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Congenital central pulmonary artery anomalies: Part 2

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Abstract

There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate both well-recognized and more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. In Part 2 the authors review abnormalities in development, origin and course of the central branch pulmonary arteries as well as abnormal pulmonary artery caliber.

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References

  1. Weichert J, Hartge DR, Axt-Fliedner R (2010) The fetal ductus arteriosus and its abnormalities — a review. Congenit Heart Dis 5:398–408

    Article  Google Scholar 

  2. Alvarez SGV, McBrien A (2018) Ductus arteriosus and fetal echocardiography: implications for practice. Semin Fetal Neonatal Med 23:285–291

    Article  Google Scholar 

  3. Yildirim A, Karabulut N, Dogan S, Herek D (2011) Congenital thoracic arterial anomalies in adults: a CT overview. Diagn Interv Radiol 17:352–362

    PubMed  Google Scholar 

  4. Collins LK, Levin TL, Berdon WE et al (2010) Rudhe syndrome: reversible right middle lobe emphysema in infants with left-to-right shunts — an historical review. Pediatr Radiol 40:762–765

    Article  Google Scholar 

  5. Calder L, Van Praagh R, Van Praagh S et al (1976) Truncus arteriosus communis. Clinical, angiocardiographic, and pathologic findings in 100 patients. Am Heart J 92:23–38

    Article  CAS  Google Scholar 

  6. Gatzoulis M, Webb G, Daubeney P (2006) Diagnosis and management of adult congenital heart disease. Churchill Livingston, Edinburgh

    Google Scholar 

  7. Coley B (2018) Caffey’s pediatric diagnostic imaging, 13th edn. Elsevier, Philadelphia

    Google Scholar 

  8. Castaner E, Gallardo X, Rimola J et al (2006) Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics 26:349–371

    Article  Google Scholar 

  9. Davis SD (2000) Case 28: proximal interruption of the right pulmonary artery. Radiology 217:437–440

    Article  CAS  Google Scholar 

  10. Carter BW, Lichtenberger JP 3rd, Wu CC (2014) Congenital abnormalities of the pulmonary arteries in adults. AJR Am J Roentgenol 202:W308–W313

    Article  Google Scholar 

  11. Liu H, Juan YH, Chen J et al (2015) Anomalous origin of one pulmonary artery branch from the aorta: role of MDCT angiography. AJR Am J Roentgenol 204:979–987

    Article  Google Scholar 

  12. Newman B, Cho Y (2010) Left pulmonary artery sling — anatomy and imaging. Semin Ultrasound CT MR 31:158–170

    Article  Google Scholar 

  13. Berdon WE (2000) Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium — the legacy of Robert E. Gross, MD, and Edward B.D. Neuhauser, MD. Radiology 216:624–632

  14. Wells TR, Gwinn JL, Landing BH, Stanley P (1988) Reconsideration of the anatomy of sling left pulmonary artery: the association of one form with bridging bronchus and imperforate anus. Anatomic and diagnostic aspects. J Pediatr Surg 23:892–898

    Article  CAS  Google Scholar 

  15. Chassagnon G, Morel B, Carpentier E et al (2016) Tracheobronchial branching abnormalities: lobe-based classification scheme. Radiographics 36:358–373

    Article  Google Scholar 

  16. Hernandez LE, Anderson RH, Hoggard E et al (2015) Crossed pulmonary arteries with hypoplasia of the transverse aortic arch. Cardiol Young 25:718–724

    Article  Google Scholar 

  17. Allanson JE, Roberts AE (2016) Noonan syndrome. In: Adam MP, Ardinger HH, Pagon RA et al (eds), GeneReviews. University of Washington, Seattle

  18. Morisaki T, Morisaki H (2016) Genetics of hereditary large vessel diseases. J Hum Genet 61:21–26

    Article  CAS  Google Scholar 

  19. Taybi H, Lachman R (2007) Radiology of syndromes, metabolic disorders, and skeletal dysplasias, 5th edn. Mosby, St. Louis

    Google Scholar 

  20. Newman B (2006) Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol 36:773–791

    Article  Google Scholar 

  21. Morris CA, Mervis CB (2000) Williams syndrome and related disorders. Annu Rev Genomics Hum Genet 1:461–484

    Article  CAS  Google Scholar 

  22. Twite MD, Stenquist S, Ing RJ (2019) Williams syndrome. Paediatr Anaesth 29:483–490

    Article  Google Scholar 

  23. Collins RT 2nd, Mainwaring RD, MacMillen KL, Hanley FL (2019) Outcomes of pulmonary artery reconstruction in Williams syndrome. Ann Thorac Surg 108:146–153

    Article  Google Scholar 

  24. Shah S, Ashwath R, Rajiaj P (2016) Imaging cardiovascular manifestations of genetic syndromes. Curr Probl Diagn Radiol 45:51–60

    Article  Google Scholar 

  25. Das KM, Momenah TS, Larsson SG et al (2014) Williams-Beuren syndrome: computed tomography imaging review. Pediatr Cardiol 35:1309–1320

    Article  CAS  Google Scholar 

  26. Turnpenny PD, Ellard S (2012) Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet 20:251–257

    Article  CAS  Google Scholar 

  27. Rodriguez RM, Feinstein JA, Chan FP (2016) CT-defined phenotype of pulmonary artery stenoses in Alagille syndrome. Pediatr Radiol 46:1120–1127

    Article  Google Scholar 

  28. Bezniakow N, Gos M, Obersztyn E (2014) The RASopathies as an example of RAS/MAPK pathway disturbances — clinical presentation and molecular pathogenesis of selected syndromes. Dev Period Med 18:285–296

    PubMed  Google Scholar 

  29. Ley S, Zaporozhan J, Arnold R et al (2007) Preoperative assessment and follow-up of congenital abnormalities of the pulmonary arteries using CT and MRI. Eur Radiol 17:151–162

    Article  Google Scholar 

  30. Grotenhuis HB, Nijveld A, Backx A (2003) Absent pulmonary valve syndrome with intact ventricular septum and patent ductus arteriosus: report of two cases and a short review of the literature. Ann Thorac Surg 75:280–282

    Article  Google Scholar 

  31. Qureshi MY, Burkhart HM, Julsrud P, Cetta F (2014) Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome. Tex Heart Inst J 41:664–667

    Article  Google Scholar 

  32. Yong MS, Yim D, Brizard CP et al (2014) Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience. Ann Thorac Surg 97:1671–1677

    Article  Google Scholar 

  33. Frank L, Dillman JR, Parish V et al (2010) Cardiovascular MR imaging of conotruncal anomalies. Radiographics 30:1069–1094

    Article  Google Scholar 

  34. Sridhar P, Kalyanpur A, Suresh P, Sharma R (2006) Helical CT evaluation of aortopulmonary window. Ind J Radiol Imaging 16:847–849

    Article  Google Scholar 

  35. Hirsig LE, Sharma PG, Verma N, Rajderkar DA (2018) Congenital pulmonary artery anomalies: a review and approach to classification. J Clin Imaging Sci 8:29

    Article  Google Scholar 

  36. Knobel Z, Kellenberger CJ, Kaiser T et al (2011) Geometry and dimensions of the pulmonary artery bifurcation in children and adolescents: assessment in vivo by contrast-enhanced MR-angiography. Int J Card Imaging 27:385–396

    Article  Google Scholar 

  37. Compton GL, Florence J, MacDonald C et al (2015) Main pulmonary artery-to-ascending aorta diameter ratio in healthy children on MDCT. AJR Am J Roentgenol 205:1322–1325

    Article  Google Scholar 

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Newman, B., Alkhori, N. Congenital central pulmonary artery anomalies: Part 2. Pediatr Radiol 50, 1030–1040 (2020). https://doi.org/10.1007/s00247-020-04703-3

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  • DOI: https://doi.org/10.1007/s00247-020-04703-3

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