Quality of Life Research

, Volume 22, Issue 10, pp 2877–2888 | Cite as

The development and validation of a health-related quality of life questionnaire for pre-school children with a chronic heart disease

  • M. Niemitz
  • D. C. M. Seitz
  • M. Oebels
  • D. Schranz
  • H. Hövels-Gürich
  • M. Hofbeck
  • R. Kaulitz
  • C. Galm
  • F. Berger
  • N. Nagdymann
  • B. Stiller
  • T. Borth-Bruhns
  • I. Konzag
  • C. Balmer
  • L. Goldbeck



Heart diseases are often associated with residual injuries, persisting functional restrictions, and long-term sequelae for psychosocial development. Currently, there are no disease-specific instruments to assess the health-related quality of life (HrQoL) of pre-school children. The aims of this study were to develop a parent proxy instrument to measure the HrQoL of children aged 3–7 years with a heart disease and to confirm its validity and reliability.


Items from the Preschool Pediatric Cardiac Quality of Life Inventory (P-PCQLI) were generated through focus groups of caregivers. In a pilot study, comprehensibility and feasibility were tested. Five subdimensions were defined theoretically. Psychometric properties were analysed within a multicentre study with 167 parental caregivers.


The final 52-item instrument contains a total score covering five moderately inter-correlated dimensions. The total score of the questionnaire showed a very high internal consistency (Cronbachs’ α = 0.95). Test–retest correlation was at r tt = 0.96. External validity was indicated by higher correlations (r = 0.24–0.68) with a generic paediatric quality of life questionnaire (KINDL) compared to the Strengths and Difficulties Questionnaire (r = 0.17 to 0.59). Low P-PCQLI total scores were significantly associated with inpatient as opposed to outpatient treatment (t = 6.04, p < .001), with at least moderate disease severity ((t = 5.05, p < .001) NYHA classification) and with poorer prognosis (t = 5.53, p < .001) as estimated by the physician.


The P-PCQLI is reliable and valid for pre-school children with a heart disease. It could be used as a screening instrument in routine care, and for evaluation of HrQoL outcomes in clinical trials and intervention research.


Chronic heart disease Disease-specific instrument Health-related quality of life Parents Pre-school children 



Heart disease


Family-orientated rehabilitation


Health-related quality of life


New York Heart Association classification system



The study was funded by the Stiftung KinderHerz, Essen, Germany.


  1. 1.
    Lindinger, A., Schwedler, G., & Hense, H.-W. (2010). Prevalence of congenital heart defects in Newborns in Germany: Results of the first registration year of the PAN study (July 2006 to June 2007). Klinische Pädiatrie, 2010(222), 321–326.CrossRefGoogle Scholar
  2. 2.
    Apitz, J. (1998). Pädiatrische Kardiologie: Erkrankungen des Herzens bei Neugeborenen, Säuglingen, Kindern und Heranwachsenden. Darmstadt: Steinkopff.CrossRefGoogle Scholar
  3. 3.
    Berkes, A., Pataki, I., Kiss, M., Kemeny, C., Kardos,L., Varni, J. W. et al. (2010). Measuring health-related quality of life in Hungarian children with heart disease: psychometric properties of the Hungarian version of the Pediatric Quality of Life InventoryTM 4.0 Generic Core Scale and Cardiac Module. Health and Quality of Life Outcomes, 8, 14.Google Scholar
  4. 4.
    Nousi, D., & Christou, A. (2010). Factors affecting the quality of life in children with congenital heart disease. Health Science Journal, 4(2), 94–100.Google Scholar
  5. 5.
    Alden, B., Gilljam, T., & Gillberg, C. (1998). Long-term psychological outcome oft children after surgery for transposition of the great arteries. Acta Paediatrica, 87, 405–410.PubMedCrossRefGoogle Scholar
  6. 6.
    Bellinger, D. C., Rappaport, L. A., Wypij, D., Wernovsky, G., & Newburger, J. W. (1997). Patterns of developmental dysfunction after surgery during infancy to correct transposition of the great arteries. Journal of Development and Behavioral Pedatrics, 18(2), 75–83.CrossRefGoogle Scholar
  7. 7.
    Hirose, Y., Ichida, F., & Oshima, Y. (2007). Developmental status of young infants with congenital heart disease. Pediatrics International, 49, 468–471.PubMedCrossRefGoogle Scholar
  8. 8.
    Holm, I., Fredriksen, M., Fosdahl, M. A., Olstad, M., & Vøllestad, N. (2007). Impaired motor competence in school-aged children with complex congenital heart disease. Archives of Pediatrics and Adolescent Medicine, 161(10), 945–950.PubMedCrossRefGoogle Scholar
  9. 9.
    Hoppe, U. C., Böhm, M., Dietz, R., Hanrath, P., Kroemer, H. K., Osterspey, A., et al. (2005). Leitlinien zur Therapie der chronischen Herzinsuffizienz. Zeitschrift für Kardiologie, 94(8), 488–509.PubMedCrossRefGoogle Scholar
  10. 10.
    Karsdorp, P. A., Everared, W., Kindt, M., & Mulder, B. J. M. (2007). Psychological and cognitive functioning in children and adolescents with congenital heart disease: A meta-analysis. Journal of Pediatric Psychology, 32(5), 527–541.PubMedCrossRefGoogle Scholar
  11. 11.
    Connolly, D., McClowry, S., Hayman, L., Mahony, L., & Artman, M. (2004). Posttraumatic stress disorder in children after cardiac surgery. Journal of Pediatrics, 144(4), 480–484.PubMedCrossRefGoogle Scholar
  12. 12.
    Goldbeck, L., & Seitz, D. (2009). Angeborene Herzerkrankungen. In C. von Hagen & H. P. Schwarz (Eds.), Psychische Entwicklung bei chronischer Krankheit im Kindes- und Jugendalter (pp. 83–96). Stuttgart: Kohlhammer.Google Scholar
  13. 13.
    Goldberg, S., Simmons, R. J., Newman, J., Campbell, K., & Fowler, R. S. (1991). Congenital heart disease, parental stress, and infant-mother relationships. The Journal of Pediatrics, 119(4), 661–666.PubMedCrossRefGoogle Scholar
  14. 14.
    Hövels-Gürich, H. H., Konrad, K., Skorzenski, D., Minkenberg, R., Herpetz-Dahlmann, B., Messmer, B. J., et al. (2007). Long-term behavior and quality of life after corrective cardiac surgery in infancy for tetralogy of fallot or ventricular septal defect. Pediatric Cardiology, 28, 346–354.PubMedCrossRefGoogle Scholar
  15. 15.
    Hövels-Gürich, H. H., Konrad, K., Wiesner, M., Minkenberg, R., Herpertz-Dahlmann, B., Messmer, B. J., et al. (2002). Long term behavioral outcome after neonatal arterial switch operation for transposition of the great arteries. Archives of Disease in Childhood, 87, 506–510.PubMedCrossRefGoogle Scholar
  16. 16.
    Stene-Larsen, K., Brandlistuen, R. E., Holmstrøm, H., Landolt, M. A., Eskedal, L. T., & Vollrath, M. E. (2010). Emotional reactivity in infants with congenital heart defects: findings from a large case-cohort study in Norway. Acta Paediatrica, 99, 52–55.PubMedGoogle Scholar
  17. 17.
    Birkeland, A.-L., Rydberg, A., & Hägglöf, B. (2005). The complexity of the psychosocial situation in children and adolescents with heart disease. Acta Paediatrica, 94, 1495–1501.PubMedCrossRefGoogle Scholar
  18. 18.
    Bjorbaekmo, W., & Engelsrud, G. (2008). “I am almost like a fish”: An investigation of how children with congenital heart disease experience and perform movement in daily life. Child: Care, Health and Development, 34(6), 781–788.Google Scholar
  19. 19.
    Brosig, C. L., Mussatto, K. A., Kuhn, E. M., & Tweddell, J. S. (2006). Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease. Pediatric Cardiology, 28, 255–262.CrossRefGoogle Scholar
  20. 20.
    Ekman-Joelsson, B.-M., Berntsson, L., & Sunnegårdh, J. (2004). Quality of life in children with pulmonary atresia and intact ventricular septum. Cardiology in the Young, 14, 615–621.PubMedCrossRefGoogle Scholar
  21. 21.
    Gardner, F. V., Freeman, N. H., Black, A. M., & Angelini, G. D. (1996). Disturbed mother-infant interaction in association with congenital heart disease. Heart, 76, 56–59.PubMedCrossRefGoogle Scholar
  22. 22.
    Goldbeck, L., & Melches, J. (2006). The impact of the severity of disease and social disadvantage and quality of life in families with congenital cardiac disease. Cardiology in the Young, 16, 67–75.PubMedCrossRefGoogle Scholar
  23. 23.
    Goldbeck, L., Melches, J., Franz, A., Voßbeck, S., Lang, D., & Mihatsch, W. (2005). Lebensqualität in Familien mit einem herzkranken Kind. Kindheit und Entwicklung, 14(2), 79–86.CrossRefGoogle Scholar
  24. 24.
    Goldbeck, L., & Melches, J. (2005). Quality of life in families of children with congenital heart disease. Quality of Life Research, 14(8), 1915–1924.PubMedCrossRefGoogle Scholar
  25. 25.
    Granberg, M., Rydberg, A., & Fischer, A. G. (2008). Activities in daily living and schoolwork task performance in children with complex congenital heart disease. Acta Paediatrica, 97, 1270–1274.PubMedCrossRefGoogle Scholar
  26. 26.
    Marino, B. S., Shera, D., Wernovsky, G., Tomlinson, R. S., Aguirre, A., Gallagher, M., et al. (2008). The development of the pediatric cardiac quality of life inventory: A quality of life measure for children and adolescents with heart disease. Quality of Life Research, 17, 613–626.PubMedCrossRefGoogle Scholar
  27. 27.
    Marino, B. S., Lipkin, P. H., Newburger, J. W., Peacock, G., Gerdes, M., Gaynor, J. W., et al. (2012). Neurodevelopmental outcomes in children with congenital heart disease: Evaluation and management: A scientific statement from the american heart association. Circulation, 126, 1143–1172.Google Scholar
  28. 28.
    Uzark, K., Jones, K., Burwinkle, T. M., & Varni, J. W. (2003). The pediatric quality of life inventory in children with heart disease. Progress in Pediatric Cardiology, 18, 141–148.CrossRefGoogle Scholar
  29. 29.
    Varni, J. W., Seid, M., & Rode, C. A. (1999). The PedsQL: Measurement model for the pediatric quality of life inventory. Medical Care, 37, 126–139.PubMedCrossRefGoogle Scholar
  30. 30.
    Eiser, Ch., & Morse, R. (2001). Can parents rate their child’s health-related quality of life? Results of a systematic review. Quality of Life Research, 10, 347–357.PubMedCrossRefGoogle Scholar
  31. 31.
    Marino, B. S., Tomlinson, R. S., Wernovsky, G., Drotar, D., Newburger, J. W., Mahony, L., et al. (2010). Validation of the pediatric cardiac quality of life inventory. Pediatrics, 126(3), 498–508.PubMedCrossRefGoogle Scholar
  32. 32.
    Theunissen, N. C. M., Vogels, T. G. C., Koopman, H. M., Verips, G. H. W., Zwinderman, K. A. H., Verloove-Vanhorick, S. P., et al. (1998). The proxy problem: Child report versus parent report in health-related quality of life research. Quality of Life Research, 7, 387–397.PubMedCrossRefGoogle Scholar
  33. 33.
    Bullinger, M., Schmidt, S., Petersen, C., & Rvans-Sieberer, U. (2006). Quality of life—Evaluation criteria for children with chronic conditions in medical care. Journal of Public Health, 14, 343–355.CrossRefGoogle Scholar
  34. 34.
    Bullinger, M., Siegrist, J., & Ravens-Sieberer, U. (Hrsg.). (2000). Lebensqualitätsforschung aus medizinischpsychologischer und—soziologischer Perspektive. Jahrbuch der Medizinischen Psychologie. Band 18. Hogrefe Verlag für Psychologie.Google Scholar
  35. 35.
    Bullinger, M. (1991). Quality of life: Definition, conceptualisation and implications: A methodologists view. Theoretical Surgery, 6, 143–148.Google Scholar
  36. 36.
    Ravens-Sieberer, U., Gosch, A., Abel, T., Auquier, P., Bellach, B.-M., Bruil, J., et al. (2001). Quality of life in children and adolescents. A European public health perspective. Social and Preventive Medicine, 46(5), 294–302.PubMedCrossRefGoogle Scholar
  37. 37.
    Bullinger, M., Brütt, A. L., Erhart, M., Ravens-Sieberer, U., & The BELLA Study Group. (2008). Psychometric properties of the KINDL-R questionnaire: Results of the BELLA study. European Child and Adolescents Psychiatry, 17(Suppl 1), 125–132.Google Scholar
  38. 38.
    Harstick-Koll, S., Kuschel, A., Bertram, H., Naumann, S., Halweg, K., Hautmann, Ch., et al. (2009). Erfassung der Lebensqualität von Vorschulkindern mit dem Kiddy-KINDLR. Zeitschrift für Gesundheitspsychologie, 17(2), 82–93.CrossRefGoogle Scholar
  39. 39.
    Bullinger, M., von Mackenesen, S., & Kirchberger, I. (1994). KINDL—ein Fragebogen zur Erfassung der gesundheisbezogenen lebensqualität von Kindern. Zeitschrift für Gesundheitspsychologie, 2, 64–77.Google Scholar
  40. 40.
    Goodman, R. (2001). Psychometric properties of the strengths and difficulties questionnaire. Journal of the American Academy of Child and Adolescent Psychiatry, 40(11), 1337–1345.PubMedCrossRefGoogle Scholar
  41. 41.
    Goodman, R., Ford, T., Simmons, H., Gatward, R., & Meltzer, H. (2000). Using the strengths and difficulties questionnaire (SDQ) to screen for child psychiatric disorders in a community sample. The British Journal of Psychiatry, 177, 534–539.PubMedCrossRefGoogle Scholar
  42. 42.
    Goodman, R., & Scott, St. (1999). Comparing the strengths and difficulties questionnaire and the child behavior checklist: Is small beautiful? Journal of Abnormal Child Psychology, 27(1), 17–24.PubMedCrossRefGoogle Scholar
  43. 43.
    Goodman, R. (1997). The strengths and difficulties questionnaire: A research note. Journal of Child Psychology and Psychiatry, 38(5), 581–586.PubMedCrossRefGoogle Scholar
  44. 44.
    Woerner, W., Becker, A., & Rothenberger, A. (2004). Normative data and scale properties of the German parent SDQ. European Journal of Child and Adolescent Psychiatry, 13(2), 11–16.Google Scholar
  45. 45.
    Goldbeck, L., & Braun, R. (2003). LQ-Kid: Ein computergestütztes Verfahren zur Erfassung de Lebensqualität chronisch kranker Kinder und Jugendlicher. Prävention und Rehabilitation, 15(3), 117–126.Google Scholar
  46. 46.
    Goldbeck, L., & Storck, M. (2002). Das Ulmer Lebensqualitäts-Inventar für Eltern chronisch kranker Kinder (ULQIE): Entwicklung und psychometrische Eigenschaften. Zeitschrift für Klinische Psychologie und Psychotherapie, 30(1), 31–39.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  • M. Niemitz
    • 1
  • D. C. M. Seitz
    • 1
  • M. Oebels
    • 2
  • D. Schranz
    • 2
  • H. Hövels-Gürich
    • 3
  • M. Hofbeck
    • 4
  • R. Kaulitz
    • 4
  • C. Galm
    • 5
  • F. Berger
    • 6
  • N. Nagdymann
    • 6
  • B. Stiller
    • 7
  • T. Borth-Bruhns
    • 8
  • I. Konzag
    • 9
  • C. Balmer
    • 10
  • L. Goldbeck
    • 1
  1. 1.Department of Child and Adolescent Psychiatry/PsychotherapyUniversity Ulm Medical CentreUlmGermany
  2. 2.Department of Pediatric Cardiac CentreUniversity Medical Centre Gießen MarburgGießenGermany
  3. 3.Department of Pediatric CardiologyUniversity AachenAachenGermany
  4. 4.Department of Pediatric Cardiology, Pulmology and Intensive Care MedicineUniversity Tübingen Medical CentreTübingenGermany
  5. 5.Section of Pediatric CardiologyUniversity UlmUlmGermany
  6. 6.Department of Congenital Heart Diseases/Paediatric CardiologyGerman Cardiology Centre BerlinBerlinGermany
  7. 7.Department of Pediatrics and Adolescent MedicineUniversity Freiburg Medical CentreFreiburgGermany
  8. 8.Pediatric Aftercare-ClinicTannheimGermany
  9. 9.Pediatric Aftercare-clinic Berlin-Brandenburg gGmbHBernau-WaldsiedlungGermany
  10. 10.Department of CardiologyUniversity Children’s HospitalZürichSwitzerland

Personalised recommendations