Pediatric Cardiology

, Volume 34, Issue 1, pp 159–164 | Cite as

Decreased Right Ventricular Function in Healthy Pediatric Cystic Fibrosis Patients Versus Non-Cystic Fibrosis Patients

  • Nazire Ozcelik
  • Richard Shell
  • Melissa Holtzlander
  • Clifford Cua
Original Article


Right ventricular (RV) dysfunction may occur in patients with cystic fibrosis (CF). Tissue Doppler imaging (TDI) and strain and strain rate analysis are new echocardiographic tools that can quantitate RV function. This study aimed to compare the RV function between healthy CF patients and non-CF patients to determine whether differences exist. Healthy CF children and age-matched non-CF children were enrolled in the study. In this study, TDI analysis of the RV free wall was performed at the level of the tricuspid valve annulus. Two-dimensional speckle echocardiography was used to measure global strain and strain rates in a six-segment model of the RV. Independent t tests were used to compare the groups. The study enrolled 18 CF patients (age, 7.7 ± 2.0 years) and 15 non-CF patients (age, 6.4 ± 2.5 years). The age difference was not significant. The forced expiratory volume in the first second (FEV1) for the CF patients was 91 ± 15 %. The systolic annular velocity by TDI was significantly lower in the CF group. The RV strain values in the CF group were significantly lower in four of the six segments (RV free-wall base, RV free-wall mid, RV septal apex, and RV septal mid). The global strain value, the systolic strain rate, and the early diastolic strain rate were significantly lower in the CF group. Decreased systolic and diastolic RV properties were present in young healthy CF patients. Further studies with longitudinal follow-up evaluation are needed to determine the significance of these findings for the pediatric CF population.


Cystic fibrosis Echocardiography Pediatrics Right ventricle Strain echocardiography Tissue doppler 


  1. 1.
    Abraham TP, Dimaano VL, Liang HY (2007) Role of tissue Doppler and strain echocardiography in current clinical practice. Circulation 116:2597–2609PubMedCrossRefGoogle Scholar
  2. 2.
    Bleeker GB, Steendijk P, Holman ER, Yu CM, Breithardt OA, Kaandorp TA et al (2006) Assessing right ventricular function: the role of echocardiography and complementary technologies. Heart 92(Suppl 1):i19–i26PubMedCrossRefGoogle Scholar
  3. 3.
    Bright-Thomas RJ, Webb AK (2002) The heart in cystic fibrosis. J R Soc Med 95(Suppl 41):2–10PubMedGoogle Scholar
  4. 4.
    Burghuber OC, Salzer-Muhar U, Gotz M (1988) Right ventricular contractility is preserved in patients with cystic fibrosis and pulmonary artery hypertension. Scand J Gastroenterol Suppl 143:93–98PubMedCrossRefGoogle Scholar
  5. 5.
    Chow PC, Liang XC, Cheung EW, Lam WW, Cheung YF (2008) New two-dimensional global longitudinal strain and strain rate imaging for assessment of systemic right ventricular function. Heart 94:855–859PubMedCrossRefGoogle Scholar
  6. 6.
    Davies JC, Alton EW, Bush A (2007) Cystic fibrosis. BMJ 335:1255–1259PubMedCrossRefGoogle Scholar
  7. 7.
    Hofmann H, Vogt L, Volkner E (1990) Quantitative two-dimensional echocardiography: study results in patients with cystic fibrosis. Kinderarztl Prax 58:523–530PubMedGoogle Scholar
  8. 8.
    Huez S, Vachiery JL, Unger P, Brimioulle S, Naeije R (2007) Tissue Doppler imaging evaluation of cardiac adaptation to severe pulmonary hypertension. Am J Cardiol 100:1473–1478PubMedCrossRefGoogle Scholar
  9. 9.
    Ionescu AA, Nixon LS, Evans WD, Stone MD, Lewis-Jenkins V, Chatham K et al (2000) Bone density, body composition, and inflammatory status in cystic fibrosis. Am J Respir Crit Care Med 162(3 Pt 1):789–794PubMedGoogle Scholar
  10. 10.
    Ionescu AA, Payne N, Obieta-Fresnedo I, Fraser AG, Shale DJ (2001) Subclinical right ventricular dysfunction in cystic fibrosis: a study using tissue Doppler echocardiography. Am J Respir Crit Care Med 163:1212–1218PubMedGoogle Scholar
  11. 11.
    Labombarda F, Pellissier A, Ellafi M, Creveuil C, Ribault V, Laurans M et al (2011) Myocardial strain assessment in cystic fibrosis. J Am Soc Echocardiogr 24:1037–1045PubMedCrossRefGoogle Scholar
  12. 12.
    Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA et al (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18:1440–1463PubMedCrossRefGoogle Scholar
  13. 13.
    Lester LA, Egge AC, Hubbard VS, Camerini-Otero CS, Fink RJ (1980) Echocardiography in cystic fibrosis: a proposed scoring system. J Pediatr 97:742–748PubMedCrossRefGoogle Scholar
  14. 14.
    Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK et al (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23:465–495 quiz 576–577PubMedCrossRefGoogle Scholar
  15. 15.
    Lopez-Candales A, Rajagopalan N, Gulyasy B, Edelman K, Bazaz R (2009) Differential strain and velocity generation along the right ventricular free wall in pulmonary hypertension. Can J Cardiol 25:e73–e77PubMedCrossRefGoogle Scholar
  16. 16.
    MacNee W (1994) Pathophysiology of cor pulmonale in chronic obstructive pulmonary disease: part one. Am J Respir Crit Care Med 150:833–852PubMedGoogle Scholar
  17. 17.
    Matsui H, Satomi G, Yasukochi S, Kaneko S, Haseyama K (2008) Evaluation of right ventricular contraction by myocardial strain in children using a two-dimensional tissue tracking method. Pediatr Cardiol 29:377–381PubMedCrossRefGoogle Scholar
  18. 18.
    Moiduddin N, Texter KM, Zaidi AN, Hershenson JA, Stefaniak CA, Hayes J et al (2010) Two-dimensional speckle strain and dyssynchrony in single right ventricles versus normal right ventricles. J Am Soc Echocardiogr 23:673–679PubMedCrossRefGoogle Scholar
  19. 19.
    Moskowitz WB, Gewitz MH, Heyman S, Ruddy RM, Scanlin TF (1985) Cardiac involvement in cystic fibrosis: early noninvasive detection and vasodilator therapy. Pediatr Pharmacol New York 5:139–148PubMedGoogle Scholar
  20. 20.
    Moss AJ (1982) The cardiovascular system in cystic fibrosis. Pediatrics 70:728–741PubMedGoogle Scholar
  21. 21.
    Nixon LS, Yung B, Bell SC, Elborn JS, Shale DJ (1998) Circulating immunoreactive interleukin-6 in cystic fibrosis. Am J Respir Crit Care Med 157(6 Pt 1):1764–1769PubMedGoogle Scholar
  22. 22.
    Panidis IP, Ren JF, Holsclaw DS, Kotler MN, Mintz GS, Ross J (1985) Cardiac function in patients with cystic fibrosis: evaluation by two-dimensional and Doppler echocardiography. J Am Coll Cardiol 6:701–706PubMedCrossRefGoogle Scholar
  23. 23.
    Piepsz A, Ham HR, Millet E, Dab I (1987) Determination of right ventricular ejection fraction in children with cystic fibrosis. Pediatr Pulmonol 3:24–28PubMedCrossRefGoogle Scholar
  24. 24.
    Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K et al (2010) 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 23:685–713 quiz 86–88PubMedCrossRefGoogle Scholar
  25. 25.
    Simon MA, Rajagopalan N, Mathier MA, Shroff SG, Pinsky MR, Lopez-Candales A (2009) Tissue Doppler imaging of right ventricular decompensation in pulmonary hypertension. Congest Heart Fail 15:271–276PubMedCrossRefGoogle Scholar
  26. 26.
    Stefani L, Toncelli L, Gianassi M, Manetti P, Di Tante V, Vono MR et al (2007) Two-dimensional tracking and TDI are consistent methods for evaluating myocardial longitudinal peak strain in left and right ventricle basal segments in athletes. Cardiovasc Ultrasound 5:7PubMedCrossRefGoogle Scholar
  27. 27.
    Stefani L, Pedrizzetti G, De Luca A, Mercuri R, Innocenti G, Galanti G (2009) Real-time evaluation of longitudinal peak systolic strain (speckle tracking measurement) in left and right ventricles of athletes. Cardiovasc Ultrasound 7:17PubMedCrossRefGoogle Scholar
  28. 28.
    Tonelli AR, Fernandez-Bussy S, Lodhi S, Akindipe OA, Carrie RD, Hamilton K et al (2010) Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival. J Heart Lung Transplant 29:865–872PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Nazire Ozcelik
    • 1
  • Richard Shell
    • 2
  • Melissa Holtzlander
    • 2
  • Clifford Cua
    • 1
  1. 1.Heart CenterNationwide Children’s HospitalColumbusUSA
  2. 2.Center for Respiratory HealthNationwide Children’s HospitalColumbusUnited States

Personalised recommendations