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A Constellation of Cardiac Anomalies: Beyond Shone's Complex.

ABSTRACT: Shone's anomaly is a very rare congenital cardiac malformation characterized by four serial obstructive lesions of the left side of the heart (i) Supravalvular mitral membrane (ii) parachute mitral valve (iii) muscular or membranous subaortic stenosis and (iv) coarctation of aorta. We report a unique presentation of Shone's complex in a 14-year-old adolescent male. In addition to the four characteristic lesions the patient had bicuspid aortic valve, aneurysm of sinus of valsalva, patent ductus arteriosus, ventricular septal defect, persistent left superior vena cava opening into coronary sinus and severe pulmonary artery hypertension. This case report highlights the importance of a strong clinical suspicion of the coexistence of multiple congenital cardiac anomalies in Shone's complex and the significance of a careful comprehensive echocardiography.

SUBMITTER: Ganju NK

PROVIDER: S-EPMC4879801 | biostudies-other | 2016 Jan-Mar

REPOSITORIES: biostudies-other

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A Constellation of Cardiac Anomalies: Beyond Shone's Complex.

Ganju Neeraj K NK Kandoria Arvind A Thakur Suresh S Ganju Sunite A SA

Heart views : the official journal of the Gulf Heart Association 20160101 1

Shone's anomaly is a very rare congenital cardiac malformation characterized by four serial obstructive lesions of the left side of the heart (i) Supravalvular mitral membrane (ii) parachute mitral valve (iii) muscular or membranous subaortic stenosis and (iv) coarctation of aorta. We report a unique presentation of Shone's complex in a 14-year-old adolescent male. In addition to the four characteristic lesions the patient had bicuspid aortic valve, aneurysm of sinus of valsalva, patent ductus a ...[more]

PMID: 27293526

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Project description:ObjectiveOne-staged surgical treatment of aortic coarctation combined with cardiac anomalies is challenging. We aim to evaluate the feasibility of bilateral aortofemoral bypass technique in one-staged surgery treating coractation by comparing surgical outcomes with catheter intervention plus stent (hybrid).MethodsBetween January 2012 and December 2017, 50 patients underwent one-staged surgical procedures to treat coarctation and repair concomitant cardiac anomalies, like aortic root dilatation, cardiac valvular disease and so on. Among them, 30 patients underwent bilateral aortofemoral bypass and 20 patients underwent hybrid procedure to treat coarctation. We retrospectively analyzed the data of these patients and compared the early and late results.ResultsAll the baseline clinical characteristics were comparable between groups except that the mean age of bypass group was 39.5 ± 14.0 years which was older than hybrid group (27.9 ± 8.5 years, P = 0.002). Technical success was achieved in all patients, with no hospital death or other severe complications. Immediately after surgery, in bypass and hybrid group, the mean upper-limb systolic blood pressure decreased from 159.4 to 119.7 mmHg and 148.4 to 111.6 mmHg, the median peak systolic gradient decreased from 68.0 to 10 mmHg and 46.5 to 10 mmHg respectively (P = 0.09). And the mean upper-lower limbs gradient decreased from 21.7 to 5.9 mmHg and 21.0 to 2.7 mmHg respectively (P = 0.104). The mean follow-up time was 76.92 ± 18.7 in bypass group and 85.4 ± 20.6 months in hybrid group. There were 4 late deaths in bypass group (one died of gastrointestinal bleeding, one died of pulmonary embolism and the other two died of heart failure caused by mechanical prosthetic valve dysfunction). The follow-up peak systolic gradient and other blood pressure parameters showed stable and no differences between two groups.ConclusionsThe bilateral aortofemoral bypass surgery is a safe and effective method which can be used in one-staged surgical strategy to treat coarctation complicated by cardiac anomalies and can be an alternative to the hybrid method.

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A Constellation of Cardiac Anomalies: Beyond Shone's Complex.

Publications

A Constellation of Cardiac Anomalies: Beyond Shone's Complex.

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