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Mechanical and Electrophysiological Substrate for Recurrent Atrial Flutter Detected by Right Atrial Speckle Tracking Echocardiography and Electroanatomic Mapping in Myotonic Dystrophy Type 1

2013; Lippincott Williams & Wilkins; Volume: 127; Issue: 13 Linguagem: Inglês

10.1161/circulationaha.112.116624

1524-4539

Piercarlo Ballo, Marzia Giaccardi, Andrea Colella, F Cellerini, Fabrizio Bandini, Leandro Chiodi, Alfredo Zuppiroli,

Cardiomyopathy and Myosin Studies

HomeCirculationVol. 127, No. 13Mechanical and Electrophysiological Substrate for Recurrent Atrial Flutter Detected by Right Atrial Speckle Tracking Echocardiography and Electroanatomic Mapping in Myotonic Dystrophy Type 1 Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessResearch ArticlePDF/EPUBMechanical and Electrophysiological Substrate for Recurrent Atrial Flutter Detected by Right Atrial Speckle Tracking Echocardiography and Electroanatomic Mapping in Myotonic Dystrophy Type 1 Piercarlo Ballo, MD, Marzia Giaccardi, MD, Andrea Colella, MD, Fabrizio Cellerini, MD, Fabrizio Bandini, MD, Leandro Chiodi, MD and Alfredo Zuppiroli, MD Piercarlo BalloPiercarlo Ballo From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. , Marzia GiaccardiMarzia Giaccardi From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. , Andrea ColellaAndrea Colella From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. , Fabrizio CelleriniFabrizio Cellerini From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. , Fabrizio BandiniFabrizio Bandini From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. , Leandro ChiodiLeandro Chiodi From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. and Alfredo ZuppiroliAlfredo Zuppiroli From the Cardiology Unit, S. Maria Annunziata Hospital (P.B., L.C.); Cardiology Unit, Nuovo S. Giovanni di Dio Hospital (M.G.); Department of Heart and Vessels, Careggi University Hospital (A.C.); Cardiology Service, Mugello Hospital, Borgo S. Lorenzo (F.C., F.B.); and Department of Cardiology, Local Health Unit (A.Z.), Florence, Italy. Originally published2 Apr 2013https://doi.org/10.1161/CIRCULATIONAHA.112.116624Circulation. 2013;127:1422–1424A 48-year-old patient with myotonic dystrophy (MD) type 1, recurrent typical atrial flutter (AF), and otherwise unremarkable history was hospitalized for an electrophysiological study. The diagnosis of MD type 1 had been made 25 years earlier and was based on typical clinical features and confirmation by genetic analysis. The ECG pattern of AF was characterized by negative waves in the inferior leads and positive waves in V1, a cycle length of 280 milliseconds, and 2:1 atrioventricular conduction. At the time of the study, the patient was asymptomatic and showed normal findings at the cardiac physical examination, ECG, and standard echocardiography. Preablation ECG showed sinus rhythm at 60 bpm with a normal PR interval (180 milliseconds), regular QRS duration and morphology, and normal ventricular repolarization. Average septal-lateral mitral annulus velocities (s′, 9.5 cm/s; e′, 10.3 cm/s; a′, 7.4 cm/s), the E/e′ ratio (7.3), and left ventricular global longitudinal and circumferential strain (−21.5% and −23.2%, respectively) were all normal, as well as right ventricular systolic function (tricuspid annulus systolic excursion and peak systolic velocity, 25 mm and 18.5 cm/s, respectively). Analysis of longitudinal right atrial deformation by speckle tracking showed impaired strain mechanics in the inferior segment of the atrial septum (Figure 1, top, and Movie I in the online-only Data Supplement). Segmental atrial strain curves confirmed an abnormal deformation pattern at this level (Figure 1, bottom), resulting in reduced global peak atrial longitudinal strain (average, 21.2%; normal value, >29.0%). Electroanatomic mapping of the right atrium in sinus rhythm (EnSite NavX System, Endocardial Solutions, St. Jude Medical Inc, St. Paul, MN) consistently showed a low-voltage area in the inferior portion of the atrial septum, suggesting regional scarring (Figure 2). Coronary sinus stimulation induced counterclockwise typical AF with the same ECG pattern of the clinical arrhythmia. Radiofrequency ablation of the cavotricuspid isthmus was performed, resulting in sinus rhythm restoration. Postprocedural activation mapping confirmed isthmus bidirectional block and success of the procedure (Figure 3). Postablation ECG was unchanged.Download figureDownload PowerPointFigure 1. Speckle tracking. Top, Color-coded visualization of right atrial (RA) 2-dimensional strain at ventricular end systole showing abnormal deformation in the inferior portion of the atrial septum (white arrow). Blue and red denote positive and negative deformation, respectively. Bottom, Strain curves confirming abnormal deformation pattern (white arrow) in the inferior segment of the atrial septum (red curve), with normal curves in the remaining segments (blue, superior atrial septum; pink, medial roof; green, lateral roof; turquoise, superior lateral wall; yellow, inferior lateral wall). AVC indicates aortic valve closure; LA, left atrium; LV, left ventricle; and RV, right ventricle.Download figureDownload PowerPointFigure 2. Electroanatomic mapping. Right ventricular voltage map obtained from the right posterior oblique (A) and left posterior oblique (B) views showing a low-voltage area (red-yellow-green-blue, <1 mV) in the inferior segment of atrial septum (yellow arrow). Normal myocardium is shown in violet. CS indicates coronary sinus; IVC, inferior vena cava; STL, septal tricuspid leaflet; and SVC, superior vena cava.Download figureDownload PowerPointFigure 3. Electroanatomic mapping after radiofrequency ablation. Right atrial activation map obtained by coronary sinus stimulation from the right lateral (A) and right anterior oblique (B) views showing conduction block after successful cavotricuspid isthmus ablation. Normal activation is shown in red-yellow-green-blue; violet indicates late activation secondary to blocked conduction. IVC indicates inferior vena cava; STL, septal tricuspid leaflet; and SVC, superior vena cava.MD is an inherited multisystemic disease representing the most common autosomal-dominant muscular dystrophy worldwide.1 In its most severe form, called MD type 1 or Steinert disease, an abnormal expansion of a CTG-trinucleotide repeat sequence exists in the gene encoding for myotonic dystrophy protein kinase, a protein that plays a key role in intracellular signal modulation within smooth, cardiac, and skeletal myocytes. This mutation leads to progressive involvement of musculoskeletal apparatus, heart, brain, eye, endocrine, respiratory, and gastroenteric systems. The typical neuromuscular pattern is characterized by progressive distal muscular atrophy and weakness, grip and percussion myotonia, ptosis, hatchet face, slurred speech, and rhinolalia, and it is often associated with multisystem manifestations such as cataracts, insulin resistance, dysphagia, oligospermia, and neurobehavioral disorders. Cardiac abnormalities are also commonly found in patients with MD type 1, including atrioventricular or intraventricular conduction defects, arrhythmias, left ventricular hypertrophy and dysfunction, heart failure, and sudden death. Magnetic resonance imaging studies and endomyocardial biopsies suggested that focal areas of myocardial fibrosis and fatty infiltration may be responsible for these disorders.2 Analysis of 2-dimensional strain by speckle tracking was recently proposed as a technique to discriminate between normal myocardium and scar tissue in other populations. However, its application in the study of atrial dynamics has not been tested in patients with MD type 1, and the existence of a relation between the segmental pattern of myocardial deformation and that of electrophysiological abnormalities in these patients was not assessed.These images for the first time provide evidence of concordance between right atrial mechanical and electrophysiological abnormalities in a subject with MD type 1. Using an invasive electroanatomic mapping system, we found a segmental area of low voltages in the inferior portion of the atrial septum, suggesting regional scarring. High agreement between the segmental pattern of low-voltage myocardium and that of myocardial scarring on magnetic resonance imaging was previously established,3 and recent data showed that electroanatomic mapping is even more sensitive than gadolinium-enhanced magnetic resonance imaging in detecting myocardial scars when tested against endomyocardial biopsy.4 Interestingly, the abnormal region identified by voltage mapping was concordant with the segmental impairment in 2-dimensional strain pattern detected by speckle tracking. Although a certain causal association between these abnormalities and the occurrence of relapsing AF cannot be established, these findings suggest that interesting future applications of speckle tracking—to be tested in populations including patients with various types of AF—might include noninvasive evaluation of potential arrhythmic substrate in the atrial myocardium; preprocedural assessment of segmental abnormalities in atrial mechanics, which may provide additional information about the potential electrophysiological mechanism underlying AF and the probable target site of ablation; and risk stratification for postablation arrhythmia recurrence.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.116624/-/DC1.Correspondence to Piercarlo Ballo, MD, Cardiology Unit, S. Maria Annunziata Hospital, Via dell'Antella 58, Florence, Italy. E-mail [email protected]References1. Romeo V. Myotonic dystrophy type 1 or Steinert's disease.Adv Exp Med Biol. 2012; 724:239–257.CrossrefMedlineGoogle Scholar2. Nazarian S, Bluemke DA, Wagner KR, Zviman MM, Turkbey E, Caffo BS, Shehata M, Edwards D, Butcher B, Calkins H, Berger RD, Halperin HR, Tomaselli GF. QRS prolongation in myotonic muscular dystrophy and diffuse fibrosis on cardiac magnetic resonance.Magn Reson Med. 2010; 64:107–114.CrossrefMedlineGoogle Scholar3. Roux JF, Dubuc M, Pressacco J, Roy D, Thibault B, Talajic M, Guerra PG, Macle L, Khairy P. Concordance between an electroanatomic mapping system and cardiac MRI in arrhythmogenic right ventricular cardiomyopathy.Pacing Clin Electrophysiol. 2006; 29:109–112.CrossrefMedlineGoogle Scholar4. Santangeli P, Hamilton-Craig C, Dello Russo A, Pieroni M, Casella M, Pelargonio G, Di Biase L, Smaldone C, Bartoletti S, Narducci ML, Tondo C, Bellocci F, Natale A. Imaging of scar in patients with ventricular arrhythmias of right ventricular origin: cardiac magnetic resonance versus electroanatomic mapping.J Cardiovasc Electrophysiol. 2011; 22:1359–1366.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Fonseca A, Almeida A, Santos M and Ferro J (2021) Neurological complications of cardiomyopathies Heart and Neurologic Disease, 10.1016/B978-0-12-819814-8.00001-9, (91-109), . Guedes H, Moreno N, dos Santos R, Marques L, Seabra D, Pereira A, Andrade A and Pinto P (2018) Importance of three-dimensional speckle tracking in the assessment of left atrial and ventricular dysfunction in patients with myotonic dystrophy type 1, Revista Portuguesa de Cardiologia (English Edition), 10.1016/j.repce.2017.10.010, 37:4, (333-338), Online publication date: 1-Apr-2018. Guedes H, Moreno N, dos Santos R, Marques L, Seabra D, Pereira A, Andrade A and Pinto P (2018) Importance of three-dimensional speckle tracking in the assessment of left atrial and ventricular dysfunction in patients with myotonic dystrophy type 1, Revista Portuguesa de Cardiologia, 10.1016/j.repc.2017.10.011, 37:4, (333-338), Online publication date: 1-Apr-2018. Lau J, Sy R, Corbett A and Kritharides L (2015) Myotonic dystrophy and the heart: A systematic review of evaluation and management, International Journal of Cardiology, 10.1016/j.ijcard.2015.03.069, 184, (600-608), Online publication date: 1-Apr-2015. April 2, 2013Vol 127, Issue 13 Advertisement Article InformationMetrics © 2013 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.112.116624PMID: 23547181 Originally publishedApril 2, 2013 PDF download Advertisement