European Radiology

, Volume 16, Issue 10, pp 2147–2152 | Cite as

Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis—initial results

  • Monika EichingerEmail author
  • Michael Puderbach
  • Christian Fink
  • Julie Gahr
  • Sebastian Ley
  • Christian Plathow
  • Siegfried Tuengerthal
  • Ivan Zuna
  • Frank-Michael Müller
  • Hans-Ulrich Kauczor


This paper is a feasibility study of magnetic resonance imaging (MRI) of lung perfusion in children with cystic fibrosis (CF) using contrast-enhanced 3D MRI. Correlation assessment of perfusion changes with structural abnormalities. Eleven CF patients (9 f, 2 m; median age 16 years) were examined at 1.5 T. Morphology: HASTE coronal, transversal (TR/TE/α/ST: 600 ms/28 ms/180°/6 mm), breath-hold 18 s. Perfusion: Time-resolved 3D GRE pulse sequence (FLASH, TE/TR/α: 0.8/1.9 ms/40°), parallel imaging (GRAPPA, PAT 2). Twenty-five data sets were acquired after intravenous injection of 0.1 mmol/kg body weight of gadodiamide, 3–5 ml/s. A total of 198 lung segments were analyzed by two radiologists in consensus and scored for morphological and perfusion changes. Statistical analysis was performed by Mantel-Haenszel chi-square test. Results showed that perfusion defects were observed in all patients and present in 80% of upper, and 39% of lower lobes. Normal lung parenchyma showed homogeneous perfusion (86%, P<0.0001). Severe morphological changes led to perfusion defects (97%, P<0.0001). Segments with moderate morphological changes showed normal (53%) or impaired perfusion (47%). In conclusion, pulmonary perfusion is easy to judge in segments with normal parenchyma or severe changes. In moderately damaged segments, MRI of lung perfusion may help to better assess actual functional impairment. Contrast-enhanced 3D MRI of lung perfusion has the potential for early vascular functional assessment and therapy control in CF patients.


Cystic fibrosis MRI Lung perfusion Lung morphology 



This study was supported by Forschungsgemeinschaft Mukoviszidose (Mukoviszidose e.V.): S06/04.


  1. 1.
    Davis PB, Drumm M, Konstan MW (1996) Cystic fibrosis. Am J Respir Crit Care Med 154(5):1229–1256PubMedGoogle Scholar
  2. 2.
    Gibson RL, Burns JL, Ramsey BW (2003) Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 168(8):918–951PubMedCrossRefGoogle Scholar
  3. 3.
    Kerem E, Reisman J, Corey M, Canny GJ, Levison H (1992) Prediction of mortality in patients with cystic fibrosis. N Engl J Med 326(18):1187–1191PubMedCrossRefGoogle Scholar
  4. 4.
    v. Euler US, Liljestrand G (1947) Observations on the pulmonary arterial blood pressure in the cat. Acta Phys Scand 12:301–320CrossRefGoogle Scholar
  5. 5.
    Fazio F, Wollmer P (1981) Clinical ventilation-perfusion scintigraphy. Clin Physiol 1(4):323–337PubMedCrossRefGoogle Scholar
  6. 6.
    Fauroux B, Itti E, Pigeot J, Isabey D, Meignan M, Ferry G, Lofaso F, Willemot JM, Clement A, Harf A (2000) Optimization of aerosol deposition by pressure support in children with cystic fibrosis: an experimental and clinical study. Am J Respir Crit Care Med 162(6):2265–2271PubMedGoogle Scholar
  7. 7.
    Itti E, Fauroux B, Pigeot J, Isabey D, Clement A, Evangelista E, Harf A, Meignan M (2004) Quantitative lung perfusion scan as a predictor of aerosol distribution heterogeneity and disease severity in children with cystic fibrosis. Nucl Med Commun 25(6):563–569PubMedCrossRefGoogle Scholar
  8. 8.
    Hatabu H, Gaa J, Kim D, Li W, Prasad PV, Edelman RR (1996) Pulmonary perfusion: qualitative assessment with dynamic contrast-enhanced MRI using ultra-short TE and inversion recovery turbo FLASH. Magn Reson Med 36(4):503–508PubMedCrossRefGoogle Scholar
  9. 9.
    Hatabu H, Tadamura E, Levin DL, Chen Q, Li W, Kim D, Prasad PV, Edelman RR (1999) Quantitative assessment of pulmonary perfusion with dynamic contrast-enhanced MRI. Magn Reson Med 42(6):1033–1038PubMedCrossRefGoogle Scholar
  10. 10.
    Uematsu H, Levin DL, Hatabu H (2001) Quantification of pulmonary perfusion with MR imaging: recent advances. Eur J Radiol 37(3):155–163PubMedCrossRefGoogle Scholar
  11. 11.
    Fink C, Puderbach M, Bock M, Lodemann KP, Zuna I, Schmahl A, Delorme S, Kauczor HU (2004) Regional lung perfusion: assessment with partially parallel three-dimensional MR imaging. Radiology 231(1):175–184PubMedCrossRefGoogle Scholar
  12. 12.
    Puderbach M, Eichinger M, Ley S, Fink C, Plathow C, Gahr J, Wiebel M, Tuengerthal S, Schmahl A, Mueller FM, Kauczor HU (2004) Visualisation of parenchymal lung changes in patients with cystic fibrosis (CF) - MRI versus HRCT. J Cyst Fibros 3(Suppl 1):54Google Scholar
  13. 13.
    Stern M, Sens B, Wiedermann B, Busse O, Damm G, Wenzlaff P (2005) Qualitätssicherung Mukoviszidose, Überblick über den Gesundheitszustand der Patienten in Deutschland 2004. Verlag Wissenschaftliche Scripten, ZwickauGoogle Scholar
  14. 14.
    Tiddens HA (2002) Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol 34(3):228–231PubMedCrossRefGoogle Scholar
  15. 15.
    Lindstrom M, Camner P, Falk R, Hjelte L, Philipson K, Svartengren M (2005) Long-term clearance from small airways in patients with cystic fibrosis. Eur Respir J 25(2):317–323PubMedCrossRefGoogle Scholar
  16. 16.
    Barnes PJ (2004) Small airways in COPD. N Engl J Med 350(26):2635–2637PubMedCrossRefGoogle Scholar
  17. 17.
    Fink C, Bock M, Puderbach M, Schmahl A, Delorme S (2003) Partially parallel three-dimensional magnetic resonance imaging for the assessment of lung perfusion-initial results. Invest Radiol 38(8):482–488PubMedCrossRefGoogle Scholar
  18. 18.
    Ley S, Fink C, Puderbach M, Plathow C, Risse F, Kreitner KF, Kauczor HU (2004) Contrast-enhanced 3D MR perfusion of the lung: application of parallel imaging technique in healthy subjects. Rofo 176(3):330–334PubMedGoogle Scholar
  19. 19.
    Rosenfeld M, Gibson RL, McNamara S, Emerson J, Burns JL, Castile R, Hiatt P, McCoy K, Wilson CB, Inglis A, Smith A, Martin TR, Ramsey BW (2001) Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol 32(5):356–366PubMedCrossRefGoogle Scholar
  20. 20.
    Fink C, Ley S, Risse F, Eichinger M, Zaporozhan J, Buhmann R, Puderbach M, Plathow C, Kauczor HU (2005) Effect of inspiratory and expiratory breathhold on pulmonary perfusion: assessment by pulmonary perfusion magnetic resonance imaging. Invest Radiol 40(2):72–79PubMedCrossRefGoogle Scholar
  21. 21.
    Chrispin AR, Norman AP (1974) The systematic evaluation of the chest radiograph in cystic fibrosis. Pediatr Radiol 2:101–106PubMedCrossRefGoogle Scholar
  22. 22.
    Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, Naidich DP (1991) Cystic fibrosis: scoring system with thin-section CT. Radiology 179(3):783–788PubMedGoogle Scholar
  23. 23.
    Helbich TH, Heinz-Peer G, Eichler I, Wunderbaldinger P, Gotz M, Wojnarowski C, Brasch RC, Herold CJ (1999) Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 213(2):537–544PubMedGoogle Scholar
  24. 24.
    Abolmaali N, Schmidt H, Anjorin A, Posselt H-G, Vogl TJ (2002) Chrispin-Norman-score and Bhalla-score of patients with cystic fibrosis: Comparative study of chest radiographs and MR-Imaging. Eur Radiol 12 [Congress Suppl]:227Google Scholar
  25. 25.
    Vonk-Noordegraaf A, van Wolferen SA, Marcus JT, Boonstra A, Postmus PE, Peeters JW, Peacock AJ (2005) Noninvasive assessment and monitoring of the pulmonary circulation. Eur Respir J 25(4):758–766PubMedCrossRefGoogle Scholar
  26. 26.
    Ley S, Puderbach M, Fink C, Eichinger M, Plathow C, Teiner S, Wiebel M, Muller FM, Kauczor HU (2005) Assessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRI. Eur Radiol 15(8):1575–1580PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Monika Eichinger
    • 1
    Email author
  • Michael Puderbach
    • 1
  • Christian Fink
    • 2
  • Julie Gahr
    • 3
  • Sebastian Ley
    • 1
    • 4
  • Christian Plathow
    • 5
  • Siegfried Tuengerthal
    • 6
  • Ivan Zuna
    • 1
  • Frank-Michael Müller
    • 3
  • Hans-Ulrich Kauczor
    • 1
  1. 1.Department of Radiology (E010)Deutsches Krebsforschungszentrum (DKFZ)HeidelbergGermany
  2. 2.Department of RadiologyInstitut für Klinische Radiologie, Klinikum der LMU GroßhadernMünchenGermany
  3. 3.Department of Pediatric Pulmonology, Cystic Fibrosis Centre and Infectious DiseasesUniversitätskinderklinik III HeidelbergHeidelbergGermany
  4. 4.Department of Pediatric RadiologyUniversitätskinderklinik HeidelbergHeidelbergGermany
  5. 5.Department of Diagnostic RadiologyEberhard-Karls UniversityTuebingenGermany
  6. 6.Department of RadiologyThoraxklinik am Universitätsklinikum HeidelbergHeidelbergGermany

Personalised recommendations