Identifying and Reducing Risk in Familial Long QT Syndrome

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dc.contributor.advisor Skinner, J en
dc.contributor.advisor Love, D en
dc.contributor.advisor Mitchell, E en
dc.contributor.author Waddell-Smith, Kathryn en
dc.date.accessioned 2018-04-04T02:15:59Z en
dc.date.issued 2017 en
dc.identifier.uri http://hdl.handle.net/2292/37039 en
dc.description.abstract Although knowledge of molecular genetics in cardiac inherited diseases (CID) has changed dramatically in recent years, the detection and clinical management of these conditions have changed little. Aims To focus on four key clinical areas relating primarily to familial long QT syndrome; (1) the family history in detection, (2) Holter recordings in detection and risk stratification, (3) adherence to betablocker therapy and (4) the side effects of left cardiac sympathetic denervation. Methods Patients enrolled with the New Zealand Cardiac Inherited Disease Registry were eligible for inclusion. Case notes were reviewed, electronic records audited and Holter monitors, ECGs and patient surveys conducted. Findings Taking a multigenerational family history increases detection of cardiac inherited diseases but is done poorly amongst general cardiology teams. Measurements of repolarisation on Holter monitors are highly repeatable, reflect disease status and clinical risk better than the initial ECG, and indicate those at low risk of symptoms and cardiac arrest. Beta-blocker adherence is suboptimal in half the Auckland LQTS population. Morbidity and patient satisfaction are both very high following left cardiac sympathetic denervation for LQTS and catecholaminergic polymorphic ventricular tachycardia. Conclusions The detection of CIDs and risk stratification and treatment of LQTS can be improved through refinements of clinically available tools. The cardiac sympathectomy is well tolerated despite side effects. Further research is needed to improve adherence to beta-blocker therapy. en
dc.publisher ResearchSpace@Auckland en
dc.relation.ispartof PhD Thesis - University of Auckland en
dc.relation.isreferencedby UoA99265061611302091 en
dc.rights Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. en
dc.rights.uri https://researchspace.auckland.ac.nz/docs/uoa-docs/rights.htm en
dc.title Identifying and Reducing Risk in Familial Long QT Syndrome en
dc.type Thesis en
thesis.degree.discipline Paediatric Medicine en
thesis.degree.grantor The University of Auckland en
thesis.degree.level Doctoral en
thesis.degree.name PhD en
dc.rights.holder Copyright: The author en
dc.rights.accessrights http://purl.org/eprint/accessRights/OpenAccess en
pubs.elements-id 735123 en
pubs.record-created-at-source-date 2018-04-04 en


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