Shone Complex

Ann Thorac Surg. 2005 Apr;79(4):1358-65.
Operative results and outcomes in children with Shone's anomaly.
Brown JW, Ruzmetov M, Vijay P, Hoyer MH, Girod D, Rodefeld MD, Turrentine MW.
Section of Cardiothoracic Surgery, James Whitcomb Riley Hospital for Children and Indiana University School of Medicine, Indianapolis, Indiana 46202-5123, USA. jobrown@iupui.edu

BACKGROUND: The outcome of children with multilevel left heart obstructions with mitral valve involvement (Shone's anomaly) is generally poor. This study reviews our results and outcomes in patients with staged repair for left ventricular outflow tract obstruction (LVOTO) and mitral valve reconstruction. METHODS: A total of 27 children underwent staged repair for Shone's anomaly between 1978 and 2003. The mean age at the first operation was 9.0 months (range, 2 days to 3 years). Mitral stenosis was present in all; with supravalvular mitral ring (n = 11), mitral valve abnormalities including parachute mitral valve, fused chordae, single papillary muscle (n = 10), and "typical" (Ruckman & Van Praagh) congenital mitral stenosis (n = 14). The LVOT obstruction features included subaortic stenosis (n = 16), valvar aortic stenosis (n = 7), bicuspid aortic valve (n = 24), and coarctation (n = 18). All 27 patients underwent 94 surgical procedures, including 22 mitral operations and 48 LVOT operations. RESULTS: There were no operative deaths at the first operation. Mean follow-up was 4.7 +/- 4.4 years (range, 6 months to 15 years). There were three late deaths (11%). All late deaths were secondary to severe mitral valve disease. Overall 15-year actuarial survival was 89%. All surviving patients are in New York Heart Association functional class I or II. CONCLUSIONS: Patients with Shone's anomaly are seen with a wide spectrum of major anatomic and hemodynamic abnormalities. A favorable outcome is possible for most. Operative mortality is adversely affected by the severity of mitral valve disease, the degree of left ventricular hypoplasia, and the need for multiple operative procedures.

Circulation. 2004 May 18;109(19):2309-13. Epub 2004 Apr 26.
Independent factors associated with outcomes of parachute mitral valve in 84 patients.
Schaverien MV, Freedom RM, McCrindle BW.
Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

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 (n=84; 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 (n=18 deaths) was 82% at 1 year and 79% at 10 years, with independent risk factors including left ventricular hypoplasia (P<0.001) and atrial septal defect (P<0.003). Freedom from surgical mitral valvotomy (n=11 patients) was 95% at age 6 months and 80% at 10 years, with independent risk factors including the absence of aortic coarctation (P<0.02) and the presence of subaortic stenosis (P<0.04). 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 (P<0.001), absence of atrial septal defect (P<0.04), presence of ventricular septal defect (P<0.02), and subsequent mitral valvotomy (P<0.01). 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.

Arch Cardiol Mex. 2001 Jul-Sep;71(3):206-13.
Congenital mitral stenosis. Experience in 1991-2001
Alva C, Gonzalez B, Melendez C, Jimenez S, Jimenez D, David F, Sanchez A, Ortegon J, Ledesma M, Magana JA, Arguero R.
Hospital de Cardiologia, Centro Medico Nacional Siglo XXI, Instituto Mexicano del Seguro Social.
alval@prodigy.net.mx

OBJECTIVE: To describe ten years of experience with congenital mitral stenosis. METHOD: All cases with congenital mitral stenosis from January 1991 to 2001 were analized. RESULTS: 16 patients with congenital mitral stenosis were found, mean age 3 +/- 3.08 years. TYPES OF OBSTRUCTIONS: Mitral commisures combined with tendinous cords fusion (45%), parachute mitral valve 37%, supravalvar mitral ring in one, fusion between papillary muscle and leaflets in one, and double mitral orifice in one. Hemodynamic (in mmHg) findings: wedge pressure 18.4 +/- 4, pulmonary artery systolic 61 +/- 21, diastolic 34 +/- 15, mean 47 +/- 18 mmHg, mean transvalvar gradient (MTG) 12.9 +/- 7.3, PRU 5.5 +/- 3.8. Four patients received medical treatment. Surgical results: Six patie nts underwent mitral repair, and four were subjected to mitral valve replacement (two with previous mitral repair). Global mortality was 2 (12.5%). Follow-up was 3.5 +/- 3 years. There was a significant difference when the initial MTG 22.6 +/- 11.5 mmHg b efore surgical repair was compared with the final MTG 4.75 +/- 1.5 (P < 0.05), also when initial MTG 12 +/- 2.3 before mitral valve replacement was compared with the final MTG 6 +/- 1.15 mmHg (P < 0.05). CONCLUSION: Most patients are candidates for mitral surgical repair, however at mid term one third will require mitral valve replacement.

Echocardiography. 1996 Mar;13(2):147-150.
A Form Fruste of Shone's Complex Diagnosed by Transesophageal Echocardiography.
Patel JJ, Ross JJ, Chandrasekaran K.
Cardiac Ultrasound Laboratory, The University of Oklahoma Health Sciences Center, P.O. Box 26901, South Pavilion, Rm. 5SP300, Oklahoma City, OK 73190.

Shone's complex is a developmental complex of parachute mitral valve, supravalvular ring of the left atrium, subaortic stenosis, and coarctation of the aorta. Little clinical experience has been accumulated since the original description by Shone et al. T his may be due to underrecognition of the condition by transthoracic echocardiography (TTE). We describe a forme fruste of Shone's complex which is definitively diagnosed only after precise demonstration of the anatomy by transesophageal echocardiography (TEE). Demonstration of these abnormalities by TEE was of little clinical significance in our patient, but may be important in others in whom prior TTE has been suboptimal.

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