Hostname: page-component-cd9895bd7-dzt6s Total loading time: 0 Render date: 2024-12-27T20:11:19.038Z Has data issue: false hasContentIssue false
  • English
  • Français

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

Published online by Cambridge University Press:  28 August 2019

Asuman N. Karhan Open the ORCID record for Asuman N. Karhan [Opens in a new window] ,
Hayrettin H. Aykan Open the ORCID record for Hayrettin H. Aykan [Opens in a new window] ,
Ersin Gümüş Open the ORCID record for Ersin Gümüş [Opens in a new window] ,
Yasemin Dönmez ,
Dursun Alehan ,
Süheyla Özkutlu ,
Hülya Demir Open the ORCID record for Hülya Demir [Opens in a new window] ,
İnci N. Saltık Temizel Open the ORCID record for İnci N. Saltık Temizel [Opens in a new window] ,
Hasan Özen Open the ORCID record for Hasan Özen [Opens in a new window]  and
Aysel Yüce
Asuman N. Karhan*
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Hayrettin H. Aykan
Affiliation:
Department of Paediatrics, Division of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Ersin Gümüş
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Yasemin Dönmez
Affiliation:
Department of Paediatrics, Division of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Dursun Alehan
Affiliation:
Department of Paediatrics, Division of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Süheyla Özkutlu
Affiliation:
Department of Paediatrics, Division of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Hülya Demir
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
İnci N. Saltık Temizel
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Hasan Özen
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Aysel Yüce
Affiliation:
Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
*
Author for correspondence: A. N. Karhan, Department of Paediatrics, Division of Gastroenterology, Faculty of Medicine, Hacettepe University, Ankara, Turkey. E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.

Method:

Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.

Results:

The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.

Conclusion:

Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

Keywords

Wilson’s diseasecardiac evaluationchildhood
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 9 , September 2019 , pp. 1183 - 1188
Copyright
© Cambridge University Press 2019 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Bandmann, O, Weiss, KH, Kaler, SG, et al. Wilson’s disease and other neurological copper disorders. Lancet Neurol 2015; 14: 103–113.CrossRefGoogle ScholarPubMed
Taly, AB, Meenakshi-Sundaram, S, Sinha, S, Swamy, HS, Arunodaya, GR. Wilson disease: description of 282 patients evaluated over 3 decades. Medicine (Baltimore) 2007; 86: 12–21.CrossRefGoogle ScholarPubMed
Arat, N, Kacar, S, Golbasi, Z, et al. P wave dispersion is prolonged in patients with Wilson’s disease. World J Gastroenterol 2008; 14: 1252–1256.CrossRefGoogle ScholarPubMed
Meenakshi-Sundaram, S, Sinha, S, Rao, M, Prashanth, LK, et al. Cardiac involvement in Wilson’s disease – an electrocardiographic observation. J Assoc Physicians India 2004; 52: 294–296.Google ScholarPubMed
Ozturk, S, Gurbuz, AS, Efe, SC, Iliaz, R, Banzragch, M, Demir, K. QTc interval is prolonged in Wilson’s disease with neurologic involvement. Acta Clin Belg 2018; 73: 328–332.Google ScholarPubMed
Grandis, DJ, Nah, G, Whitman, IR, et al. Wilson’s disease and cardiac myopathy. Am J Cardiol 2017; 120: 2056–2060.CrossRefGoogle ScholarPubMed
Wang, M, Yip, GW, Wang, AY, et al. Peak early diastolic mitral annulus velocity by tissue Doppler imaging adds independent and incremental prognostic value. J Am Coll Cardiol 2003; 41: 820–826.CrossRefGoogle ScholarPubMed
European Association for Study of Liver. EASL clinical practice guidelines: Wilson’s disease. J Hepatol 2012; 56: 671–685.CrossRefGoogle Scholar
Gow, PJ, Smallwood, RA, Angus, PW, Smith, AL, Wall, AJ, Sewell, RB. Diagnosis of Wilson’s disease: an experience over three decades. Gut 2000; 46: 415–419.CrossRefGoogle ScholarPubMed
Devereux, RB, Alonso, DR, Lutas, EM, et al. Echocardiographic assessment of left ventricular hypertrophy: comparison to necropsy findings. Am J Cardiol 1986; 57: 450–458.CrossRefGoogle ScholarPubMed
Daniels, SR, Loggie, JM, Khoury, P, Kimball, TR. Left ventricular geometry and severe left ventricular hypertrophy in children and adolescents with essential hypertension. Circulation 1998; 97: 1907–1911.CrossRefGoogle ScholarPubMed
Gaffney, D, Fell, GS, and O’Reilly, DS. ACP Best Practice No 163. Wilson’s disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol 2000; 53: 807–812.CrossRefGoogle ScholarPubMed
Hedera, P. Update on the clinical management of Wilson’s disease. Appl Clin Genet 2017; 10: 9–19.CrossRefGoogle ScholarPubMed
Socha, P, Janczyk, W, Dhawan, A, et al. Wilson’s disease in children: a position paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2018; 66: 334–344.CrossRefGoogle ScholarPubMed
Kuan, P. Cardiac Wilson’s disease. Chest 1987; 91: 579–583.CrossRefGoogle ScholarPubMed
Soni, D, Shukla, G, Singh, S, Goyal, V, Behari, M. Cardiovascular and sudomotor autonomic dysfunction in Wilson’s disease – limited correlation with clinical severity. Auton Neurosci 2009; 151: 154–158.CrossRefGoogle ScholarPubMed
Li, K, Lindauer, C, Haase, R, et al. Autonomic dysfunction in Wilson’s disease: a comprehensive evaluation during a 3-year follow up. Front Physiol 2017; 8: 778.CrossRefGoogle ScholarPubMed
Okutucu, S, Aytemir, K, and Oto, A. P wave dispersion: what we know till now? JRSM Cardiovasc Dis 2016; 5: 2048004016639443.Google Scholar
Malik, M, Batchvarov, VN. Measurement, interpretation and clinical potential of QT dispersion. J Am Coll Cardiol 2000; 36: 1749–1766.CrossRefGoogle ScholarPubMed
Azevedo, EM, Scaff, M, Barbosa, ER, Neto, AE, Canelas, HM. Heart involvement in hepatolenticular degeneration. Acta Neurol Scand 1978; 58: 296–303.CrossRefGoogle ScholarPubMed
Factor, SM, Cho, S, Sternlieb, I, Scheinberg, IH, Goldfischer, S. The cardiomyopathy of Wilson’s disease. Myocardial alterations in nine cases. Virchows Arch A Pathol Anat Histol 1982; 397: 301–311.CrossRefGoogle ScholarPubMed
Elkiran, O, Karakurt, C, Selimoglu, A, et al. Subclinical diastolic dysfunction in children with Wilson’s disease assessed by tissue Doppler echocardiography: a possible early predictor of cardiac involvement. Acta Cardiol 2013; 68: 181–187.CrossRefGoogle ScholarPubMed
Hlubocka, Z, Marecek, Z, Linhart, A. Cardiac involvement in Wilson disease. J Inherit Metab Dis 2002. 25: 269–277.CrossRefGoogle ScholarPubMed
Dragulescu, A, Mertens, LL. Developments in echocardiographic techniques for the evaluation of ventricular function in children. Arch Cardiovasc Dis. 2010; 103: 603–614.CrossRefGoogle ScholarPubMed
Verdecchia, P, Schillaci, G, Borgioni, C, et al. Adverse prognostic significance of concentric remodeling of the left ventricle in hypertensive patients with normal left ventricular mass. J Am Coll Cardiol 1995; 25: 871–878.CrossRefGoogle ScholarPubMed
Tei, C, Ling, LH, Hodge, DO, et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function – a study in normals and dilated cardiomyopathy. J Cardiol 1995; 26: 357–366.Google ScholarPubMed
Rudski, LG, Lai, WW, Afilalo, J, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010; 23: 685–713; quiz 786–8.CrossRefGoogle Scholar
Kadappu, KK, Thomas, L Tissue Doppler imaging in echocardiography: value and limitations. Heart Lung Circ 2015; 24: 224–233.CrossRefGoogle ScholarPubMed