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 language | English (US) |
|---|---|
| Pages (from-to) | 1232-1239 |
| Number of pages | 8 |
| Journal | Heart rhythm |
| Volume | 16 |
| Issue number | 8 |
| DOIs | |
| State | Published - Aug 1 2019 |
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Keywords
- CPVT
- Idiopathic ventricular fibrillation
- Long QT syndrome
- SCA
- SCD
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Physiology (medical)
Cite this
Exercise testing oversights underlie missed and delayed diagnosis of catecholaminergic polymorphic ventricular tachycardia in young sudden cardiac arrest survivors. / Giudicessi, John R.; Ackerman, Michael John.
In: Heart rhythm, Vol. 16, No. 8, 01.08.2019, p. 1232-1239.Research output: Contribution to journal › Article
}
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
UR - http://www.scopus.com/inward/record.url?scp=85068834788&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85068834788&partnerID=8YFLogxK
U2 - 10.1016/j.hrthm.2019.02.012
DO - 10.1016/j.hrthm.2019.02.012
M3 - Article
C2 - 30763784
AN - SCOPUS:85068834788
VL - 16
SP - 1232
EP - 1239
JO - Heart Rhythm
JF - Heart Rhythm
SN - 1547-5271
IS - 8
ER -