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Sponsored by: |
Children's Healthcare of Atlanta |
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Information provided by: | Children's Healthcare of Atlanta |
ClinicalTrials.gov Identifier: | NCT00268060 |
Infants with congenital long QT syndrome (LQTS) and 2:1 atrioventricular block (AVB) have been recognized as a clinical subset of children with LQTS.
The purpose of this study is to describe the clinical course and outcome of a patient with congenital long QT syndrome (LQTS).
Condition |
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Congenital Disorders |
Study Type: | Observational |
Study Design: | Natural History, Longitudinal, Defined Population, Retrospective Study |
Official Title: | Baby R. Case Report Proposal |
Estimated Enrollment: | 1 |
Study Start Date: | September 2004 |
It has been previously reported that 2:1 AVB in neonatal LQTS seems preferentially associated with HERG mutations . It has also been reported in association with the SCN5A gene. We report on a neonate with congenital LQTS and congenital complete heart block who experienced frequent episodes of torades des pointe and ventricular tachycardia, which, over the first 2 days of life, was recalcitrant to traditional therapy of beta-blockers and other antiarrhythmics. Eventually hypotension necessitated the use of an epinephrine infusion, which halted the ventricular tachyarrhythmias. A pacemaker was implanted once the infant was stable and, currently, he is thriving. Genetic analysis revealed a HERG mutation.
For this study, we will need access to the infant’s inpatient and outpatient medical records at Children's Healthcare of Atlanta at Egleston Children’s Hospital and Sibley Heart Center Cardiology to include his age, date of birth, medical record number, inpatient labs, inpatient echo reports, outpatient echo reports, inpatient and outpatient EKG’s, inpatient pharmacy records, the results of his genetic analysis, his history and physical, transfer notes, daily progress notes, consultation reports and operative reports from his admission beginning 9/30/04.
Ages Eligible for Study: | up to 21 Years |
Genders Eligible for Study: | Male |
Accepts Healthy Volunteers: | No |
Inclusion Criteria:
Exclusion Criteria:
United States, Georgia | |
Chldren's Healthcare of Atlanta | |
Atlanta, Georgia, United States, 30322 |
Principal Investigator: | Tracy Froehlich, MD | Children's Healthcare of Atlanta |
Study ID Numbers: | 05-050 |
Study First Received: | December 20, 2005 |
Last Updated: | May 3, 2007 |
ClinicalTrials.gov Identifier: | NCT00268060 |
Health Authority: | United States: Food and Drug Administration |
pediatric cardiac Long QT Syndrome Pacemaker Herg mutation |
Long QT Syndrome |