Heart Rate Turbulence as a Noninvasive Risk Predictor of Ventricular Tachyarrhythmias in Myotonic Dystrophy Type 1
MICHELA CASELLA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorANTONIO DELLO RUSSO M.D., Ph.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorMANUELA PACE M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorGEMMA PELARGONIO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorCAROLINA IERARDI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorTOMMASO SANNA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorLOREDANA MESSANO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorGIANLUIGI BENCARDINO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFORTUNATO MANGIOLA M.D.
Neuromuscular Centre, UILDM, Rome, Italy
Search for more papers by this authorGAETANO A. LANZA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorPAOLO ZECCHI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFILIPPO CREA M.D., F.A.C.C.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFULVIO BELLOCCI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorMICHELA CASELLA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorANTONIO DELLO RUSSO M.D., Ph.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorMANUELA PACE M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorGEMMA PELARGONIO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorCAROLINA IERARDI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorTOMMASO SANNA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorLOREDANA MESSANO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorGIANLUIGI BENCARDINO M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFORTUNATO MANGIOLA M.D.
Neuromuscular Centre, UILDM, Rome, Italy
Search for more papers by this authorGAETANO A. LANZA M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorPAOLO ZECCHI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFILIPPO CREA M.D., F.A.C.C.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorFULVIO BELLOCCI M.D.
Institute of Cardiology, Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy
Search for more papers by this authorThis study was supported by Telethon, Italy, Grant GUP02067. Mr. Valsecchi is a Medtronic employee.
Manuscript received 20 January 2006; Revised manuscript received 21 March 2006; Accepted for publication 28 March 2006.
Abstract
Introduction: Myotonic dystrophy type 1 (MD1) is the most common muscular dystrophy of adult life. Cardiac involvement is characterized by disorders of atrioventricular conduction, ventricular arrhythmias, and sudden death. Heart rate turbulence (HRT) is a noninvasive risk predictor in patients affected by ischemic heart disease. The aim of our study is to assess the prognostic value of HRT in MD1 patients.
Methods and Results: We performed HRT analysis by 24-hour Holter recording to calculate turbulence onset (TO) and turbulence slope (TS) in 29 MD1 patients (mean age 52 ± 10 years), and in 30 patients (mean age 52 ± 13 years) with frequent ventricular arrhythmias and structurally normal heart (VANH). An electrophysiological study (EPS) tested ventricular arrhythmias inducibility in 22 MD1 patients.
TO was significantly different between MD1 and VANH patients (−1.66 ± 2.04 and −2.98 ± 1.79%, respectively, P 0.01), while no difference was observed in TS between MD1 and VANH patients (11.12 ± 6.46 and 9.12 ± 6 msec/beat, respectively). On EPS, sustained ventricular arrhythmias (SVA) were induced in six MD1 patients. TO was significantly different in inducible MD1 patients (0.88 ± 1.95%), as compared with both noninducible (−2.49 ± 1.43%, P < 0.001) or no eligible to EPS (−1.93 ± 1.63%, P < 0.005) MD1 patients and to VANH patients (−2.98 ± 1.79%, P < 0.001).
Conclusions: An impairment of TO, a measure of HRT, suggesting impaired cardiac parasympathetic activity, may be a useful, noninvasive predictor of arrhythmic risk in MD1 patients.
References
- 1 Ashizawa T, Epstein HF: Ethnic distribution of myotonic dystrophy gene. Lancet 1991; 338: 642-643.
- 2 Harley HG, Brook JD, Rundle SA, Crow S, Reardon W, Buckler AJ, Housman DE, Shaw DJ: Expansion of an unstable DNA region and phenotypic variation in myotonic dystrophy. Nature 1992; 355: 545-546.
- 3 Harper PS: Myotonic Dystrophy. Second edition. London : WB Saunders, 1989.
- 4 Philips MF, Harper PS: Cardiac disease in myotonic dystrophy. Cardiovasc Res 1997; 33: 13-22.
- 5 Lazarus A, Varin J, Ounnoughene Z, Radvanyi H, Junien C, Laforet P, Eymard B, Becane HM, Weber S, Duboc D: Relationship among electrophysiological findings and clinical status, heart function, and extent of DNA mutation in myotonic dystrophy. Circulation 1999; 99: 1041-1046.
- 6 Prystowsky EN, Pritchett EL, Roses AD, Gallagher J: The natural history of conduction system disease in myotonic muscular dystrophy as determined by serial electrophysiologic studies. Circulation 1979 Dec; 60: 1360-1364.
- 7 De Die-Smulders CE, Howeler CJ, Thijs C, Mirandolle JF, Anten HB, Smeets HJ, Chandler KE, Geraedts JP: Age and causes of death in adult-onset myotonic dystrophy. Brain 1998; 121: 1557-1563.
- 8 Hiromasa S, Ikeda T, Kubota K, Hattori N, Nishimura M, Watanabe Y, Maldonado C, Palakurthy PM, Kupersmith J: Myotonic dystrophy: Ambulatory electrocardiogram, electrophysiologic study, and echocardiographic evaluation. Am Heart J 1987; 113: 1482-1488.
- 9 Melacini P, Villanova C, Menegazzo E, Novelli G, Danieli G, Rizzoli G, Fasoli G, Angelini C, Buja G, Miorelli M, Dallapiccola B, Dalla Volta S: Correlation between cardiac involvement and CTG trinucleotide repeat length in myotonic dystrophy. J Am Coll Cardiol 1995; 25: 239-245.
- 10 Grigg LE, Chan W, Mond HG, Vohra JK, Downey WF: Ventricular tachycardia and sudden death in myotonic dystrophy: Clinical, electrophysiologic, and pathologic features. J Am Coll Cardiol 1985; 6: 254-258.
- 11 Church SC: The heart in myotonia atrophica. Arch Intern Med 1967; 119: 176-181.
- 12 Clarke NR, Kelion AD, Nixon J, Hilton-Jones D, Forfar JC: Does cytosine-thymine-guanine (CTG) expansion size predict cardiac events and electrocardiographic progression in myotonic dystrophy? Heart 2001; 86: 411-416.
- 13 Baciarello G, Villani M, Di Maio F, Sciacca A: Late surface potentials in myotonic dystrophy with ventricular tachycardia. Am Heart J 1986; 111: 413-414.
- 14 Babuty D, Fauchier L, Tena-Carbi D, Poret P, Leche J, Raynaud M, Fauchier JP, Cosnay P: Significance of late ventricular potentials in myotonic dystrophy. Am J Cardiol 1999; 84: 1099-1101.
- 15 Forsberg H, Olofsson BO, Andersson S, Henriksson A, Bjerle P: 24-Hour electrocardiographic study in myotonic dystrophy. Cardiology 1988; 75: 241-249.
- 16 Babuty D, Fauchier L, Tena-Carbi D, Poret P, Leche J, Raynaud M, Fauchier JP, Cosnay P: Is it possible to identify infrahissian cardiac conduction abnormalities in myotonic dystrophy by non invasive methods? Heart 1999; 82: 634-637.
- 17 Dello Russo A, Pelargonio G, Parisi Q, Santamaria M, Messano L, Sanna T, Casella M, De Martino G, De Ponti R, Pace M, Giglio V, Ierardi C, Zecchi P, Crea F, Bellocci F: Widespread electroanatomic alterations of right cardiac chambers in patients with Myotonic Dystrophy type 1. J Cardiovasc Electrophysiol 2006; 17: 34-40.
- 18 Schmidt G, Malik M, Barthel P, Schneider R, Ulm K, Rolnitzky L, Camm AJ, Bigger JT Jr, Schömig A: Heart-rate turbulence after ventricular premature beats as a predictor of mortality after acute myocardial infarction. Lancet 1999; 353: 1390-1396.
- 19 Lin L, Lai L, Lin J, Du C, Shau W, Chan H, Tseng Y, Huang SKS: Tight mechanism correlation between heart rate turbulence and baroreflex sensitivity: Sequential autonomic blockade analysis. J Cardiovasc Electrophysiol 2002; 13: 427-431.
- 20 Barthel P, Schneider R, Bauer A, Ulm K, Schmitt C, Schomig A, Schmidt G: Risk stratification after acute myocardial infarction by heart rate turbulence. Circulation 2003; 108: 1221-1226.
- 21 Sade E, Aytemir K, Oto A, Nazli N, Ozmen F, Ozkutlu H, Tokgozoglu L, Aksoyek S, Ovunc K, Kabakci G, Ozer N, Kes S: Assessment of heart rate turbulence in the acute phase of myocardial infarction for long-term prognosis. Pacing Clin Electrophysiol 2003; 26(2 Pt 1): 544-550.
- 22 Ghuran A, Reid F, La Rovere MT, Schmidt G, Bigger JT, Camm AJ, Schwartz PJ, Malik M: Heart rate turbulence-based predictors of fatal and nonfatal cardiac arrest (the autonomic tone and reflexes after myocardial infarction substudy). Am J Cardiol 2002; 89: 184-190.
- 23 Berkowitsch A, Zareba W, Neumann T, Erdogan A, Mc Nitt S, Moss AJ, Pitschner HF: Risk stratification using heart rate turbulence and ventricular arrhythmia in MADIT II: Usefulness and limitations of a 10-minute Holter recording. Ann of Noninvasive Electrocardiol 2004; 9: 270-279.
- 24 Bonnemeier H, Wiegand UK, Friedlbinder J, Schulenburg S, Hartmann F, Bode F, Katus HA, Richardt G: Reflex cardiac activity in ischemia and reperfusion: Heart rate turbulence in patients undergoing direct percutaneous coronary intervention for acute myocardial infarction. Circulation 2003; 108: 958-964.
- 25 Cygankiewicz I, Wranicz JK, Bolinska H, Zaslonka J, Zareba W: Relationship between heart rate turbulence and heart rate, heart rate variability, and number of ventricular premature beats in coronary patients. J Cardiovasc Electrophysiol 2004; 15: 731-737.
- 26 Grimm W, Sharkova J, Christ M, Müller HH, Schmidt G, Maisch B: Prognostic significance of heart rate turbulence following ventricular premature beats in patients with idiopathic dilated cardiomyopathy. J Cardiovasc Electrophysiol 2003; 14: 819-824.
- 27 Malberg H, Bauernschmitt R, Meyerfeldt U, Schirdewan A, Wessel N: Short-term heart rate turbulence analysis versus variability and baroceptor sensitivity in patients with dilated cardiomyopathy. Z Kardiol 2003; 92: 547-557.
- 28 Ribeiro AL, Schmidt G, Sousa MR, Lombardi F, Gomes ME, Perez AA, Barros MV, Machado FS, Rocha MO: Heart rate turbulence in Chagas disease. Pacing Clin Electrophysiol 2003; 26: 406-410.
- 29 Watanabe M, Schmidt G: Heart rate turbulence: A 5-year review. Heart Rhythm 2004; 1: 732-738.
- 30 Sestito A, Valsecchi S, Infusino F, Sgueglia GA, Bellocci F, Zecchi P, Crea F, Lanza GA: Differences in heart rate turbulence between patients with coronary artery disease and patients with ventricular arrhythmias but structurally normal hearts. Am J Cardiol 2004; 94: 1105-1106.
- 31 Pelargonio G, Dello Russo A, Sanna T, De Martino G, Bellocci F: Myotonic dystrophy and the heart. Heart 2002; 88: 665-670.
- 32
Merino JL,
Carmona JR,
Fernandez-Lozano I,
Peinado R,
Basterra N,
Sobrino JA: Mechanisms of sustained ventricular tachycardia in myotonic dystrophy: Implication for catheter ablation.
Circulation
1988; 98: 541-546.
10.1161/01.CIR.98.6.541 Google Scholar
- 33 Mathieu J, De Braekeleer M, Prévost C, Boily C: Myotonic dystrophy: Clinical assessment of muscular disability in an isolated population with presumed homogeneous mutation. Neurology 1992; 42: 203-208.
- 34 Mathieu J, Allard P, Potvin L, Prevost C, Begin P: A 10-year study of mortality in a cohort of patients with myotonic dystrophy. Neurology 1999; 52: 1658-1664.
- 35 Kawasaki T, Azuma A, Asada S, Hadase M, Kamitani T, Kawasaki S, Kuribayashi T, Sugihara H: Heart rate turbulence and clinical prognosis in hypertrophic cardiomyopathy and myocardial infarction. Circ J 2003; 67: 601-604.
- 36 Hiromasa S, Ikeda T, Kubota K, Hattori N, Coto H, Maldonado C, Kupersmith J: Ventricular tachycardia and sudden death in myotonic dystrophy. Am Heart J 1988; 115: 914-915.
- 37 Mrowka R, Persson PB, Theres H, Patzak A: Blunted arterial baroreflex causes “pathological” heart rate turbulence. Am J Physiol Regu Integr Comp Physiol 2000; 279: R1171-R1175.
- 38 Watanabe MA, Marine JE, Sheldon R, Josephson ME: Effects of ventricular premature stimulus coupling interval on blood pressure and heart rate turbulence. Circulation 2002; 106: 325-330.
- 39 Di Leo R, Rodolico C, De Gregorio C, Recupero A, Coglitore S, Annessi G, Toscano A, Messina C, Vita G: Cardiovascular autonomic control in myotonic dystrophy type 1: A correlative study with clinical and genetic data. Neuromuscul Disord 2004; 14: 136-141.
- 40 Hardin BA, Lowe MR, Bhakta D: Heart rate variability declines with increasing age and CTG repeat length in patients with myotonic dystrophy type 1. Ann Noninvasive Electrocardiol 2003; 8: 227-232.
- 41 Marine JE, Watanabe MA, Smith TW, Monahan KM: Effect of atropine on heart rate turbulence. Am J Cardiol 2002; 89: 767-769.
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