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Surgical outcome of partial Shone complex.
Antonio Grimaldi1, Anna Chiara Vermi, Siew Yen Ho
1Cardiovascular and Thoracic Department, San Raffaele Scientific Institute and Università Vita-Salute San Raffaele, Milan, Italy. antonio.grimaldi@hsr.it
Interactive Cardiovascular and Thoracic Surgery
|January 27, 2012
Summary
Partial Shone complex, a rare condition, shows favorable surgical outcomes in adults when properly diagnosed and treated. Early surgical intervention, especially before age five, leads to excellent long-term results and event-free survival.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Surgery
Background:
- Partial Shone complex is a rare congenital heart defect with limited long-term surgical outcome data for adults.
- Complete forms of Shone complex often have poor surgical outcomes.
- Adults with partial Shone complex present with diverse valvular heart disease, frequently requiring re-intervention.
Purpose of the Study:
- To evaluate the long-term surgical outcomes of adult patients with partial Shone complex.
- To identify factors influencing the success of surgical interventions in this population.
- To provide data on the efficacy of surgical management for partial Shone complex.
Main Methods:
- Retrospective analysis of nine adult patients diagnosed with partial Shone complex between 2001 and 2011.
- Diagnostic methods included transthoracic and transoesophageal echocardiography, confirmed by intra-operative findings.
- Surgical interventions comprised mitral valve procedures, aortic valve replacement, and subvalvular aortic ridge resection.
Main Results:
- The majority of patients (89%) had prior cardiac surgery, primarily for coarctation of the aorta.
- Surgically treated patients, particularly those operated on before age five, demonstrated a 100% survival rate with a mean follow-up of 23.6 years, free from major events.
- One patient undergoing initial intervention at age 50 and another with mitral stenosis who refused surgery had a 45-year follow-up with no major morbidity.
Conclusions:
- Partial Shone complex, when accurately diagnosed and surgically managed, yields favorable long-term outcomes in adults.
- Early surgical intervention in childhood appears to be a critical factor for achieving excellent long-term, event-free survival.
- Timely and appropriate surgical treatment is associated with favorable outcomes and low morbidity in adult patients with partial Shone complex.