Marfanoid habitus as a predictor of cardiac arrhythmias in Persons of different age groups


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Abstract

Risk factors leading to the development of cardiac arrhythmias (CA) in young people without organic cardiac pathology are usually related to hereditary disorders (dysplasia) of connective tissue; in their structure, a number of dysplastic phenotypes are distinguished, the most common of which is the marfanoid habitus (MH). Aim of the research. To study the correlation between CA and signs of MH in young people; previously such a relationship was not evaluated, nor was estimated the contribution of MH feature to the heart rhythm structure in older age groups. Material and methods. The study included 119 young people aged 18 to 25 years and 111 patients of older age groups (average age 61.2 ± 8.7 years), with stable over coronary heart disease. Phenotypic examination and holter monitoring of the ECG are performed to all of them. Results. In young people with MH significantly more often were found CA - single supraventricular (SVE) and ventricular (VE) extrasystoles in pathological amounts, as well as paired and group SVE. In the group of older age groups patients with MH, paroxysms of atrial fibrillation (AF) (75% vs 38%, p <0.05) are detected more often. Significantly, the following markers of myocardial electrical instability were determined in persons with MH: the pathological values of the turbulence onset - TO (50% vs 17.9%, p = 0.02) and the turbulence slope - TS (12.5% vs 0%, p = 0.05), elongation QTc> 480ms (21% vs 0%, p = 0.03), increase in QT dispersion > 50ms (37.5% vs 2.6%, p = 0.0004). Conclusion. Young people are often characterized by frequent identification of the predictors of ventricle rhythm disorders, to which we refer the lengthening and increasing of QT interval dispersion, pathological values of heart rhythm turbulence. Signs of electrical instability of the myocardium (EIM) in people with MH are often combining with the identification of clinically significant ventricle rhythm disorders.

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About the authors

Evgeny V. Timofeev

FSBEI of Higher Education «St. Petersburg state pediatric medical university» of the Ministry of Health of Russia

Email: darrieux@mail.ru
PhD, associate professor of the Department of propaedeutics of internal medicine

Svetlana V. Reeva

FSBEI of Higher Education «St. Petersburg state pediatric medical university» of the Ministry of Health of Russia

Email: reeva@mail.ru
PhD., associate professor of the Department of propaedeutics of internal medicine

Mikhail Yu. Lobanov

FSBEI of Higher Education «St. Petersburg state pediatric medical university» of the Ministry of Health of Russia

Email: lobano78@mail.ru
PhD, associate professor of the Department of propaedeutics of internal medicine

Eduard V. Zemtsovsky

FSBEI of Higher Education «St. Petersburg state pediatric medical university» of the Ministry of Health of Russia

Email: zemtsovsky@mail.ru
MD, professor, head of the Department of propaedeutics of internal medicine

References

  1. Бокерия Л.А., Ревишвили А.Ш., Неминущий Н.М. Внезапная сердечная смерть. М., 201 1. 272 с. УДК: 616.12-08-039.71-036.886(035.3).
  2. Priori S.G., Blomstrom-Lundqvist C., Mazzanti A. et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Eur. Heart J. 2015; 17: 1601-87.
  3. De Paepe A., Devereux R.B., Deitz H.C. et al. Revised diagnostic criteria for the Marfan syndrome. Am. J. Med. Genet. 1996; 62: 417-26. doi: 10.1002/(sici)1096-8628(19960424)62:4<417:aid-ajmg15>3.0.co;2-r.
  4. Loeys B.L., Dietz H.C., Braverman A.C. et al. The revised Ghent nosology for the Marfan syndrome. J. Med. Genet. 2010; 47(7): 476-85. doi: 10.1136/jmg.2009.072785.
  5. Beighton P., De Paepe А., Steinmann B. et al. Ehlers-Danlos syndromes: Revised nosology, Villefranche, 1997. Am. J. Med. Genet. 1998; 77 (1): 31-7. PMID: 9557891.
  6. Malfait F., Francomano C., Byers P., Belmont J. et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am. J. Med. Genet. C Semin. Med. Genet. 2017; 75(1): 8-26. doi: 10.1002/ajmg.c.31552.
  7. Aydin A., Adsay B.A., Sheikhzadeh S., Keyser B., Rybczynski M. Observational cohort study of ventricular arrhythmia in adults with Marfan syndrome caused by FBN1 mutations. PLoS One. 2013; 8(12): e81281. doi: 10.1371/journal. pone.0081281.
  8. Hodzic E. Assesment of Rhythm Disorders in Classical and Nonclassical Mitral Valve Prolapse. Med. Arch. 2018; 72(1): 9-12. doi: 10.5455/medarh. 2018.72.9-12.
  9. Schaeffer B.N., Rybczynski M., Sheikhzadeh S., Akbulak R.Ö., Moser J. Heart rate turbulence and deceleration capacity for risk prediction of serious arrhythmic events in Marfan syndrome. Clin. Res. Cardiol. 2015; 104(12): 1054-63. doi: 10.1007/s00392-015-0873-9.
  10. Cherkas A., Zhuraev R. A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations. Cardiol. J. 2016; 23(1): 23-33. doi: 10.5603/CJ.a2015.0076.
  11. Наследственные нарушения соединительной ткани. Российские рекомендации. Кардиоваскулярная терапия и профилактика, 2009, №8(6 S5). С. 2-24. ISSN: 1728-8800.
  12. Наследственные нарушения соединительной ткани в кардиологии. Диагностика и лечение. Российские рекомендации (I пересмотр). Российский кардиологический журнал, 2013, №1. С. 1-32. ISSN: 1560-4071.
  13. Земцовский Э.В., Реева С.В., Малев Э.Г. и др. Алгоритмы диагностики распространенных диспластических синдромов и фенотипов. Теоретические подходы и практическое применение классификации. Артериальная гипертензия, 2009, т. 15, №2. С. 162-165.
  14. Земцовский Э.В., Тимофеев Е.В., Малев Э.Г. Наследственные нарушения (дисплазии) соединительной ткани. Какая из двух действующих национальных рекомендаций предпочтительна? Педиатр, 2017, т. 8, №4. С. 6-18. doi: 10.17816/ ped846-18.
  15. Тимофеев Е.В., Зарипов Б.И., Лобанов М.Ю., Малев Э.Г. и др. Долихостеномелия как критерий диагностики марфаноидной внешности. Трансляционная медицина, 2013, № 4. С. 62-69. УДК: 616.71-007.158.
  16. Kiotsekoglou A., Saha S., Moggridge J.C. et al. Impaired Biventricular Deformation in Marfan Syndrome: A Strain and Strain Rate Study in Adult Unoperated Patients. Echocardiography. 2011; 28(4): 416-30. doi: 10. 1111/j. 1540-8175.2010.01359.x.
  17. Малев Э.Г., Реева С.В., Тимофеев Е.В. и др. Анализ деформации миокарда левого желудочка при пролапсе митрального клапана. Вестник Санкт-Петербургской медицинской академии последипломного образования, 2011, т. 3, №2. С. 134-41. УДК: 616.124.2:616.126.42.
  18. Лунева Е.Б., Малев Э.Г., Коршунова А.Л., Реева С.В. и др. Проявления кардиомиопатии у пациентов с синдромом Марфана и марфаноидной внешностью. Педиатр, 2016, т. 7, №4. С. 96-101. doi: 10.17816/ped7496-101.
  19. Тимофеев Е.В., Малев Э.Г., Земцовский Э.В. Систолическая дисфункция ЛЖ у лиц молодого возраста с марфаноидной внешностью. Кардиология, 2018, №4. С. 29-36.
  20. Реева С.В., Малев Э.Г., Тимофеев Е.В. и др. Вегетативная дисфункция и нарушения реполяризации на ЭКГ покоя и нагрузки у лиц молодого возраста с марфаноидной внешностью и пролапсом митрального клапана. Российский кардиологический журнал, 2015, №7. С. 84-88. doi: 10.15829/1560-4071-2015-07-84-88.
  21. Земцовский Э.В., Парфенова Н.Н., Реева С.В. и др. Возрастные аспекты проблемы диагностики наследственных нарушений структуры и функции соединительной ткани. Артериальная гипертензия, 2008, №14 (2 S2). С. 63-68. ISSN: 1607-419X.
  22. Zipes D.P., Camm A.J., Borggrefe M., Buxton A.E., Chaitman B. et al. ACC/AHA/ESC. 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association. Circulation. 2006; 114(10): e385-484. doi: 10.1161/circulationaha.106.178233.
  23. Schwartz P.J., Moss A.J., Vincent G.M., Crampton R.S. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993; 88(2): 782-4. PMID: 8339437.
  24. Faivre L., Collod-Beroud G., Child A. et al. Contribution of molecular analyses in diagnosing Marfan syndrome and type I fibrillinopathies an international study of 1009 probands. J. Med. Genet. 2008; 45: 384-90. doi: 10.1136/jmg.2007.056382.
  25. Тимофеев Е.В., Зарипов Б.И., Малев Э.Г., Земцовский Э.В. Алгоритм диагностики марфаноидной внешности и морфофункциональные особенности сердца при этом диспластическом фенотипе. Педиатр, 2017, т. 8, №2. С. 24-31. doi: 10.17816/ped8224-31.

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