Key result
Surgery for partial Shone complex before age five is linked to ~100% survival without major events.
Why the study?
Does surgical intervention improve survival and event-free follow-up in adult patients with partial Shone complex?
Observational (n=9)
Does surgical intervention improve survival and event-free follow-up in adult patients with partial Shone complex?
Adult patients with partial Shone complex who are properly diagnosed and surgically treated demonstrate favorable long-term outcomes free from major clinical events.
May support early surgery in partial Shone complex; leaves open validation in prospective adult cohorts.
Partial forms of Shone complex are rare. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of surgically treated adult partial complex. Between 2001 and 2011, nine patients (age: 38 ± 8 years; six males, 67%) were referred for valvular heart disease. Transthoracic and transoesophageal echocardiography was performed. Data were confirmed by intra-operative findings and reports. Patients were diagnosed as partial Shone complex and presented with mitral stenosis (MS) (45%) or mitral regurgitation (22%) or aortic regurgitation (22%). All but one patient (89%) reported previous surgery: coarctation of the aorta repair (87.5%) and aortic valvulotomy (12.5%). Redo intervention included: mitral valve replacement (25%), mitral repair (25%), aortic valve replacement (37.5%) and subvalvular aortic ridge resection (25%). One patient refused surgery. Patients surgically treated before the age of 5 (87.5%) showed favourable outcome (survival rate: 100%) and a 23.6 (± 4.6)-year follow-up free from events. The patient who underwent first intervention at the age of 50 and the patient with MS who refused surgery showed a 45 (± 7)-year follow-up free from major morbidity. Patients with partial Shone complex, properly diagnosed and treated, show favourable surgical outcome free from major clinical events.
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Grimaldi et al. (2012) conducted an observational in Partial Shone complex (n=9). Surgical treatment was evaluated on Survival and freedom from major clinical events. Surgical treatment of partial Shone complex before age 5 was associated with a 100% survival rate and 23.6 years free from major clinical events.
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