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Late outcomes in children with Shone’s complex: a single-centre, 20-year experience
- George T. Nicholson, Michael S. Kelleman, Caridad M. De la Uz, Ricardo H. Pignatelli, Nancy A. Ayres, Christopher J. Petit
- Journal: Cardiology in the Young / Volume 27 / Issue 4 / May 2017
- Published online by Cambridge University Press: 26 July 2016, pp. 697-705
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View abstractObjective
Shone’s syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone’s syndrome and the factors associated with left heart growth.
MethodsAll patients diagnosed with Shone’s syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed.
ResultsA total of 121 patients with Shone’s syndrome presented at a median age of 28 days (0–17.3 years) and were followed-up for 7.2 years (0.01–35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time.
ConclusionsShone’s syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.
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- By Rose Teteki Abbey, K. C. Abraham, David Tuesday Adamo, LeRoy H. Aden, Efrain Agosto, Victor Aguilan, Gillian T. W. Ahlgren, Charanjit Kaur AjitSingh, Dorothy B E A Akoto, Giuseppe Alberigo, Daniel E. Albrecht, Ruth Albrecht, Daniel O. Aleshire, Urs Altermatt, Anand Amaladass, Michael Amaladoss, James N. Amanze, Lesley G. Anderson, Thomas C. Anderson, Victor Anderson, Hope S. Antone, María Pilar Aquino, Paula Arai, Victorio Araya Guillén, S. Wesley Ariarajah, Ellen T. Armour, Brett Gregory Armstrong, Atsuhiro Asano, Naim Stifan Ateek, Mahmoud Ayoub, John Alembillah Azumah, Mercedes L. García Bachmann, Irena Backus, J. Wayne Baker, Mieke Bal, Lewis V. Baldwin, William Barbieri, António Barbosa da Silva, David Basinger, Bolaji Olukemi Bateye, Oswald Bayer, Daniel H. Bays, Rosalie Beck, Nancy Elizabeth Bedford, Guy-Thomas Bedouelle, Chorbishop Seely Beggiani, Wolfgang Behringer, Christopher M. Bellitto, Byard Bennett, Harold V. Bennett, Teresa Berger, Miguel A. Bernad, Henley Bernard, Alan E. Bernstein, Jon L. Berquist, Johannes Beutler, Ana María Bidegain, Matthew P. Binkewicz, Jennifer Bird, Joseph Blenkinsopp, Dmytro Bondarenko, Paulo Bonfatti, Riet en Pim Bons-Storm, Jessica A. Boon, Marcus J. Borg, Mark Bosco, Peter C. Bouteneff, François Bovon, William D. Bowman, Paul S. Boyer, David Brakke, Richard E. Brantley, Marcus Braybrooke, Ian Breward, Ênio José da Costa Brito, Jewel Spears Brooker, Johannes Brosseder, Nicholas Canfield Read Brown, Robert F. Brown, Pamela K. Brubaker, Walter Brueggemann, Bishop Colin O. Buchanan, Stanley M. Burgess, Amy Nelson Burnett, J. Patout Burns, David B. Burrell, David Buttrick, James P. Byrd, Lavinia Byrne, Gerado Caetano, Marcos Caldas, Alkiviadis Calivas, William J. Callahan, Salvatore Calomino, Euan K. Cameron, William S. Campbell, Marcelo Ayres Camurça, Daniel F. Caner, Paul E. Capetz, Carlos F. Cardoza-Orlandi, Patrick W. Carey, Barbara Carvill, Hal Cauthron, Subhadra Mitra Channa, Mark D. 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Yee, Viktor Yelensky, Yeo Khiok-Khng, Gustav K. K. Yeung, Angela Yiu, Amos Yong, Yong Ting Jin, You Bin, Youhanna Nessim Youssef, Eliana Yunes, Robert Michael Zaller, Valarie H. Ziegler, Barbara Brown Zikmund, Joyce Ann Zimmerman, Aurora Zlotnik, Zhuo Xinping
- Edited by Daniel Patte, Vanderbilt University, Tennessee
- Book: The Cambridge Dictionary of Christianity
- Published online: 05 August 2012
- Print publication: 20 September 2010, pp xi-xliv
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Cardiac rhabdomyoma: a report of alternative strategies to surgical resection
- Colin J. McMahon, Nancy A. Ayres, Mark B. Lewin
- Journal: Cardiology in the Young / Volume 11 / Issue 6 / November 2001
- Published online by Cambridge University Press: 15 August 2006, pp. 670-672
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Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.
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Echocardiographic presentations of endocarditis, and risk factors for rupture of a sinus of Valsalva in childhood
- Colin J. McMahon, Nancy Ayres, Ricardo H. Pignatelli, Wayne Franklin, Thomas A. Vargo, J. Timothy Bricker, Howaida G. El-Said
- Journal: Cardiology in the Young / Volume 13 / Issue 2 / April 2003
- Published online by Cambridge University Press: 18 April 2005, pp. 168-172
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Background: In recent years, the diagnosis of infective endocarditis has been enhanced by the use of echocardiography. We sought, therefore, to review its effect on the management of endocarditis in children. Methods: We reviewed all the patients presenting to our institution for evaluation for infective endocarditis from May 1994 to January 2002. The patients were stratified according to whether or not they had congenitally malformed hearts. Results: Of the 90 referred patients identified, 46 (51%) had positive ultrasonic findings. Of these, we excluded 26 patients because of the presence of indwelling lines. The remaining 20 patients with features of endocarditis had a median age of 6.5 years, and a range from 0.14 to 8.5 years. There were 4 patients with normal hearts, and 16 with congenital cardiac malformations. We identified rupture of a sinus of Valsalva in four patients, with rupture into the left ventricle in two, and into the right ventricle and right atrium in one each. The mitral valve was involved in six patients, the aortic valve in another six, including all four with rupture of the sinus of Valsalva, both mitral and aortic valves in three, the pulmonary trunk in three patients, and the tricuspid valve and a Blalock-Taussig shunt in one patient each. Organisms isolated included Streptococcus mitis in 4 patients, Streptococcus pneumoniae in 2 patients, Streptococcus sanguis in 1, Staphylococcus aureus in 3, Staphylococcus epidermidis in 1, and Enteroccocus in 2. Cultures proved negative in 7 patients. Surgical intervention was needed in 12 patients, and one died (5%). Only the left-sided chambers were involved in those with normal hearts. Both patients infected with Streptococcus pneumoniae had rupture of a sinus of Valsalva. Conclusion: Involvement of the left-sided chambers is more likely in structurally normal hearts, and in cases with rupture of a sinus of Valsalva, in which case infection with Streptococcus pneumonia should be suspected.
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