The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta

Shone, John; Sellers, Robert D.; Anderson, Ray C.; Adams, Paul; Lillehei, C. Walton; Edwards, Jesse E.

doi:10.1016/0002-9149(63)90098-5

Cited by 558 publications

(281 citation statements)

References 4 publications

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“…4 The unifocal attachment of the chordae results in a restricted valve opening and the potential for subvalvar obstruction and, less frequently, valvar regurgitation. PMV is rarely found as an isolated lesion and may occur as part of an anomaly complex described by Shone et al 5 in 1963, consisting of a PMV, supravalvular ring of the left atrium, subaortic stenosis, and coarctation of the aorta, with both complete and incomplete forms having been described. The outcome of children with these multilevel left-heart obstructions and mitral valve involvement is generally poor.…”

mentioning

confidence: 99%

Independent Factors Associated With Outcomes of Parachute Mitral Valve in 84 Patients

2004

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Background-Parachute mitral valve (PMV) is defined as a unifocal attachment of the mitral valve chordae to a single or dominant papillary muscle and may cause subvalvar obstruction. We sought to determine factors associated with outcomes. Methods and Results-Patients (nϭ84; 64% male) who presented between 1977 and 2001 at a median age of 3 days (range, birth to 5.4 years) were assessed with PMV (without atrioventricular septal defect). Associated cardiac anomalies in 99% included aortic coarctation in 68%, atrial septal defect in 54%, ventricular septal defect in 46%, aortic valve stenosis in 32%, subaortic stenosis in 20%, and left ventricular hypoplasia in 19%, with complex anomalies in 14%. Noncardiac anomalies were noted in 32%. Survival (nϭ18 deaths) was 82% at 1 year and 79% at 10 years, with independent risk factors including left ventricular hypoplasia (PϽ0.001) and atrial septal defect (PϽ0.003). Freedom from surgical mitral valvotomy (nϭ11 patients) was 95% at age 6 months and 80% at 10 years, with independent risk factors including the absence of aortic coarctation (PϽ0.02) and the presence of subaortic stenosis (PϽ0.04). There was no significant increase in mean gradient of the PMV over time, but higher gradient was independently associated with the presence of supravalvar mitral stenosis (PϽ0.001), absence of atrial septal defect (PϽ0.04), presence of ventricular septal defect (PϽ0.02), and subsequent mitral valvotomy (PϽ0.01). Conclusions-Outcomes for patients with PMV are dependent on the spectrum of associated cardiac lesions. The degree of mitral valve obstruction remains stable, and the majority will not require valvotomy.

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confidence: 99%

Independent Factors Associated With Outcomes of Parachute Mitral Valve in 84 Patients

2004

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“…Shone's, complex is a syndrome consisting of four components, first described by Shone et al 2 Absence of any one of these component forms an incomplete Shone's complex, which is more common than the complete form. 1 Suspected to be due to mitral inflow obstruction during fetal development, it leads to under development of left ventricle (LV) and varying degrees of left outflow obstruction.…”

Section: Discussionmentioning

confidence: 99%

Role of TEE in Improving Diagnostic Accuracy of Congenital Heart Disease

Rana¹,

Kumar²,

J³

2014

Journal of Perioperative Echocardiography

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Complex congenital heart diseases (CHD) often present as multiple cardiac lesions. The presence of one anomaly should stimulate the physician to perform a comprehensive assessment and look for other associated anomalies.1 Multimodal imaging may be necessary to diagnose such associated lesions as single imaging may occasionally miss them. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are complimentary to each other. Routine use of TEE during intraoperative period may help us to refine diagnosis, detect the missing components and guide effective surgical repair. We present one such case where diagnosis and management were optimized by multimodal imaging.

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“…(1) The developmental complex may exist in its complete form when all four lesions are present or it may be incomplete.…”

Section: Shone's Complexmentioning

confidence: 99%

Shone’s complex

Takawira

Sinyangwe

Mwangi

et al. 2017

SAHJ

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The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta

Cited by 558 publications

References 4 publications

Independent Factors Associated With Outcomes of Parachute Mitral Valve in 84 Patients

Independent Factors Associated With Outcomes of Parachute Mitral Valve in 84 Patients

Role of TEE in Improving Diagnostic Accuracy of Congenital Heart Disease

Shone’s complex

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