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Incomplete Shone's Complex: Surgical Resolution Through Minimally Invasive Approach
Carlos A. Jimenez Fernandez, MD1, Victor H. Sainz Escarrega, MD
1, Oscar S. Lomeli Sanchez, MD, MSc
1, Francisco J. Gutierrez Lopez, MD
1, Italo D. Masini Aguilera, MD
1, Benigno Ferreira Piña, MD
1, Dalia F. Feliz Alcantara, MD
2;
1Instituto Cardiovascular de Minima Invasion, Zapopan, Mexico,
2Hospital Civil de Guadalajara Fray Antonio Alcalde, Guadalajara, Mexico
BACKGROUND: Anomalies in the mitral valve are frequently reported in congenital diseases, Shone et al. first described a complex in 1963 that consisted of 4 simultaneous anomalies: parachute mitral valve, supra-annular mitral ring, subaortic stenosis, and coarctation of the aorta
METHODS: We present the case of a 16-year-old female with 4 previous surgical events throughout her life; pulmonary artery banding at 9 months old, interventricular septum defect closure at 5 years old, and pulmonary artery reconstruction at age 9.She presented to the outpatient clinic with a history of progressive dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. Initial Eco showed a “hammock sign” and a mean gradient of 17 mm Hg in the mitral valve with deacreased valvular area (1.2 cm2) related to the presence of a supra valvular ring and a hypoplastic posterior leaflet.A minimally invasive approach was proposed due to the high risk of complications related to previous surgeries and the benefits of shorter in-hospital stay, and recovery time
RESULTS: An anterolateral right thoracotomy was made, approaching the posterior interatrial septum through 4 intercostal space. Surgical findings include the presence of a firm supra-annular mitral ring and a hypoplastic posterior leaflet (Figure 1). We place a mechanical prosthesis 27 mm in an annular position.
CONCLUSIONS: The initial definition of Shone`s complex described a multilevel obstruction due to a mitral deformity (parachute), the supra-annular mitral valve ring, sub-aortic stenosis and coarctation of the aorta. Since then individuals with some of these features are referred to as having a partial or incomplete form of this anomaly, which is very well recognized in children. However, reports of this partial form are not so commonly reported in adults. Because of these particularities and the uncommon presentation of these patients, and also variability and severity of the anatomic features, surgical outcomes in adults are not well established and are mostly limited to case reportsLEGEND: Endoscopic Vision of Mitral Valve A) Supravalvular annular fibrous ring B) Exposure of thickened anterior leaflet and hypoplastic posterior leaflet
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