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Evaluation of cardiac functions in children with familial Mediterranean fever

Published online by Cambridge University Press:  08 April 2024

Sema Yildirim Arslan ,
Dolunay Gurses Open the ORCID record for Dolunay Gurses [Opens in a new window]  and
Selcuk Yuksel
Sema Yildirim Arslan
Affiliation:
Department of Pediatrics, Pamukkale University Faculty of Medicine, Denizli, Turkey
Dolunay Gurses*
Affiliation:
Department of Pediatric Cardiology, Pamukkale University Faculty of Medicine, Denizli, Turkey
Selcuk Yuksel
Affiliation:
Department of Pediatric Rheumatology and Nephrology, Pamukkale University Faculty of Medicine, Denizli, Turkey
*
Corresponding author: D. Gurses; Email: dolunayk@yahoo.com
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Abstract

Objectives:

Familial Mediterranean fever is an autosomal recessive autoinflammatory inherited disease. We aimed to evaluate cardiac involvement in children with familial Mediterranean fever during the attack-free period.

Material and Methods:

The prospective study included 75 familial Mediterranean fever patients during the attack-free period and 50 healthy children. Cardiac evaluation was performed using electrocardiography, 24-hour ambulatory Holter monitoring, and conventional and tissue Doppler echocardiography. Aortic stiffness indices were calculated.

Results:

There were no differences between the groups in age, height, sex, body mass index, and arterial blood pressure parameters (p > 0.05). QT and corrected QT dispersion parameters were similar in both groups (p > 0.05). The E wave velocity and the E/A ratio of the mitral and tricuspid valves decreased, and the A wave velocity of the tricuspid and mitral valve increased in familial Mediterranean fever by the Doppler echocardiography (p < 0.05). The myocardial contraction velocities (Sd), early relaxation velocity (Ed), and Ed/late relaxation velocity (Ad) of both ventricles were decreased in familial Mediterranean fever group, whereas the Ad of both ventricles and the interventricular septum was increased in familial Mediterranean fever group. Aortic strain and distensibility were decreased, and pressure strain elastic modules (Ep), pressure strain normalised (Ep*) by diastolic pressure, and aortic stiffness β index were increased in familial Mediterranean fever patients (p < 0.05). When time domain heart rate variability parameters were evaluated, SDNN-i, RMSSD, and PNN50 significantly decreased in familial Mediterranean fever patients (p < 0.05), whereas SDNN and SDANN were similar in both groups (p > 0.05).

Conclusion:

Our findings showed that cardiac involvement could exist in familial Mediterranean fever patients, even during nonattack periods.

Keywords

Aortic stiffnessfamilial Mediterranean feverheart rate variabilityMEFV geneQT dispersiontissue Doppler echocardiography
Type
Original Article
Information
Cardiology in the Young , First View , pp. 1 - 10
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Copyright
© The Author(s), 2024. Published by Cambridge University Press

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References

Heller, H, Sohar, E, Sherf, L. Familial Mediterranean fever. Arch Int Med 1958; 102: 50–71.CrossRefGoogle ScholarPubMed
Bernot, A, Clepet, C, Dasilva, C, The French FMF Consorsium, et al. A candidate gene for familial Mediterranean fever. Natur Genet 1997; 17: 25–31.Google Scholar
Goldfinger, S. The inherited autoinflammatory syndrome: a decade of discovery. Trans Am Clin Climatol Assoc 2009; 120: 413–418.Google ScholarPubMed
The International FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely the cause familial Mediterranean fever. Cell 1997; 90: 797–807.CrossRefGoogle Scholar
Knockaert, DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J 2007; 28: 1797–1804. DOI: 10.1093/eurheartj/ehm193.CrossRefGoogle ScholarPubMed
Livneh, A, Langevitz, P, Zemer, D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997; 40: 1879–1885. DOI: 10.1002/art.1780401023.CrossRefGoogle ScholarPubMed
Ben-Chetrit, E, Levy, M. Familial Mediterranean fever. Lancet 1998; 351: 659–664. DOI: 10.1016/S0140-6736(97)09408-7.CrossRefGoogle ScholarPubMed
Colak, B, Gurlek, B, Yegin, ZA, et al. The relationship between the MEFV genotype, clinical features, and cytokine-inflammatory activities in patients with familial mediterranean fever. Ren Fail 2008; 30: 187–191. DOI: 10.1080/08860220701810364.CrossRefGoogle ScholarPubMed
Gang Gang, N, Drenth, JP, Langevitz, P, et al. Activation of the cytokine network in familial Mediterranean fever. J Rheumatol 1999; 26: 890–897.Google ScholarPubMed
Baysal, T, Peru, H, Oran, B, et al. Left ventricular diastolic function evaluated with tissue Doppler imaging in children with familial Mediterranean fever. Clin Rheumatol 2009; 28: 23–28. DOI: 10.1007/s10067-008-0976-z.CrossRefGoogle ScholarPubMed
Caliskan, M, Gullu, H, Yilmaz, S, et al. Impaired coronary microvascular function in familial Mediterranean fever. Atherosclerosis 2007; 195: e161–e167. DOI: 10.1016/j.atherosclerosis.2007.06.014.CrossRefGoogle ScholarPubMed
Canpolat, U, Dural, M, Aytemir, K, et al. Evaluation of various cardiac autonomic indices in patients with familial Mediterranean fever on colchicine treatment. Auton Neurosci Basic Clin 2012; 167: 70–74. DOI: 10.1016/j.autneu.2011.11.001.CrossRefGoogle ScholarPubMed
Tavil, Y, Öztürk, MA, Üreten, K, et al. Assessment of aortic wall stiffness in patients with familial Mediterranean fever. Jt Bone Spine 2008; 75: 280–285. DOI: 10.1016/j.jbspin.2007.05.021.CrossRefGoogle ScholarPubMed
Cole, TJ, Flegal, KM, Nicholls, D, et al. Body mass index cut offs to define thinness in children and adolescents: international survey. Br Med J 2007; 335: 194–197. DOI: 10.1136/bmj.39238.399444.55.CrossRefGoogle ScholarPubMed
Bazett, HC. An analysis of the time relations of electrocardiograms. Heart 1920; 7: 353–355.Google Scholar
Sahn, DJ, DeMaria, A, Kisslo, J, Weyman, A. Recommendations regarding quantitation in M-mode echocardiography: results of a survey of echocardiographic measurements. Circulation 1978; 58: 1072–1083. DOI: 10.1161/01.CIR.58.6.1072.CrossRefGoogle ScholarPubMed
Silverman, NH. Quantitative methods to enhance morphological information using M-mode, Doppler, and cross-sectional ultrasound. In: Silverman, NH (ed.) Pediatric Echocardiography. Williams & Wilkins, Baltimore, 1993, pp 35–108.Google Scholar
de Simone, G, Daniels, SR, Devereux, RB, et al. Left ventricular mass and body size in normotensive children and adults: assessment of allometric relations and impact of overweight. J Am Coll Cardiol 1992; 20: 1251–1260. DOI: 10.1016/0735-1097(92)90385-z.CrossRefGoogle ScholarPubMed
Devereux, RB, Reichek, N. Echocardiographic determination of left ventricular mass in man. Anatomic validation of the method. Circulation 1977; 55: 613–618. DOI: 10.1161/01.CIR.55.4.613.CrossRefGoogle ScholarPubMed
Lage, SG, Kopel, L. Arterial elastic properties: comparison of results and units. J Am Coll Cardiol 1992; 20: 1046–1047. DOI: 10.1016/0735-1097(92)90209-6.CrossRefGoogle ScholarPubMed
Stefanadis, C, Stratos, C, Boudoulas, H, et al. Distensibility of the ascending aorta: comparison of invasive and non-invasive techniques in healthy men and in men with coronary artery disease. Eur Heart J 1990; 11: 990–996. DOI: 10.1093/oxfordjournals.eurheartj.a059639.CrossRefGoogle ScholarPubMed
Lacombe, F, Dart, A, Dewar, E, et al. Arterial elastic properties in man: a comparison of echo-Doppler indices of aortic stiffness. Eur Heart J 1992; 13: 1040–1045. DOI: 10.1093/oxfordjournals.eurheartj.a060311.CrossRefGoogle Scholar
Heart Rate Variability: Standards of Measurement, Physiological Interpretation and Clinical Use. Task force of the European society of cardiology and the North American society of pacing and electrophysiology. Circulation 1996; 93: 1043–1065.Google Scholar
Onen, F. Familial Mediterranean fever. Rheumatol Int 2006; 26: 489–496. DOI: 10.1007/s00296-005-0074-3.CrossRefGoogle ScholarPubMed
Yalçinkaya, F, Özen, S, Özçakar, ZB, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology 2009; 48: 395–398. DOI: 10.1093/rheumatology/ken509.CrossRefGoogle ScholarPubMed
Ece, A, Çakmak, E, Uluca, Ü., et al. The MEFV mutations and their clinical correlations in children with familial Mediterranean fever in southeast Turkey. Rheumatol Int 2014; 34: 207–212. DOI: 10.1007/s00296-013-2858-1.CrossRefGoogle ScholarPubMed
Cobankara, V, Kiraz, S. Ailesel Akdeniz Ateşi. Hacettepe Tıp Dergisi 2000; 31: 310–319.Google Scholar
Farag, Y, Sayed, S, Mostafa, FA, Marzouk, H, Mohamed, RH, Sobhy, R. Cardiac repolarization abnormalities in children with familial Mediterranean fever. Pediatr Rheumatol 2022; 20: 1–7. DOI: 10.1186/s12969-022-00696-5.CrossRefGoogle ScholarPubMed
Koca, B, Kasapçopur, Ö., Bakari, S, et al. QT dispersion and cardiac involvement in children with familial Mediterranean fever. Cardiol Young 2012; 22: 404–409. DOI: 10.1017/S1047951111001867.CrossRefGoogle ScholarPubMed
Akcay, A, Acar, G, Sayarlioglu, M, et al. QT dispersion and transmural dispersion of repolarization in patients with familial Mediterranean fever. Mod Rheumatol 2009; 19: 550–555. DOI: 10.3109/s10165-009-0196-7.CrossRefGoogle ScholarPubMed
Şahin, M, Kır, M, Makay, B, et al. Cardiac autonomic functions in children with familial Mediterranean fever. Clin Rheumatol 2016; 35: 1237–1244. DOI: 10.1007/s10067-014-2714-z.CrossRefGoogle ScholarPubMed
Topal, F, Tanindi, A, Kurtoglu, HG, et al. QT dispersion is not increased in familial mediterranean fever. J Int Med Res 2011; 39: 2006–2011. DOI: 10.1177/147323001103900547.CrossRefGoogle Scholar
Nussinovitch, N, Livneh, A, Katz, K, et al. QT dispersion in uncomplicated familial Mediterranean fever. Clin Rheumatol 2010; 29: 1353–1356. DOI: 10.1007/s10067-010-1434-2.CrossRefGoogle ScholarPubMed
Nussinovitch, U, Kaminer, K, Nussinovitch, M, et al. QT interval variability in familial Mediterranean fever: a study in colchicine-responsive and colchicine-resistant patients. Clin Rheumatol 2012; 31: 795–799. DOI: 10.1007/s10067-011-1926-8.CrossRefGoogle ScholarPubMed
Topaloglu, R, Ozaltin, F, Yilmaz, E, et al. E148Q is a disease-causing MEFV mutation: a phenotypic evaluation in patients with familial Mediterranean fever. Ann Rheum Dis 2005; 64: 750–752. DOI: 10.1136/ard.2004.026963.CrossRefGoogle ScholarPubMed
Yalçinkaya, F, Çakar, N, Misirlioğlu, M, et al. Genotype-phenotype correlation in a large group of Turkish patients with familial Mediterranean fever: evidence for mutation-independent amyloidosis. Rheumatology 2000; 39: 67–72. DOI: 10.1093/rheumatology/39.1.67.CrossRefGoogle Scholar
Panossian, A, Hambartsumyan, M, Panosyan, L, et al. Plasma nitric oxide level in familial Mediterranean fever and its modulations by immuno-guard. Nitric Oxide - Biol Chem 2003; 9: 103–110. DOI: 10.1016/j.niox.2003.08.005.CrossRefGoogle ScholarPubMed
Tavil, Y, Üreten, K, Öztürk, MA, et al. The detailed assessment of left and right ventricular functions by tissue Doppler imaging in patients with familial Mediterranean fever. Clin Rheumatol 2008; 27: 189–194. DOI: 10.1007/s10067-007-0676-0.CrossRefGoogle ScholarPubMed
Ardic, I, Kaya, MG, Yarlioglues, M, et al. Assessment of heart rate recovery index in patients with familial Mediterranean fever. Rheumatol Int 2011; 31: 121–125. DOI: 10.1007/s00296-010-1550-y.CrossRefGoogle ScholarPubMed
Ozdemir, O, Agras, PI, Aydin, Y, et al. Assessment of cardiac functions using tissue Doppler imaging in children with familial Mediterranean fever. Cardiol Young 2012; 22: 188–193. DOI: 10.1017/S1047951111001168.CrossRefGoogle ScholarPubMed
Kalkan, GY, Bayram, NA, Erten, S, et al. Evaluation of left ventricle function by strain imaging in patients with familial Mediterranean fever. Echocardiography 2010; 27: 1056–1060. DOI: 10.1111/j.1540-8175.2010.01217.x.CrossRefGoogle ScholarPubMed
Sari, I, Arican, Ö., Can, G, et al. Assessment of aortic stiffness and ventricular functions in familial Mediterranean fever. Anadolu Kardiyol Derg 2008; 8: 271–278.Google ScholarPubMed
Terekeci, HM, Ulusoy, ER, Kucukarslan, NM, et al. Familial Mediterranean fever attacks do not alter functIonal and morphologIc tissue Doppler echocardIographIc parameters. Rheumatol Int 2008; 28: 1239–1243. DOI: 10.1007/s00296-008-0648-y.CrossRefGoogle Scholar
Ceylan, Ö., Özgür, S, Örün, UA, et al. Assessment of left ventricular functions with tissue Doppler, strain, and strain rate echocardiography in patients with familial Mediterranean fever. Anadolu Kardiyol Derg 2015; 15: 663–668. DOI: 10.5152/akd.2014.5544.Google ScholarPubMed
Mitchell, GF, Izzo, JL Jr. Evaluation of arterial stiffness. In Hypertension Primer, 3rded. American Heart Association, Dallas, Tex, 2003: 351–355.Google Scholar
Pannier, B, Guérin, AP, Marchais, SJ, et al. Stiffness of capacitive and conduit arteries: prognostic significance for end-stage renal disease patients. Hypertension 2005; 45: 592–596. DOI: 10.1161/01.HYP.0000159190.71253.c3.CrossRefGoogle ScholarPubMed
Pereira, T, Maldonado, J, Pereira, L, et al. Aortic stiffness is an independent predictor of stroke in hypertensive patients. Arq Bras Cardiol 2013; 100: 437–443. DOI: 10.5935/abc.20130079.Google ScholarPubMed
Yildiz, M, Masathoglu, S, Seymen, P, et al. The carotid-femoral (aortic) pulse wave velocity as a marker of arterial stiffness in familial Mediterranean fever. Can J Cardiol 2006; 22: 1127–1131. DOI: 10.1016/S0828-282X(06)70949-7.CrossRefGoogle ScholarPubMed
Sgouropoulou, V, Stabouli, S, Trachana, M. Arterial stiffness in familial Mediterranean fever: correlations with disease-related parameters and colchicine treatment. Clin Rheumatol 2019; 38: 2577–2584. DOI: 10.1007/s10067-019-04601-6.CrossRefGoogle ScholarPubMed
Türkuçar, S, Yıldız, K, Küme, T, et al. Does familial Mediterranean fever provoke atherosclerosis in children? Evaluation of arterial stiffness and serum endocan levels. Clin Rheumatol 2021; 40: 4199–4206. DOI: 10.1007/s10067-021-05721-8.CrossRefGoogle ScholarPubMed
Kaya, H, Suner, A, Koroglu, S, et al. Heart rate variability in familial Mediterranean fever patients. Eur J Rheumatol 2014; 49: 58–61. DOI: 10.5152/eurjrheumatol.2014.013.CrossRefGoogle Scholar
Moog, P, Valda, K, Eren, OE, et al. A complex pattern of autonomic dysfunction in familial Mediterranean fever. Results from a controlled cross-sectional study. Clin Exp Rheumatol 2019; 121: 35–42.Google Scholar
Nussinovitch, N, Esev, K, Lidar, M, et al. Normal heart rate variability in colchicine-resistant familial Mediterranean fever patients. Isr Med Assoc J 2015; 17: 306–309.Google ScholarPubMed
Fidanci, K, Gulgun, M, Demirkaya, E, et al. Assessment of autonomic functions in children with familial Mediterranean fever by using heart rate variability measurements. Int J Rheum Dis 2017; 20: 2086–2092. DOI: 10.1111/1756-185X.12401.CrossRefGoogle ScholarPubMed
Touitou, I. The spectrum of familial Mediterranean fever (FMF) mutations. Eur J Hum Genet 2001; 9: 473–483. DOI: 10.1038/sj.ejhg.5200658.CrossRefGoogle ScholarPubMed
Urfali, M, Yilmaz, G, Özkul, B, Urfali, FE. Indirect evaluation of amyloid deposition by ultrasonography and its relationship with MEFV gene mutation in FMF patients. J Clin Ultrasound 2022; 51: 1–8. DOI: 10.1002/jcu.23409.Google ScholarPubMed
Grimaldi, MP, Candore, G, Vasto, S, et al. Role of the pyrin M694V (A2080G) allele in acute myocardial infarction and longevity: a study in the Sicilian population. J Leukoc Biol 2006; 79: 611–615. DOI: 10.1189/jlb.0705416.CrossRefGoogle ScholarPubMed
Evrengül, H, Yüksel, S, Doğan, M, et al. Deteriorated systolic blood pressure recovery and heart rate recovery after graded exercise in children with familial mediterranean fever. Arch Rheumatol 2017; 32: 244–249. DOI: 10.5606/ArchRheumatol.2017.6071.CrossRefGoogle ScholarPubMed
Imazio, M, Nidorf, M. Colchicine and the heart. Eur Heart J 2021; 42: 2745–2760. DOI: 10.1093/eurheartj/ehab221.CrossRefGoogle ScholarPubMed
Gasparyan, AY, Ayvazyan, L, Yessirkepov, M, et al. Colchicine as an anti-inflammatory and cardioprotective agent. Expert Opin Drug Metab Toxicol 2015; 11: 1781–1794.CrossRefGoogle ScholarPubMed
Yuksel, S, Ayvazyan, L, Gasparyan, AY. Familial Mediterranean fever as an emerging clinical model of atherogenesis associated with low-grade inflammation. Open Cardiovasc Med J 2010; 4: 51–56.Google ScholarPubMed
Adler, Y, Finkelstein, Y, Guindo, J, et al. Colchicine treatment for recurrent pericarditis. A decade of experience. Circulation 1998; 97: 2183–2185.CrossRefGoogle ScholarPubMed
Ambartsymian, SV. Myocardial infarction in patients with familial Mediterranean fever and cardiac lesions. Georgian Med News 2012; 204: 62–66.Google Scholar
Langevitz, P, Livneh, A, Neumann, L, et al. Prevalence of ischemic heart disease in patients with familial Mediterranean fever. Isr Med Assoc J 2001; 3: 9–12.Google ScholarPubMed
Sari, I, Karaoglu, O, Can, G, et al. Early ultrasonographic markers of atherosclerosis in patients with familial Mediterranean fever. Clin Rheumatol 2007; 26: 1467–1473.CrossRefGoogle ScholarPubMed
Ocal, AG, Ocal, L, Kup, A, Eren, H, Tezcan, ME. Colchicine’s effects on electrocardiographic parameters in newly diagnosed familial Mediterranean fever patients. Zeitschrift für Rheumatologie 2020; 79: 210–215. DOI: 10.1007/s00393-019-0642-7.CrossRefGoogle ScholarPubMed
Nussinovitch, U, Stahi, T, Livneh, A. Evaluation of a proarrhythmic repolarization marker (Total cosine R to T) in patients with uncomplicated familial Mediterranean fever. J Clin Rheumatol 2020; 26: 334–337. DOI: 10.1097/RHU.0000000000001465.CrossRefGoogle Scholar