Exercise testing oversights underlie missed and delayed diagnosis of catecholaminergic polymorphic ventricular tachycardia in young sudden cardiac arrest survivors

John R. Giudicessi, Michael John Ackerman

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by adrenergically induced ventricular tachycardia, syncope, and sudden cardiac arrest (SCA). In the absence of structural disease, exercise-provoked premature ventricular contractions in bigeminy or couplets and nonsustained ventricular tachycardia are highly predictive of CPVT. Objective: The purpose of this study was to determine the number of missed or delayed CPVT diagnoses attributable to exercise testing oversights in a cohort of young SCA survivors. Methods: A retrospective review of 101 young SCA survivors (younger than 35 years at the time of SCA) with otherwise structurally normal hearts was used to identify those with a missed or delayed CPVT diagnosis because of overlooked evidence or lack of an exercise stress test (EST) or catecholamine provocation test (CPT) post-SCA. Results: Of the 101 young SCA survivors, 41 (41%) had exertion/emotion-associated SCA (EEA-SCA). After primary post-SCA investigations, a probable root cause was established in 20 of 41 EEA-SCA survivors (49%; CPVT in 8) and in 30 of 60 non-EEA-SCA survivors (50%; CPVT in 2) (P = 1). Only 14 of 21 unexplained EEA-SCA survivors (67%) had an EST/CPT performed before their referral evaluation. Secondary review of these prior ESTs/CPTs provided evidence of CPVT in 3 of 14 (21%). Of the 7 remaining unexplained cases of EAA-SCA who had never undergone an EST/CPT, 2 (29%) underwent their first EST at our institution that led to CPVT diagnosis. Conclusion: Of the 15 SCA survivors diagnosed ultimately with CPVT, one-third had a delay in diagnosis because an EST was either never performed or performed but misinterpreted. EST/CPT must become the standard of care after SCA in the young, especially if the SCA occurred during either exertion or emotion.

Original languageEnglish (US)
Pages (from-to)1232-1239
Number of pages8
JournalHeart rhythm
Volume16
Issue number8
DOIs
StatePublished - Aug 1 2019

Fingerprint

Delayed Diagnosis
Sudden Cardiac Death
Exercise Test
Emotions
Catecholamines
Ventricular Tachycardia
Polymorphic catecholergic ventricular tachycardia
Ventricular Premature Complexes
Syncope
Standard of Care
Referral and Consultation

Keywords

  • CPVT
  • Idiopathic ventricular fibrillation
  • Long QT syndrome
  • SCA
  • SCD

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

@article{a8021952a3454be7a14eda5038d3503b,
title = "Exercise testing oversights underlie missed and delayed diagnosis of catecholaminergic polymorphic ventricular tachycardia in young sudden cardiac arrest survivors",
abstract = "Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by adrenergically induced ventricular tachycardia, syncope, and sudden cardiac arrest (SCA). In the absence of structural disease, exercise-provoked premature ventricular contractions in bigeminy or couplets and nonsustained ventricular tachycardia are highly predictive of CPVT. Objective: The purpose of this study was to determine the number of missed or delayed CPVT diagnoses attributable to exercise testing oversights in a cohort of young SCA survivors. Methods: A retrospective review of 101 young SCA survivors (younger than 35 years at the time of SCA) with otherwise structurally normal hearts was used to identify those with a missed or delayed CPVT diagnosis because of overlooked evidence or lack of an exercise stress test (EST) or catecholamine provocation test (CPT) post-SCA. Results: Of the 101 young SCA survivors, 41 (41{\%}) had exertion/emotion-associated SCA (EEA-SCA). After primary post-SCA investigations, a probable root cause was established in 20 of 41 EEA-SCA survivors (49{\%}; CPVT in 8) and in 30 of 60 non-EEA-SCA survivors (50{\%}; CPVT in 2) (P = 1). Only 14 of 21 unexplained EEA-SCA survivors (67{\%}) had an EST/CPT performed before their referral evaluation. Secondary review of these prior ESTs/CPTs provided evidence of CPVT in 3 of 14 (21{\%}). Of the 7 remaining unexplained cases of EAA-SCA who had never undergone an EST/CPT, 2 (29{\%}) underwent their first EST at our institution that led to CPVT diagnosis. Conclusion: Of the 15 SCA survivors diagnosed ultimately with CPVT, one-third had a delay in diagnosis because an EST was either never performed or performed but misinterpreted. EST/CPT must become the standard of care after SCA in the young, especially if the SCA occurred during either exertion or emotion.",
keywords = "CPVT, Idiopathic ventricular fibrillation, Long QT syndrome, SCA, SCD",
author = "Giudicessi, {John R.} and Ackerman, {Michael John}",
year = "2019",
month = "8",
day = "1",
doi = "10.1016/j.hrthm.2019.02.012",
language = "English (US)",
volume = "16",
pages = "1232--1239",
journal = "Heart Rhythm",
issn = "1547-5271",
publisher = "Elsevier",
number = "8",

}

TY - JOUR

T1 - Exercise testing oversights underlie missed and delayed diagnosis of catecholaminergic polymorphic ventricular tachycardia in young sudden cardiac arrest survivors

AU - Giudicessi, John R.

AU - Ackerman, Michael John

PY - 2019/8/1

Y1 - 2019/8/1

N2 - Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by adrenergically induced ventricular tachycardia, syncope, and sudden cardiac arrest (SCA). In the absence of structural disease, exercise-provoked premature ventricular contractions in bigeminy or couplets and nonsustained ventricular tachycardia are highly predictive of CPVT. Objective: The purpose of this study was to determine the number of missed or delayed CPVT diagnoses attributable to exercise testing oversights in a cohort of young SCA survivors. Methods: A retrospective review of 101 young SCA survivors (younger than 35 years at the time of SCA) with otherwise structurally normal hearts was used to identify those with a missed or delayed CPVT diagnosis because of overlooked evidence or lack of an exercise stress test (EST) or catecholamine provocation test (CPT) post-SCA. Results: Of the 101 young SCA survivors, 41 (41%) had exertion/emotion-associated SCA (EEA-SCA). After primary post-SCA investigations, a probable root cause was established in 20 of 41 EEA-SCA survivors (49%; CPVT in 8) and in 30 of 60 non-EEA-SCA survivors (50%; CPVT in 2) (P = 1). Only 14 of 21 unexplained EEA-SCA survivors (67%) had an EST/CPT performed before their referral evaluation. Secondary review of these prior ESTs/CPTs provided evidence of CPVT in 3 of 14 (21%). Of the 7 remaining unexplained cases of EAA-SCA who had never undergone an EST/CPT, 2 (29%) underwent their first EST at our institution that led to CPVT diagnosis. Conclusion: Of the 15 SCA survivors diagnosed ultimately with CPVT, one-third had a delay in diagnosis because an EST was either never performed or performed but misinterpreted. EST/CPT must become the standard of care after SCA in the young, especially if the SCA occurred during either exertion or emotion.

AB - Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by adrenergically induced ventricular tachycardia, syncope, and sudden cardiac arrest (SCA). In the absence of structural disease, exercise-provoked premature ventricular contractions in bigeminy or couplets and nonsustained ventricular tachycardia are highly predictive of CPVT. Objective: The purpose of this study was to determine the number of missed or delayed CPVT diagnoses attributable to exercise testing oversights in a cohort of young SCA survivors. Methods: A retrospective review of 101 young SCA survivors (younger than 35 years at the time of SCA) with otherwise structurally normal hearts was used to identify those with a missed or delayed CPVT diagnosis because of overlooked evidence or lack of an exercise stress test (EST) or catecholamine provocation test (CPT) post-SCA. Results: Of the 101 young SCA survivors, 41 (41%) had exertion/emotion-associated SCA (EEA-SCA). After primary post-SCA investigations, a probable root cause was established in 20 of 41 EEA-SCA survivors (49%; CPVT in 8) and in 30 of 60 non-EEA-SCA survivors (50%; CPVT in 2) (P = 1). Only 14 of 21 unexplained EEA-SCA survivors (67%) had an EST/CPT performed before their referral evaluation. Secondary review of these prior ESTs/CPTs provided evidence of CPVT in 3 of 14 (21%). Of the 7 remaining unexplained cases of EAA-SCA who had never undergone an EST/CPT, 2 (29%) underwent their first EST at our institution that led to CPVT diagnosis. Conclusion: Of the 15 SCA survivors diagnosed ultimately with CPVT, one-third had a delay in diagnosis because an EST was either never performed or performed but misinterpreted. EST/CPT must become the standard of care after SCA in the young, especially if the SCA occurred during either exertion or emotion.

KW - CPVT

KW - Idiopathic ventricular fibrillation

KW - Long QT syndrome

KW - SCA

KW - SCD

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