Holter monitoring in the evaluation of congenital long QT syndrome

Daniel A. Mauriello, Jonathan N. Johnson, Michael John Ackerman

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Background: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy that affects one in 2,000 persons; causes syncope, seizures, and sudden death; and is both under- and overdiagnosed. LQTS diagnostic miscues have stemmed from assessment of ambulatory electrocardiographic monitoring (Holter) results. Objective: We sought to determine the prevalence of positive Holter monitor tests and its diagnostic significance in evaluating LQTS. Methods: We performed an institutional review board-approved review of patients evaluated in our LQTS clinic from 2000 to 2009 who had Holter testing during their evaluation. Included patients (N = 473) were diagnosed with LQTS or dismissed as otherwise normal. Holters classified as positive had an episode of nonsustained ventricular tachycardia, supraventricular tachycardia, ≥4 couplets/day, ≥10 premature ventricular contractions/hour, or >5-second sinus pause. Results: Among 209 patients dismissed as normal (128 females, average age 21 ± 15 years, average QTc 424 ± 39 ms), 27 (12.9%) had a positive Holter, while among 264 patients with LQTS (149 females, average age 22 ± 16 years, average QTc 472 ± 41 ms), 30 (11.3%) had a positive Holter (P = NS). Patients with LQT3 (5/23, 21%) and genotype-negative LQTS (5/19, 26%) had a higher rate of positive Holter testing compared to LQT1 patients (7/124, 6%, P < 0.03). Among the 473 Holters, only one (0.2%) impacted clinical decision making. Conclusion: Routine Holter monitoring appears to be of minimal clinical utility from a diagnostic and prognostic perspective in evaluating LQTS, and may not be cost effective. Whether Holter monitoring aids in therapeutic decisions such as dosing or whether ambulatory QTc measurements, provided by some newer devices, might help in the diagnostic evaluation warrants further scrutiny.

Original languageEnglish (US)
Pages (from-to)1100-1104
Number of pages5
JournalPACE - Pacing and Clinical Electrophysiology
Volume34
Issue number9
DOIs
StatePublished - Sep 2011

Fingerprint

Long QT Syndrome
Ambulatory Electrocardiography
Channelopathies
Supraventricular Tachycardia
Ventricular Premature Complexes
Research Ethics Committees
Syncope
Ventricular Tachycardia
Sudden Death
Routine Diagnostic Tests
Seizures
Genotype
Costs and Cost Analysis
Equipment and Supplies

Keywords

  • Ambulatory ECG monitoring
  • Holter
  • Long QT Syndrome

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Holter monitoring in the evaluation of congenital long QT syndrome. / Mauriello, Daniel A.; Johnson, Jonathan N.; Ackerman, Michael John.

In: PACE - Pacing and Clinical Electrophysiology, Vol. 34, No. 9, 09.2011, p. 1100-1104.

Research output: Contribution to journalArticle

Mauriello, Daniel A. ; Johnson, Jonathan N. ; Ackerman, Michael John. / Holter monitoring in the evaluation of congenital long QT syndrome. In: PACE - Pacing and Clinical Electrophysiology. 2011 ; Vol. 34, No. 9. pp. 1100-1104.
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