Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease

Hunter C. Wilson, Robert J. Hopkin, Peace C. Madueme, Richard J. Czosek, Laurie A. Bailey, Michael D. Taylor, John Jefferies

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9.7 ± 3.8 years, n = 12 males). Sinus bradycardia was the most frequent rhythm abnormality (23%), followed by ectopic atrial rhythm (12%) and premature atrial contractions (8%). No PR, QRS, or QTc intervals were prolonged. First-degree atrioventricular block developed in 1 female during follow-up. Chest pain (35%) and palpitations (23%) were highly prevalent complaints in clinical follow-up and did not differ significantly between genders. Structural findings included aortic root dilation in 3 patients and concurrent aortic insufficiency in 1. Among 593 patients aged < 18 years with electrocardiographic data identified in the Fabry Registry, sinus bradycardia, defined as heart rate <60 beats per minute per registry guidelines, was the most common arrhythmia (12.3%). In conclusion, clinical findings and subtle abnormalities of conduction, rhythm, and structure point toward a heterogeneous inception of Fabry cardiomyopathy. Bradycardia, common in adults, is frequent even among children with AFD. Given the potential for early initiation of enzyme replacement therapy to reduce cardiovascular morbidity, continued work to develop paradigms of therapy and longitudinal cardiovascular surveillance is warranted.

Original languageEnglish (US)
Pages (from-to)251-255
Number of pages5
JournalAmerican Journal of Cardiology
Volume120
Issue number2
DOIs
StatePublished - Jul 15 2017
Externally publishedYes

Fingerprint

Fabry Disease
Cardiac Arrhythmias
Bradycardia
Registries
Atrial Premature Complexes
Enzyme Replacement Therapy
Atrioventricular Block
Chest Pain
Cardiomyopathies
Hypertrophy
Cicatrix
Dilatation
Heart Rate
Guidelines
Pediatrics
Morbidity
Phenotype

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Wilson, H. C., Hopkin, R. J., Madueme, P. C., Czosek, R. J., Bailey, L. A., Taylor, M. D., & Jefferies, J. (2017). Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease. American Journal of Cardiology, 120(2), 251-255. https://doi.org/10.1016/j.amjcard.2017.04.016

Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease. / Wilson, Hunter C.; Hopkin, Robert J.; Madueme, Peace C.; Czosek, Richard J.; Bailey, Laurie A.; Taylor, Michael D.; Jefferies, John.

In: American Journal of Cardiology, Vol. 120, No. 2, 15.07.2017, p. 251-255.

Research output: Contribution to journalArticle

Wilson, HC, Hopkin, RJ, Madueme, PC, Czosek, RJ, Bailey, LA, Taylor, MD & Jefferies, J 2017, 'Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease', American Journal of Cardiology, vol. 120, no. 2, pp. 251-255. https://doi.org/10.1016/j.amjcard.2017.04.016
Wilson HC, Hopkin RJ, Madueme PC, Czosek RJ, Bailey LA, Taylor MD et al. Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease. American Journal of Cardiology. 2017 Jul 15;120(2):251-255. https://doi.org/10.1016/j.amjcard.2017.04.016
Wilson, Hunter C. ; Hopkin, Robert J. ; Madueme, Peace C. ; Czosek, Richard J. ; Bailey, Laurie A. ; Taylor, Michael D. ; Jefferies, John. / Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease. In: American Journal of Cardiology. 2017 ; Vol. 120, No. 2. pp. 251-255.
@article{b67fcac8407546d48aecae0b7ef15d44,
title = "Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease",
abstract = "Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9.7 ± 3.8 years, n = 12 males). Sinus bradycardia was the most frequent rhythm abnormality (23{\%}), followed by ectopic atrial rhythm (12{\%}) and premature atrial contractions (8{\%}). No PR, QRS, or QTc intervals were prolonged. First-degree atrioventricular block developed in 1 female during follow-up. Chest pain (35{\%}) and palpitations (23{\%}) were highly prevalent complaints in clinical follow-up and did not differ significantly between genders. Structural findings included aortic root dilation in 3 patients and concurrent aortic insufficiency in 1. Among 593 patients aged < 18 years with electrocardiographic data identified in the Fabry Registry, sinus bradycardia, defined as heart rate <60 beats per minute per registry guidelines, was the most common arrhythmia (12.3{\%}). In conclusion, clinical findings and subtle abnormalities of conduction, rhythm, and structure point toward a heterogeneous inception of Fabry cardiomyopathy. Bradycardia, common in adults, is frequent even among children with AFD. Given the potential for early initiation of enzyme replacement therapy to reduce cardiovascular morbidity, continued work to develop paradigms of therapy and longitudinal cardiovascular surveillance is warranted.",
author = "Wilson, {Hunter C.} and Hopkin, {Robert J.} and Madueme, {Peace C.} and Czosek, {Richard J.} and Bailey, {Laurie A.} and Taylor, {Michael D.} and John Jefferies",
year = "2017",
month = "7",
day = "15",
doi = "10.1016/j.amjcard.2017.04.016",
language = "English (US)",
volume = "120",
pages = "251--255",
journal = "American Journal of Cardiology",
issn = "0002-9149",
publisher = "Elsevier Inc.",
number = "2",

}

TY - JOUR

T1 - Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease

AU - Wilson, Hunter C.

AU - Hopkin, Robert J.

AU - Madueme, Peace C.

AU - Czosek, Richard J.

AU - Bailey, Laurie A.

AU - Taylor, Michael D.

AU - Jefferies, John

PY - 2017/7/15

Y1 - 2017/7/15

N2 - Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9.7 ± 3.8 years, n = 12 males). Sinus bradycardia was the most frequent rhythm abnormality (23%), followed by ectopic atrial rhythm (12%) and premature atrial contractions (8%). No PR, QRS, or QTc intervals were prolonged. First-degree atrioventricular block developed in 1 female during follow-up. Chest pain (35%) and palpitations (23%) were highly prevalent complaints in clinical follow-up and did not differ significantly between genders. Structural findings included aortic root dilation in 3 patients and concurrent aortic insufficiency in 1. Among 593 patients aged < 18 years with electrocardiographic data identified in the Fabry Registry, sinus bradycardia, defined as heart rate <60 beats per minute per registry guidelines, was the most common arrhythmia (12.3%). In conclusion, clinical findings and subtle abnormalities of conduction, rhythm, and structure point toward a heterogeneous inception of Fabry cardiomyopathy. Bradycardia, common in adults, is frequent even among children with AFD. Given the potential for early initiation of enzyme replacement therapy to reduce cardiovascular morbidity, continued work to develop paradigms of therapy and longitudinal cardiovascular surveillance is warranted.

AB - Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9.7 ± 3.8 years, n = 12 males). Sinus bradycardia was the most frequent rhythm abnormality (23%), followed by ectopic atrial rhythm (12%) and premature atrial contractions (8%). No PR, QRS, or QTc intervals were prolonged. First-degree atrioventricular block developed in 1 female during follow-up. Chest pain (35%) and palpitations (23%) were highly prevalent complaints in clinical follow-up and did not differ significantly between genders. Structural findings included aortic root dilation in 3 patients and concurrent aortic insufficiency in 1. Among 593 patients aged < 18 years with electrocardiographic data identified in the Fabry Registry, sinus bradycardia, defined as heart rate <60 beats per minute per registry guidelines, was the most common arrhythmia (12.3%). In conclusion, clinical findings and subtle abnormalities of conduction, rhythm, and structure point toward a heterogeneous inception of Fabry cardiomyopathy. Bradycardia, common in adults, is frequent even among children with AFD. Given the potential for early initiation of enzyme replacement therapy to reduce cardiovascular morbidity, continued work to develop paradigms of therapy and longitudinal cardiovascular surveillance is warranted.

UR - http://www.scopus.com/inward/record.url?scp=85019620097&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85019620097&partnerID=8YFLogxK

U2 - 10.1016/j.amjcard.2017.04.016

DO - 10.1016/j.amjcard.2017.04.016

M3 - Article

VL - 120

SP - 251

EP - 255

JO - American Journal of Cardiology

JF - American Journal of Cardiology

SN - 0002-9149

IS - 2

ER -