Shone syndrome

Shone Syndrome is a rare and complex congenital heart disease characterized by four left-sided heart defects. Named after Dr. John Shone who first described the condition in 1963, the syndrome is also known as Shone's Complex or Shone's Anomaly.

Overview[edit | edit source]

Shone Syndrome involves four primary defects: parachute mitral valve, subaortic stenosis, supravalvular mitral ring, and coarctation of the aorta. These defects can obstruct blood flow from the left side of the heart to the body, leading to various health complications.

Symptoms[edit | edit source]

Symptoms of Shone Syndrome can vary greatly depending on the severity of the defects. Common symptoms include shortness of breath, fatigue, rapid breathing, and poor weight gain in infants. In severe cases, the condition can lead to heart failure.

Diagnosis[edit | edit source]

Diagnosis of Shone Syndrome typically involves a combination of echocardiography, cardiac catheterization, and magnetic resonance imaging (MRI). Early diagnosis is crucial for effective treatment and management of the condition.

Treatment[edit | edit source]

Treatment for Shone Syndrome is primarily surgical and aims to correct or alleviate the four defects. This may involve valve repair or replacement, removal of the subaortic membrane, and repair of the aortic coarctation. Post-surgery, patients often require lifelong follow-up care and may need additional surgeries or interventions.

Prognosis[edit | edit source]

The prognosis for individuals with Shone Syndrome varies depending on the severity of the defects and the success of surgical interventions. With early diagnosis and appropriate treatment, many individuals with Shone Syndrome can lead healthy lives.

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Brown atrophy of the heart

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Keshan disease

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Loeffler endocarditis

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Nonbacterial thrombotic endocarditis

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Traumatic cardiac arrest

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Waon therapy

NIH genetic and rare disease info[edit source]

NIH info on Shone syndrome

Shone syndrome is a rare disease.

Shone syndrome Resources
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