Shone syndrome

Last revised by Henry Knipe on 04 Oct 2022

Shone syndrome, also known as Shone complex, is a rare syndrome characterized 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 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 utilized 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.

Surgical intervention is required in all cases. Generally, patients will need multiple surgeries at initial presentation, as well as repeated surgeries throughout their life 2,3.

The syndrome is named after John D Shone (1924-2002), an English pediatric cardiologist, who described the constellation of features in his 1963 seminal paper 5.

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Cases and figures

  • Case 1
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