Mitral valve morphology and morbidity/mortality in Shone's complex

Ikemba, Catherine; Eidem, Benjamin W.; Fraley, J. Kennard; Eapen, Reenu S.; Pignatelli, Ricardo H.; Ayres, Nancy A.; Bezold, Louis I.

doi:10.1016/j.amjcard.2004.10.030

Cited by 42 publications

(32 citation statements)

References 12 publications

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“…Because coarctation repair can unmask effects of intra-cardiac obstructive lesions, it is advisable to closely monitor patients with structurally abnormal mitral valves with serial echocardiograms to prevent delayed intervention. Ikemba and colleagues [11] identified that the presence of thickened leaflets and shortened chordae by echocardiography were predictive of the need for mitral operation as well as death.…”

Section: Commentmentioning

confidence: 99%

Outcomes of Reparative and Transplantation Strategies for Multilevel Left Heart Obstructions With Mitral Stenosis

Malhotra¹,

Lacour-Gayet²,

Campbell³

et al. 2008

The Annals of Thoracic Surgery

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Background Conventional management for multilevel left heart obstructions and mitral stenosis (Shone’s complex) involves multiple operations that carry additive risks. This study reviews our experience with reconstructive and transplantation approaches for Shone’s complex. Methods Between 1987 and 2007, 43 patients with mitral stenosis and one or more left-sided obstructions were identified: supramitral ring (n = 13), subaortic stenosis (n = 25), aortic stenosis (n = 24), hypoplastic arch (n = 20), and coarctation (n = 38). Thirty patients underwent a staged reparative approach, including 27 mitral and 51 left ventricular outflow tract operations. Thirteen patients were referred for transplantation. Patients with severe hypoplasia of the left ventricle were excluded. Results There was one in-hospital death (2.5%) and six late deaths (14.2%). Actuarial 5- and 10-year survival for staged surgical and transplantation was 88% vs 61.3% and 83.1% vs 61.3% (p = 0.035). At a mean follow-up of 7.9 years, freedom from mitral reoperation was 83.3% and freedom from reoperation for subaortic stenosis was 78.0%. Wait-list mortality was 13.3% (2 of 13). Wait-list time exceeding 90 days was an incremental risk factor for death after transplantation (p = 0.005). Conclusions Despite the challenges of a reparative strategy for Shone’s complex, favorable survival and durability outcomes can be expected. Heart transplantation, although avoiding the pitfalls of staged repair, confers increased risks from ongoing physiologic derangements due to uncorrected left heart inflow and outflow obstructions during the wait for donor heart availability.

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Section: Commentmentioning

confidence: 99%

Outcomes of Reparative and Transplantation Strategies for Multilevel Left Heart Obstructions With Mitral Stenosis

Malhotra¹,

Lacour-Gayet²,

Campbell³

et al. 2008

The Annals of Thoracic Surgery

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“…The obstructive lesions in Shone's complex have a tendency to worsen over time as compared to other congenital heart defects [14] . The antenatal recognition of Shone's syndrome is still challenging, the complex hemodynamic interactions between multiple levels of left-side obstructions challenge the clinical determination of prognoses [15] and sometimes the diagnosis at mid-gestation is misleading, as both steady state and worsening by the end point are possible [5] .…”

Section: Discussionmentioning

confidence: 99%

Prenatal Diagnosis of Shone’s Syndrome by Fetal Echocardiography

Li¹,

Wu²,

Sun³

2020

Preprint

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“…Some cases are even diagnosed in adulthood [3][4][5]. For the most severe cases, the prognosis continues to be poor [6][7][8][9]. Our patient was unique because in addition to the left obstructive lesions he also suffered from right-sided anomalies already diagnosed on his initial echocardiogram at age of 2 days: the tricuspid valve was dysplastic with moderate stenosis, mild-to-moderate tricuspid regurgitation, and a small annulus (Z-score, − 1.08).…”

Section: Discussionmentioning

confidence: 99%