Electroanatomic mapping and late gadolinium enhancement MRI in a genetic model of arrhythmogenic atrial cardiomyopathy

Marcello Disertori, Michela Masè, Massimiliano Marini, Silvia Mazzola, Alessandro Cristoforetti, Maurizio Del Greco, Hans Kottkamp, Eloisa Arbustini, Flavia Ravelli

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Evaluation of the Substrate in Genetic Atrial Arrhythmias. Introduction: Although atrial arrhythmiasmay have genetic causes, very few data are available on evaluation of the arrhythmic substrate in genetic atrial diseases in humans. In this study, we evaluate the nature and evolution of the atrial arrhythmic substrate in a genetic atrial cardiomyopathy.

Methods and Results: Repeated electroanatomic mapping and tomographic evaluations were used to investigate the evolving arrhythmic substrate in 5 patients with isolated arrhythmogenic atrial cardiomyopathy, caused by Natriuretic Peptide Precursor A (NPPA) gene mutation. Atrial fibrosis was assessed using late gadolinium enhancement magnetic resonance imaging (LGE-MRI). The substrate of atrial tachycardia (AT) and atrial fibrillation (AF) was biatrial dilatation with patchy areas of low voltage and atrial wall scarring (in the right atrium: 68.5%±6.0% and 22.2%±10.2%, respectively). The evolution of the arrhythmic patterns to sinus node disease with atrial standstill (AS) was associated with giant atria with extensive low voltage and atrial scarring areas (in the right atrium: 99.5% ± 0.7% and 57.5% ± 33.2%, respectively). LGE-MRI-proven biatrial fibrosis (Utah stage IV) was associated with AS. Atrial conduction was slow and heterogeneous, with lines of conduction blocks. The progressive extension and spatial distribution of the scarring/fibrosis were strictly associated with the different types of arrhythmias.

Conclusion: The evolution of the amount and distribution of atrial scarring/fibrosis constitutes the structural substrate for the different types of atrial arrhythmias in a pure genetic model of arrhythmogenic atrial cardiomyopathy.

Original languageEnglish
Pages (from-to)964-970
Number of pages7
JournalJournal of Cardiovascular Electrophysiology
Volume25
Issue number9
DOIs
Publication statusPublished - 2014

Fingerprint

Genetic Models
Gadolinium
Cardiomyopathies
Cicatrix
Fibrosis
Cardiac Arrhythmias
Heart Atria
Magnetic Resonance Imaging
Sick Sinus Syndrome
Natriuretic Peptides
Inborn Genetic Diseases
Tachycardia
Atrial Fibrillation
Dilatation
Mutation
Genes
Atrial Standstill

Keywords

  • Atrial arrhythmias
  • Atrial cardiomyopathy
  • Atrial fibrosis
  • Electroanatomic mapping
  • Late gadolinium enhancement magnetic resonance imaging
  • NPPA gene

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)
  • Medicine(all)

Cite this

Electroanatomic mapping and late gadolinium enhancement MRI in a genetic model of arrhythmogenic atrial cardiomyopathy. / Disertori, Marcello; Masè, Michela; Marini, Massimiliano; Mazzola, Silvia; Cristoforetti, Alessandro; Del Greco, Maurizio; Kottkamp, Hans; Arbustini, Eloisa; Ravelli, Flavia.

In: Journal of Cardiovascular Electrophysiology, Vol. 25, No. 9, 2014, p. 964-970.

Research output: Contribution to journalArticle

Disertori, M, Masè, M, Marini, M, Mazzola, S, Cristoforetti, A, Del Greco, M, Kottkamp, H, Arbustini, E & Ravelli, F 2014, 'Electroanatomic mapping and late gadolinium enhancement MRI in a genetic model of arrhythmogenic atrial cardiomyopathy', Journal of Cardiovascular Electrophysiology, vol. 25, no. 9, pp. 964-970. https://doi.org/10.1111/jce.12440
Disertori, Marcello ; Masè, Michela ; Marini, Massimiliano ; Mazzola, Silvia ; Cristoforetti, Alessandro ; Del Greco, Maurizio ; Kottkamp, Hans ; Arbustini, Eloisa ; Ravelli, Flavia. / Electroanatomic mapping and late gadolinium enhancement MRI in a genetic model of arrhythmogenic atrial cardiomyopathy. In: Journal of Cardiovascular Electrophysiology. 2014 ; Vol. 25, No. 9. pp. 964-970.
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abstract = "Evaluation of the Substrate in Genetic Atrial Arrhythmias. Introduction: Although atrial arrhythmiasmay have genetic causes, very few data are available on evaluation of the arrhythmic substrate in genetic atrial diseases in humans. In this study, we evaluate the nature and evolution of the atrial arrhythmic substrate in a genetic atrial cardiomyopathy.Methods and Results: Repeated electroanatomic mapping and tomographic evaluations were used to investigate the evolving arrhythmic substrate in 5 patients with isolated arrhythmogenic atrial cardiomyopathy, caused by Natriuretic Peptide Precursor A (NPPA) gene mutation. Atrial fibrosis was assessed using late gadolinium enhancement magnetic resonance imaging (LGE-MRI). The substrate of atrial tachycardia (AT) and atrial fibrillation (AF) was biatrial dilatation with patchy areas of low voltage and atrial wall scarring (in the right atrium: 68.5{\%}±6.0{\%} and 22.2{\%}±10.2{\%}, respectively). The evolution of the arrhythmic patterns to sinus node disease with atrial standstill (AS) was associated with giant atria with extensive low voltage and atrial scarring areas (in the right atrium: 99.5{\%} ± 0.7{\%} and 57.5{\%} ± 33.2{\%}, respectively). LGE-MRI-proven biatrial fibrosis (Utah stage IV) was associated with AS. Atrial conduction was slow and heterogeneous, with lines of conduction blocks. The progressive extension and spatial distribution of the scarring/fibrosis were strictly associated with the different types of arrhythmias.Conclusion: The evolution of the amount and distribution of atrial scarring/fibrosis constitutes the structural substrate for the different types of atrial arrhythmias in a pure genetic model of arrhythmogenic atrial cardiomyopathy.",
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AU - Mazzola, Silvia

AU - Cristoforetti, Alessandro

AU - Del Greco, Maurizio

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N2 - Evaluation of the Substrate in Genetic Atrial Arrhythmias. Introduction: Although atrial arrhythmiasmay have genetic causes, very few data are available on evaluation of the arrhythmic substrate in genetic atrial diseases in humans. In this study, we evaluate the nature and evolution of the atrial arrhythmic substrate in a genetic atrial cardiomyopathy.Methods and Results: Repeated electroanatomic mapping and tomographic evaluations were used to investigate the evolving arrhythmic substrate in 5 patients with isolated arrhythmogenic atrial cardiomyopathy, caused by Natriuretic Peptide Precursor A (NPPA) gene mutation. Atrial fibrosis was assessed using late gadolinium enhancement magnetic resonance imaging (LGE-MRI). The substrate of atrial tachycardia (AT) and atrial fibrillation (AF) was biatrial dilatation with patchy areas of low voltage and atrial wall scarring (in the right atrium: 68.5%±6.0% and 22.2%±10.2%, respectively). The evolution of the arrhythmic patterns to sinus node disease with atrial standstill (AS) was associated with giant atria with extensive low voltage and atrial scarring areas (in the right atrium: 99.5% ± 0.7% and 57.5% ± 33.2%, respectively). LGE-MRI-proven biatrial fibrosis (Utah stage IV) was associated with AS. Atrial conduction was slow and heterogeneous, with lines of conduction blocks. The progressive extension and spatial distribution of the scarring/fibrosis were strictly associated with the different types of arrhythmias.Conclusion: The evolution of the amount and distribution of atrial scarring/fibrosis constitutes the structural substrate for the different types of atrial arrhythmias in a pure genetic model of arrhythmogenic atrial cardiomyopathy.

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