Skip to main content

Advertisement

SpringerLink
Log in

Echocardiographic and electrocardiographic analyses of patients with severe motor and intellectual disabilities

  • Original Article
  • Published:
Heart and Vessels Aims and scope Submit manuscript

Abstract

Severe motor and intellectual disabilities (SMID) syndrome is a heterogeneous group of disorders with severe physical disabilities and mental retardation. Higher incidence of sudden death is also known in these patients. However, little is known about the cardiovascular features of patients with SMID. We examine the patients with severe motor and intellectual disabilities using echocardiogram and clarify their characterization of ventricular function. We performed electrocardiographic and echocardiographic analyses in SMID patients. In all patients, two-dimensional echocardiography with tissue Doppler analysis in the pulsed Doppler mode was performed. Of 121 patients, 104 patients had abnormal findings: 81 had poor R-wave progression, and 15 patients had low-voltage QRS on ECG. These findings strongly correlated with the degree of physical disability. However, on echocardiography, most patients had LVEF in the normal range, while LV Tei indices were significantly higher (0.43 vs 0.31 cm/s) and left ventricular end-diastolic dimension significantly smaller than healthy controls (P < 0.05 for each comparison). Patients had significantly decreased early diastolic tissue Doppler velocities at the lateral mitral (5.3 vs 6.7 cm/s), tricuspid (6.7 vs 9.2 cm/s), and septal (5.9 vs 8.8 cm/s) annuli compared with controls (P < 0.05 for each comparison). We show for the first time that SMID patients have low E/Ea ratios on tissue Doppler imaging while LV contractions are normal, suggesting the existence of latent diastolic dysfunction. This may be one of the reasons why the incidence of sudden death is higher in SMID patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Seddon PC, Khan Y (2003) Respiratory problems in children with neurological impairment. Arch Dis Child 881:75–78

    Article  Google Scholar 

  2. Liptak GS, O’Donnell M, Conaway M, Chumlea WC, Wolrey G, Henderson RC, Fung E, Stallings VA, Samson-Fang L, Calvert R, Rosenbaum P, Stevenson RD (2001) Health status of children with moderate to severe cerebral palsy. Dev Med Child Neurol 43:364–370

    Article  PubMed  CAS  Google Scholar 

  3. Saito N, Ebara S, Ohotsuka K, Kumeta H, Takaoka K (1998) Natural history of scoliosis in spastic cerebral palsy. Lancet 351:1687–1692

    Article  PubMed  CAS  Google Scholar 

  4. Sullivan PB, Lambert B, Rose M, Ford-Adams M, Johnson A, Griffiths P (2000) Prevalence and severity of feeding and nutritional problems in children with neurological impairment: Oxford Feeding Study. Dev Med Child Neurol 42:674–680

    Article  PubMed  CAS  Google Scholar 

  5. Couriel JM, Bisset R, Miller R, Thomas A, Clarke M (1993) Assessment of feeding problems in neurodevelopmental handicap: a team approach. Arch Dis Child 69:609–613

    Article  PubMed  CAS  Google Scholar 

  6. Dahl M, Thommessen M, Rasmussen M, Selberg T (1996) Feeding and nutritional characteristics in children with moderate or severe cerebral palsy. Acta Paediatr 85:697–701

    Article  PubMed  CAS  Google Scholar 

  7. Stevenson RD, Hayes RP, Cater LV, Blackman JA (1994) Clinical correlates of linear growth in children with cerebral palsy. Dev Med Child Neurol 36:135–142

    PubMed  CAS  Google Scholar 

  8. Stallings VA, Charney EB, Davies JC, Cronk CE (1993) Nutrition-related growth failure of children with quadriplegic cerebral palsy. Dev Med Child Neurol 35:126–138

    Article  PubMed  CAS  Google Scholar 

  9. Schwarz SM, Corredor J, Fisher-Medina J, Cohen J, Rabinowitz S (2001) Diagnosis and treatment of feeding disorders in children with developmental disabilities. Pediatrics 108:671–676

    Article  PubMed  CAS  Google Scholar 

  10. Reilly S, Skuse D, Poblete X (1996) Prevalence of feeding problems and oral motor dysfunction in children with cerebral palsy: a community survey. J Pediatr 129:877–882

    Article  PubMed  CAS  Google Scholar 

  11. Staiano A, Cucchiara S, Del Giudice E, Andreotti MR, Minella R (1991) Disorders of oesophageal motility in children with psychomotor retardation and gastro-oesophageal reflux. Eur J Pediatr 150:638–641

    Article  PubMed  CAS  Google Scholar 

  12. Spiroglou K, Xinias I, Karatzas N, Karatza E, Arsos G, Panteliadis C(2004) Gastric emptying in children with cerebral palsy and gastroesophageal reflux. Pediatr Neurol 31:177–182

    Article  PubMed  Google Scholar 

  13. Henderson RC, Kairalla J, Abbas A, Stevenson RD (2004) Predicting low bone density in children and young adults with quadriplegic cerebral palsy. Dev Med Child Neurol 46:416–419

    Article  PubMed  Google Scholar 

  14. Soo B, Howard JJ, Boyd RN, Reid SM, Lanigan A, Wolfe R, Reddihough D, Graham HK (2006) Hip displacement in cerebral palsy. J Bone Joint Surg Am 88:121–129

    Article  PubMed  Google Scholar 

  15. Blair E, Watson L, Badawi N, Stanley FJ (2001) Life expectancy among people with cerebral palsy in Western Australia. Dev Med Child Neurol 43:508–515

    Article  PubMed  CAS  Google Scholar 

  16. Reddihough DS, Baikie G, Walstab JE (2001) Cerebral palsy in Victoria, Australia: mortality and causes of death. J Paediatr Child Health 37:183–186

    Article  PubMed  CAS  Google Scholar 

  17. Strauss D, Cable W, Shavelle R (1999) Causes of excess mortality in cerebral palsy. Dev Med Child Neurol 41:580–585

    Article  PubMed  CAS  Google Scholar 

  18. Plioplys AV, Kasnicka I, Lewis S, Moller D (1998) Survival rates among children with severe neurologic disabilities. South Med J 91:161–172

    PubMed  CAS  Google Scholar 

  19. Strauss DJ, Shavelle RM, Anderson TW(1998) Life expectancy of children with cerebral palsy. Pediatr Neurol 18:143–149

    Article  PubMed  CAS  Google Scholar 

  20. Hollins S, Attard MT, von Fraunhofer N, McGuigan S, Sedgwick P (1998) Mortality in people with learning disability: risks, causes, and death certification findings in London. Dev Med Child Neurol 40:50–56

    PubMed  CAS  Google Scholar 

  21. Katz RT (2003) Life expectancy for children with cerebral palsy and mental retardation: implications for life care planning. Neuro-Rehabilitation 18:261–270

    PubMed  Google Scholar 

  22. Oshima K (1971) Basic problem of severely mentally and physically disabled children (in Japanese). Koshu Eisei (Tokyo) 35:648–655

    Google Scholar 

  23. Schiller NB, Shah PM, Crawford M, DeMaria A, Devereux R, Feigenbaum H, Gutgesell H, Reicheck N, Sahn D, Schnittger I, Silverman NH, Tajik AJ (1989) Recommendation for quantitation of the left ventricle by two-dimensional echocardiography: Ameri can Society of Echocardiography Committee on Standards, Subcommittee on Quantitation of Two-Dimensional Echocardiograms. J Am Soc Echocardiogr 2:358–367

    PubMed  CAS  Google Scholar 

  24. Patel R, Nagueh SF, Tsybouleva N, Abdellatif M, Lutucuta S, Kopelen HA, Quinones MA, Zoghbi WA, Entman ML, Roberts R, Marian AJ (2001) Simvastatin induces regression of cardiac hypertrophy and fibrosis and improves cardiac function in a transgenic rabbit model of human hypertrophic cardiomyopathy. Circulation 104:317–324

    Article  PubMed  CAS  Google Scholar 

  25. Tei C (1995) New non-invasive index for combined systolic and diastolic ventricular function. J Cardiol 26:135–136

    PubMed  CAS  Google Scholar 

  26. Weidemann F, Eyskens B, Sutherland GR (2002) New ultrasound methods to quantify regional myocardial function in children with heart disease. Pediatr Cardiol 23:292–306

    Article  PubMed  CAS  Google Scholar 

  27. Nagueh SF, Bachinski LL, Meyer D, Hill R, Zoghbi WA, Tam JW, Quiñones MA, Roberts R, Marian AJ (2001) Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy. Circulation 104:128–130

    PubMed  CAS  Google Scholar 

  28. Nagueh SF, Middleton KJ, Kopelen HA, Zoghbi WA, Quiñones MA (1997) Doppler tissue imaging: a noninvasive technique for evaluation of left ventricular relaxation and estimation of filling pressures. J Am Coll Cardiol 30:1527–1533

    Article  PubMed  CAS  Google Scholar 

  29. Kampmann C, Wiethoff CM, Wenzel A, Stolz G, Betancor M, Wippermann CF, Huth RG, Habermehl P, Knuf M, Emschermann T, Stopfkuchen H (2000) Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe. Heart 83:667–672

    Article  PubMed  CAS  Google Scholar 

  30. Zema MJ, Luminais SK, Chiaramida S, Goldman M, Kligfield P (1980) Electrocardiographic poor R wave progression III. The normal variant. J Electrocardiol 13:135–142

    Article  PubMed  CAS  Google Scholar 

  31. Park HS, Naik SD, Aronow WS, Ahn CW, McClung JA, Belkin RN (2007) Age- and sex-related differences in the tissue Doppler imaging parameters of left ventricular diastolic dysfunction. Echocardiography 24:567–571

    Article  PubMed  Google Scholar 

  32. Roberson DA, Cui W (2007) Right ventricular Tei index in children: effect of method, age, body surface area, and heart rate. J Am Soc Echocardiogr 20:764–770

    Article  PubMed  Google Scholar 

  33. Cui W, Roberson DA, Chen Z, Madronero LF, Cuneo BF (2007) Annular and septal Doppler tissue imaging in children: normal values and Z-score tables, and effects of age, heart rate, and body surface area. J Am Soc Echocardiogr 21:361–370

    PubMed  Google Scholar 

  34. Hogg J, Juhlberg K, Lambe L (2007) Policy, service pathways and mortality: a 10-year longitudinal study of people with profound intellectual and multiple disabilities. J Intellect Disabil Res 51:366–376

    Article  PubMed  CAS  Google Scholar 

  35. Tyrer F, Smith LK, McGrother CW (2007) Mortality in adults with moderate to profound intellectual disability: a population-based study. J Intellect Disabil Res 51:520–527

    Article  PubMed  CAS  Google Scholar 

  36. Patja K, Molsa P, Iivanainen M (2001) Cause-specific mortality of people with intellectual disability in a population-based, 35-year follow-up study. J Intellect Disabil Res 45:30–40

    Article  PubMed  CAS  Google Scholar 

  37. Phillips CA, Danopulos DM, Kezdi P (1988) Noninvasive cardiac material mechanics: application to left ventricular function in quadriplegia. Med Biol Eng Comput 26:333–341

    Article  PubMed  CAS  Google Scholar 

  38. Kessler KM, Pina I, Green B, Burnett B, Laighold M, Bilsker M, Palomo AR, Myerburg RJ (1986) Cardiovascular findings in quadriplegic and paraplegic patients and in normal subjects. Am J Cardiol 58:525–530

    Article  PubMed  CAS  Google Scholar 

  39. Hellerstein HK, Santiago-Stevenson D (1950) Atrophy of the heart: a correlative study of eighty-five proved cases. Circulation 1:93–126

    PubMed  CAS  Google Scholar 

  40. Terzi S, Sayar N, Bilsel T, Enc Y, Yildirim A, Ciloǧlu F, Yesilcimen K (2007) Tissue Doppler imaging adds incremental value in predicting exercise capacity in patients with congestive heart failure. Heart Vessels 22:237–244

    Article  PubMed  Google Scholar 

  41. Koyama J, Ray-Sequin PA, Falk RH (2003) Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 107:2446–2452

    Article  PubMed  Google Scholar 

  42. Poulsen SH, Andersen NH, Ivarsen PI, Mogensen CE, Egeblad H (2003) Doppler tissue imaging reveals systolic dysfunction in patients with hypertension and apparent “isolated” diastolic dysfunction. J Am Soc Echocardiogr 16:724–731

    Article  PubMed  Google Scholar 

  43. Bolognesi R, Tsialtas D, Barilli AL, Manca C, Zeppellini R, Javernaro A, Cucchini F (2001) Detection of early abnormalities of left ventricular function by hemodynamic, echo-tissue Doppler imaging, and mitral Doppler flow techniques in patients with coronary artery disease and normal ejection fraction. J Am Soc Echocardiogr 14:764–772

    Article  PubMed  CAS  Google Scholar 

  44. Yip G, Wang M, Zhang Y, Fung JW, Ho PY, Sanderson JE (2002) Left ventricular long axis function in diastolic heart failure is reduced in both diastole and systole: time for a redefinition? Heart 87:121–125

    Article  PubMed  CAS  Google Scholar 

  45. Vinereanu D, Florescu N, Sculthorpe N, Tweddel AC, Stephens MR, Fraser AG (2001) Differentiation between pathologic and physiologic left ventricular hypertrophy by tissue Doppler assessment of long-axis function in patients with hypertrophic cardiomyopathy or systemic hypertension and in athletes. Am J Cardiol 88:53–58

    Article  PubMed  CAS  Google Scholar 

  46. Senju N, Ikeda S, Koga S, Miyahara Y, Tsukasaki K, Tomonaga M, Kohno S (2007) The echocardiographic Tei-index reflects early myocardial damage induced by anthracyclines in patients with hematological malignancies. Heart Vessels 22:393–397

    Article  PubMed  Google Scholar 

  47. Sohn DW, Chai IH, Lee DJ, Kim HC, Kim HS, Oh BH, Lee MM, Park YB, Choi YS, Seo JD, Lee YW (1997) Assessment of mitral annulus velocity by Doppler tissue imaging in the evaluation of left ventricular diastolic function. J Am Coll Cardiol 30:474–480

    Article  PubMed  CAS  Google Scholar 

  48. Palecek T, Linhart A (2007) Comparison of early diastolic annular velocities measured at various sites of mitral annulus in detection of mild to moderate left ventricular diastolic dysfunction. Heart Vessels 22:67–72

    Article  PubMed  Google Scholar 

  49. Palecek T, Linhart A, Lubanda JC, Magage S, Karetova D, Bultas J, Aschermann M (2006) Early diastolic mitral annular velocity and color M-mode flow propagation velocity in the evaluation of left ventricular diastolic function in patients with Fabry disease. Heart Vessels 21:13–19

    Article  PubMed  Google Scholar 

  50. Farias CA, Rodriguez L, Garcia MJ, Sun JP, Klein AL, Thomas JD (1999) Assessment of diastolic function by tissue Doppler echocardiography: comparison with standard transmitral and pulmonary venous flow. J Am Soc Echocardiogr 12:609–617

    Article  PubMed  CAS  Google Scholar 

  51. Tahara Y, Mizuno H, Ono A, Ishikawa K (1991) Evaluation of the electrocardiographic transitional zone by cardiac computed tomography. J Electrocardiol 24:239–245

    Article  PubMed  CAS  Google Scholar 

  52. Durmala J, Sosnowska M, Sosnowski M (2006) Prevalence of subtle cardiac electrical abnormalities in children with idiopathic scoliosis. Stud Health Technol Inform 123:425–430

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Faculty of Medicine, University of Toyama, 2630 Sugitani, 930-0194, Toyama, Japan

    Keiichi Hirono, Fukiko Ichida & Toshio Miyawaki

  2. Division of Biostatistics and Clinical Epidemiology, Faculty of Medicine, University of Toyama, Toyama, Japan

    Hideki Origasa

  3. Nagaoka Ryoikuen, Niigata, Japan

    Tohru Konishi

Authors
  1. Keiichi Hirono
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tohru Konishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hideki Origasa
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Fukiko Ichida
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Toshio Miyawaki
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Keiichi Hirono.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hirono, K., Konishi, T., Origasa, H. et al. Echocardiographic and electrocardiographic analyses of patients with severe motor and intellectual disabilities. Heart Vessels 24, 46–53 (2009). https://doi.org/10.1007/s00380-008-1070-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00380-008-1070-9

Key words

Search

Navigation