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. 2012 Apr;14(4):440-4.
doi: 10.1093/icvts/ivr169. Epub 2012 Jan 25.

Surgical outcome of partial Shone complex

Antonio Grimaldi  1 , Anna Chiara Vermi, Siew Yen Ho, Federico Pappalardo, Alessandro Castiglioni, Stefano Benussi, Alberto Zangrillo, Ottavio Alfieri
Affiliations

Surgical outcome of partial Shone complex

Antonio Grimaldi et al. Interact Cardiovasc Thorac Surg. 2012 Apr.

Abstract

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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Figures

Figure 1:
Figure 1:
Examples of the common inflow and outflow obstructive lesions in Shone complex. (a) supramitral shelf (arrows); (b) aortic coarctation; (c) subaortic ridge (open arrow), bicuspid aortic valve (arrows); (d) parachute mitral valve.
Figure 2:
Figure 2:
(a) Transthoracic short-axis views showing asymmetrical mitral orifice (white arrows) with unique papillary muscle. (b) Suprasternal views showing the residual narrowing of the aortic isthmus (yellow arrow). M: mitral valve; Ao: aorta.
Figure 3:
Figure 3:
Transthoracic apical views illustrating a ‘parachute mitral valve’ associated with MS (yellow arrows) and remnants of SVMR (white arrows). LV: left ventricle; LA: left atrium; MS: mitral stenosis; SVMR: supravalvular mitral ring.
Figure 4:
Figure 4:
Surgical outcome and freedom from clinical events in the population study.
See this image and copyright information in PMC

References

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