Multiple Left Heart Obstructions (Shone’s Anomaly) With Mitral Valve Involvement: Long-Term Surgical Outcome

Brauner, R.; Laks, Hillel; Drinkwater, Davis C.; Scholl, Frank G.; McCaffery, Sharon

doi:10.1016/s0003-4975(97)00632-2

Cited by 103 publications

(90 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Bolling et al 18 described the operative results and late outcomes of 30 patients with multiple levels of left-heart obstruction with mitral valve involvement, 26 of whom had PMV, and concluded that increasing severity of the mitral valve obstruction was associated with worsening long-term outcome and that operative mortality in patients with Shone's anomaly is adversely affected by the degree of mitral valve disease. Brauner et al 19 also reported the long-term surgical outcome of 19 patients with multiple left-heart obstructions, 12 of whom had PMV, and concluded that worsening late outcome was associated with increased mitral valve involvement and the degree of pulmonary hypertension. Worsening outcomes after mitral valve surgery for congenital mitral stenosis are also reported to be associated with the severity of left ventricular outflow tract obstruction.…”

Section: Discussionmentioning

confidence: 99%

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

2004

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

2004

View full text Add to dashboard Cite

show abstract

“…2,3,9 MV hypoplasia and stenosis have been associated with higher mortality after biventricular repair for patients with multiple left heart obstructions. 2,3,9 Others have found preoperative pulmonary hypertension to be another important risk factor. 2 Most of the patients in the present series underwent only noninvasive preoperative evaluation, and direct measurement of right ventricular pressure was frequently not available.…”

Section: Discussionmentioning

confidence: 99%

“…1 However, progressive, recurrent, or newly developed obstructive lesions, as well as persistent or progressive pulmonary hypertension, may complicate the clinical course after biventricular repair. [2][3][4] Moreover, the early surgical strategy is seldom reversible. Some patients require takedown to a single-ventricle palliation or heart transplantation, but most patients who fail a biventricular approach ultimately die.…”

mentioning

confidence: 99%

Predictors of Outcome of Biventricular Repair in Infants With Multiple Left Heart Obstructive Lesions

2001

View full text Add to dashboard Cite

Background-Decisions regarding surgical strategy in patients with multiple left heart obstructive or hypoplastic lesions often must be made in the newborn period and are seldom reversible. Predictors of outcome of biventricular repair have not been well defined in this heterogeneous group of patients, and risk factors described for critical aortic valve stenosis have been shown to be inapplicable to patients with other left heart obstructive lesions. The goal of this study was to identify echocardiographic predictors of outcome of biventricular repair for infants with multiple left heart obstructive lesions. Methods and Results-Patients with Ն2 areas of left heart obstruction or hypoplasia, diagnosed at Յ3 months of age, who had not previously undergone surgical or catheter intervention and maintained biventricular physiology were included (nϭ72). Failure of biventricular repair was defined as takedown to a univentricular repair, cardiac transplantation, and/or death (nϭ14; 19%). This group was compared with the patients who survived a biventricular approach (nϭ58

show abstract

“…(27) In addition to valve morphology, more complete phenotypic classifications also include typing of aortic dilation. (25) In most patients, a BAV is an isolated anomaly, but can be more prevalent in certain conditions ( (47)(48)(49) 23% (50) 84 -89% (51,52) 39 -47% (53,54) 14 -30% (56)(57)(58) 5 -11% (55,59) Notes Large variability due in part due to heterogeneous reporting and nomenclature Subaortic stenosis predisposes to valve damage leading to aortic regurgitation Recurrence of subaortic stenosis after resection is common (20%) BAV was not initially described as part of the Shone complex Frequent structural abnormalities of aortic valve are present (55) 95% show R-L cusp fusion High prevalence of both ascending aortic dilation and aortic coarctation…”

Section: Phenotypementioning

confidence: 99%

Bicuspid aortic valve disease

Lee

Otto

2017

SAHJ

View full text Add to dashboard Cite

and pulmonary valves with the leaflets developing via a process of cavitation into three distinct layers (fibrosa, spongiosa, and ventricularis). (11) The development of the aorta is also a complex process in which the common truncus arteriosus is septated into the aorta and pulmonary arteries. Abnormalities in septation are the cause of common congenital heart defects such as tetraology of Fallot. This process is also likely related to the migration of cardiac neural crest cells from the pharyngeal arches and heart; (10) and these neuronal crest cells may be similar in origin to the cells involved in the development of the aortic outflow tract and aortic valve.

show abstract

Multiple Left Heart Obstructions (Shone’s Anomaly) With Mitral Valve Involvement: Long-Term Surgical Outcome

Cited by 103 publications

References 19 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

Predictors of Outcome of Biventricular Repair in Infants With Multiple Left Heart Obstructive Lesions

Bicuspid aortic valve disease

Contact Info

Product

Resources

About