syndrome, also known as Shone complex, is a rare syndrome characterised by left-sided, obstructive congenital heart defects.
Shone syndrome is thought to be very rare, accounting for less than 1% of all congenital heart disease 1.
Patients, usually neonates and infants, present with signs and symptoms of congestive cardiac failure 1-3. On clinical examination, patients may have murmurs heard over both the aortic and mitral areas during precordial auscultation 4.
The complete Shone syndrome consists of four cardinal left-sided cardiac defects:
- supravalvular mitral membrane 1-3
- subvalvular aortic stenosis (membranous or muscular) 1-3
- parachute mitral valve 1-3
- coarctation of the aorta 1-3
Although four features were originally described, it is more common for patients to have incomplete Shone syndrome with only some of the obstructive lesions 3.
Case studies have utilised both echocardiography and CT in order to identify each of the four obstructive cardiac defects in Shone syndrome 1,4.
See individual articles for a detailed description of each defect.
Treatment and prognosis
Surgical intervention is required in all cases 2,3. Generally, patients will need multiple surgeries at initial presentation, as well as repeated surgeries throughout their life 2,3.
History and etymology
The syndrome is named after John D Shone (1924-2002), an English paediatric cardiologist, who described the constellation of features in his 1963 seminal paper 5.
- 1. Popescu BA, Jurcut R, Serban M, Parascan L, Ginghina C. Shone's syndrome diagnosed with echocardiography and confirmed at pathology. European journal of echocardiography : the journal of the Working Group on Echocardiography of the European Society of Cardiology. 9 (6): 865-7. doi:10.1093/ejechocard/jen200 - Pubmed
- 2. Bolling SF, Iannettoni MD, Dick M et-al. Shone's anomaly: operative results and late outcome. Ann. Thorac. Surg. 1990;49 (6): 887-93. Pubmed citation
- 3. St Louis JD, Bannan MM, Lutin WA, Wiles HB. Surgical strategies and outcomes in patients with Shone complex: a retrospective review. The Annals of thoracic surgery. 84 (4): 1357-62; discussion 1362-3. doi:10.1016/j.athoracsur.2007.05.003 - Pubmed
- 4. Ganju NK, Kandoria A, Thakur S, Ganju SA. A Constellation of Cardiac Anomalies: Beyond Shone's Complex. Heart Views : The Official Journal of the Gulf Heart Association. 17 (1): 23. doi:10.4103/1995-705X.182643 - Pubmed
- 5. Shone JD, Sellers RD, Anderson RC, Adams P, Lillehei CW, Edwards JE. The developmental complex of "parachute mitral valve," supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. The American journal of cardiology. 11: 714-25. Pubmed
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt