Advertisement

Altered 4-D magnetic resonance imaging flow characteristics in complex congenital aortic arch repair

  • Lajja P. DesaiEmail author
  • Haben Berhane
  • Nazia Husain
  • Joshua D. Robinson
  • Cynthia K. Rigsby
  • Michael Markl
Original Article

Abstract

Background

Interrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown.

Objective

Investigate effect of IAA repairs on aortic hemodynamics, wall shear stress and flow derangements via 4-D flow MRI.

Materials and methods

We retrospectively analyzed age- and gender-matched cohorts (IAA [n=6], BAV alone [n=6], controls [n=6]) undergoing cardiac MRI including 4-D flow. Aortic dimensions were measured from standard MR angiography. We quantified peak systolic velocities, regurgitant fractions and wall shear stress in the ascending aorta (AAo), transverse arch and descending aorta (DAo) from 4-D flow, and we graded helix/vortex flow patterns from 3-D blood flow visualization.

Results

Children and young adults with IAA had a wide range of arch dimensions, peak systolic velocities, regurgitant fractions and flow grades. Peak transverse arch systolic velocities were higher in patients with IAA versus controls (P=0.02). Flow derangements in the AAo were found in patients with IAA (median grade=2, 5/6 patients, P=0.04) and BAV (median grade=3, 5/6 patients, P=0.03) versus controls. Flow derangements in the DAo were only seen in patients with IAA (median grade=1, 5/6 patients, P=0.04), and 5/6 people with IAA had helical flow in head and neck vessels. Wall shear stress was increased in people with IAA along the superior transverse arch and proximal DAo versus controls (P=0.02).

Conclusion

Complex congenital aortic arch repairs can change aortic hemodynamics. Associated cardiac defects can further alter findings. Studies are warranted to investigate clinical implications in larger cohorts.

Keywords

Aorta Children Congenital heart disease Four-dimensional flow Heart Interrupted aortic arch Magnetic resonance imaging 

Notes

Acknowledgments

Funding for this study came from a grant from the National Institutes of Health’s National Center for Advancing Translational Sciences.

Compliance with ethical standards

Conflicts of interest

None

Supplementary material

ESM 1

Three-dimensional MRI pathline videos compare flow patterns in the aortic arch, corresponding to Fig. 2 (MP4 1976 kb)

Three-dimensional MRI pathline videos compare flow patterns in the aortic arch, corresponding to Fig. 2 (MP4 1966 kb)

Three-dimensional MRI pathline videos compare flow patterns in the aortic arch, corresponding to Fig. 2 (MP4 1,534 kb)

ESM 2

Three-dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 2,290 kb)

Three‐dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 1949 kb)

Three‐dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 1976 kb)

Three‐dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 1955 kb)

Three‐dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 1,642 kb)

Three‐dimensional MRI pathline videos for all patients with interrupted aortic arch (IAA), corresponding to Fig. 4, show a wide range of flow derangements (MP4 1959 kb)

References

  1. 1.
    Loffredo CA, Ferencz C, Wilson PD, Lurie IW (2000) Interrupted aortic arch: an epidemiologic study. Teratology 61:368–375CrossRefGoogle Scholar
  2. 2.
    Ezon DS, Penny DJ (2016) Aortic arch and vascular anomalies. In: Allen HD, Shaddy RE, Penny DJ (eds) Moss and Adams’ heart disease in infants, children, and adolescents: Including the fetus and young adult, 9th edn. Lippincott Williams & Wilkins, Philadelphia, pp 858–860Google Scholar
  3. 3.
    Sugimoto A, Ota N, Miyakoshi C et al (2014) Mid to long-term aortic valve related outcomes after conventional repair for patients with interrupted aortic arch or coarctation of the aorta combined with ventricular septal defect: the impact of bicuspid aortic valve. Eur J Cardiothorac Surg 46:952–960CrossRefGoogle Scholar
  4. 4.
    Niaz T, Poterucha JT, Johnson JN et al (2017) Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects. Congenit Heart Dis 12:261–269CrossRefGoogle Scholar
  5. 5.
    Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900CrossRefGoogle Scholar
  6. 6.
    Michelena HI, Khanna AD, Mahoney D et al (2011) Incidence of aortic complications in patients with bicuspid aortic valves. JAMA 306:1104–1112CrossRefGoogle Scholar
  7. 7.
    Roger VL, Go AS, Lloyd-Jones DM et al (2011) Heart disease and stroke statistics — 2011 update: a report from the American Heart Association. Circulation 123:e18–e209CrossRefGoogle Scholar
  8. 8.
    Peyvandi S, Lupo PJ, Garbarini J et al (2013) 22q11.2 deletions in patients with conotruncal defects: data from 1610 consecutive cases. Pediatr Cardiol 34:1687–1694CrossRefGoogle Scholar
  9. 9.
    Alsoufi B, McCracken C, Shashidharan S et al (2017) The impact of 22q11.2 deletion syndrome on surgical repair outcomes of conotruncal cardiac anomalies. Ann Thorac Surg 104:1597–1604CrossRefGoogle Scholar
  10. 10.
    McCrindle BW, Tchervenkov CI, Konstantinov IE et al (2005) Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a congenital heart surgeons society study. J Thorac Cardiovasc Surg 129:343–350CrossRefGoogle Scholar
  11. 11.
    Schreiber C, Eicken A, Vogt M et al (2000) Repair of interrupted aortic arch: results after more than 20 years. Ann Thorac Surg 70:1896–1900CrossRefGoogle Scholar
  12. 12.
    Jonas RA, Quaegebeur JM, Kirklin JW et al (1994) Outcomes in patients with interrupted aortic arch and ventricular septal defect. J Thorac Cardiovasc Surg 107:1099–1109Google Scholar
  13. 13.
    Kostelka M, Walther T, Geerdts I et al (2004) Primary repair for aortic arch obstruction associated with ventricular septal defect. Ann Thorac Surg 78:1989–1993CrossRefGoogle Scholar
  14. 14.
    Oosterhof T, Azakie A, Freedom RM et al (2004) Associated factors and trends in outcomes of interrupted aortic arch. Ann Thorac Surg 78:1696–1702CrossRefGoogle Scholar
  15. 15.
    O’Brien SM, Clarke DR, Jacobs JP et al (2009) An empirically based tool for analyzing mortality associated with congenital heart surgery. J Thorac Cardiovasc Surg 138:1139–1153CrossRefGoogle Scholar
  16. 16.
    Bürk J, Blanke P, Stankovic Z et al (2012) Evaluation of 3D blood flow patterns and wall shear stress in the normal and dilated thoracic aorta using flow-sensitive 4D CMR. J Cardiovasc Magn Reson 14:84CrossRefGoogle Scholar
  17. 17.
    Hope TA, Markl M, Wigstrom L et al (2007) Comparison of flow patterns in ascending aortic aneurysms and volunteers using four-dimensional magnetic resonance velocity mapping. J Magn Reson Imaging 26:1471–1479CrossRefGoogle Scholar
  18. 18.
    Van Ooij P, Markl M, Collins JD et al (2017) Aortic valve stenosis alters expression of regional aortic wall shear stress: new insights from a 4-dimensional flow magnetic resonance imaging study of 571 subjects. J Am Heart Assoc 6Google Scholar
  19. 19.
    Bissell MM, Hess AT, Biasiolli L et al (2013) Aortic dilation in bicuspid aortic valve disease: flow pattern is a major contributor and differs with valve fusion type. Circ Cardiovasc Imaging 6:499–507CrossRefGoogle Scholar
  20. 20.
    Mahadevia R, Barker AJ, Schnell S et al (2014) Bicuspid aortic cusp fusion morphology alters aortic 3D outflow patterns, wall shear stress and expression of aortopathy. Circulation 129:673–682CrossRefGoogle Scholar
  21. 21.
    Allen BD, van Ooij P, Barker AJ et al (2015) Thoracic aorta 3D hemodynamics in pediatric and young adult patients with bicuspid aortic valve. J Magn Reson Imaging 42:954–963CrossRefGoogle Scholar
  22. 22.
    Guzzardi DG, Barker AJ, van Ooij P et al (2015) Valve-related hemodynamics mediate human bicuspid aortopathy: insights from wall shear stress mapping. J Am Coll Cardiol 66:892–900CrossRefGoogle Scholar
  23. 23.
    Hirtler D, Geiger J, Jung B et al (2013) 4-D MRI flow analysis in the course of interrupted aortic arch reveals complex morphology and quantifies amount of collateral blood flow. Pediatr Radiol 43:1037–1040CrossRefGoogle Scholar
  24. 24.
    Dyverfeldt P, Bissell M, Barker AJ et al (2015) 4D flow cardiovascular magnetic resonance consensus statement. J Cardiovasc Magn Reson 17:72CrossRefGoogle Scholar
  25. 25.
    Stankovic Z, Allen B, Garcia J et al (2014) 4D flow imaging with MRI. Cardiovasc Diag Ther 4:173–192Google Scholar
  26. 26.
    Markl M, Harloff A, Bley TA et al (2007) Time-resolved 3D MR velocity mapping at 3T: improved navigator-gated assessment of vascular anatomy and blood flow. J Magn Reson Imaging 25:824–831CrossRefGoogle Scholar
  27. 27.
    Nishimura RA, Otto CM, Bonow RO et al (2014) 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines. Circulation 129:e521–e643Google Scholar
  28. 28.
    Rose MJ, Jarvis K, Chowdhary V et al (2016) Efficient method for volumetric assessment of peak blood flow velocity using 4D flow MRI. J Magn Reson Imaging 44:1673–1682CrossRefGoogle Scholar
  29. 29.
    Schnell S, Smith DA, Barker AJ et al (2016) Altered aortic shape in bicuspid aortic valve relatives influences blood flow patterns. Eur Heart J Cardiovasc Imaging 17:1239–1247CrossRefGoogle Scholar
  30. 30.
    Geiger J, Markl M, Jung B et al (2011) 4D-MR flow analysis in patients after repair for tetralogy of Fallot. Eur Radiol 21:1651–1657CrossRefGoogle Scholar
  31. 31.
    Geiger J, Markl M, Herzer L et al (2012) Aortic flow patterns in patients with Marfan syndrome assessed by flow-sensitive four dimensional MRI. J Magn Reson Imaging 35:594–600CrossRefGoogle Scholar
  32. 32.
    Potters WV, van Ooij P, Marquering H et al (2015) Volumetric arterial wall shear stress calculation based on cine phase contrast MRI. J Magn Reson Imaging 41:505–516CrossRefGoogle Scholar
  33. 33.
    R Core Team (2017) R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna https://www.R-project.org. Accessed 18 July 2019Google Scholar
  34. 34.
    Pohlert T (2018) PMCMRplus: calculate pairwise multiple comparisons of mean rank sums extended. R package version 1.0.0. https://CRAN.R-project.org/package=PMCMRplus. Accessed 18 July 2019
  35. 35.
    Dillman JR, Yarram SG, D’Amico AR, Hernandez RJ (2008) Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol 190:1467–1474CrossRefGoogle Scholar
  36. 36.
    Hoffman JL, Gray RG, LuAnn Minich L et al (2014) Screening for aortic aneurysm after treatment of coarctation. Pediatr Cardiol 35:47–52CrossRefGoogle Scholar
  37. 37.
    Marino BS, Lipkin PH, Newburger JW et al (2012) Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 126:1143–1172CrossRefGoogle Scholar
  38. 38.
    Egbe AC, Padang R, Brown RD et al (2017) Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve. Heart 103:1508–1514CrossRefGoogle Scholar
  39. 39.
    Connolly HM, Huston J 3rd, Brown RD Jr et al (2003) Intracranial aneurysms in patients with coarctation of the aorta: a prospective magnetic resonance angiography study of 100 patients. Mayo Clin Proc 78:1491–1499CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Ann and Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA
  2. 2.Northwestern University Feinberg School of MedicineChicagoUSA

Personalised recommendations