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Anomalies of the Pulmonary Arteries

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Pediatric Cardiology
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Abstract

Anomalies of the pulmonary arteries are frequently seen in association with more complex congenital heart defects such as forms of tetralogy of Fallot, transposition of the great arteries, and common arterial trunk; however, they also occur in isolation. The significance of these anomalies may vary from an incidental finding to life-threatening lesions. For complete discussion of pulmonary artery anomalies that are a component of more complex forms of congenital heart disease, the reader is referred to the relevant chapters in this book. This chapter focuses on isolated pulmonary artery anomalies including peripheral pulmonary artery stenosis, abnormal origin of a pulmonary artery from the aorta, and pulmonary artery sling.

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References

  1. Schwalbe E. Die Morphologie Der Missbidugen Des Menschen Und Der Tiere. In: Jena: Gustav Fisher. 1909. p. 426.

    Google Scholar 

  2. Monge MC, Mainwaring RD, Sheikh AY, et al. Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes. J Thorac Cardiovasc Surg. 2013;145:476–81.

    Article  PubMed  Google Scholar 

  3. Williams JC, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961;24:1311–8.

    Article  CAS  PubMed  Google Scholar 

  4. Beuren AJ, Schulze C, Eberle P, et al. The syndrome of supravalvular aortic stenosis, peripheral pulmonary stenosis, mental retardation and similar facial appearance. Am J Cardiol. 1964;13:471–83.

    Article  CAS  PubMed  Google Scholar 

  5. McElhinney DB, Krantz ID, Bason L, et al. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with JAG1 mutatoin and/or Alagille syndrome. Circulation. 2002;106:2567–74.

    Article  PubMed  Google Scholar 

  6. Collins RT. Cardiovascular disease in Williams syndrome. Curr Opin Pediatr. 2018;30:609–15.

    Article  PubMed  Google Scholar 

  7. Collins RT, Kaplan P, Somes GW, Rome JJ. Long-term outcomes of patients with cardiovascular abnormalities and Williams syndrome. J Pediatr. 2010;156:253–258.e1.

    Article  PubMed  Google Scholar 

  8. Collins RT. Cardiovascular disease in Williams syndrome. Circulation. 2013;127:2125–34.

    Article  PubMed  Google Scholar 

  9. Oda T, Elkahloun AG, Pike BL, et al. Mutations in the human Jagged1 gene are responsible for Alagille syndrome. Nat Genet. 1997;16:235–42.

    Article  CAS  PubMed  Google Scholar 

  10. McDaniell R, Warthen DM, Sanchez-Lara PA, et al. NOTCH2 mutations cause Alagille syndrome, a heterogeneous disorder of the notch signaling pathway. Am J Hum Genet. 2006;79:169–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Kamath BM, Bauer RC, Loomes KM, et al. NOTCH2 mutations in Alagille syndrome. J Med Genet. 2012;49:138–44.

    Article  CAS  PubMed  Google Scholar 

  12. Qureshi AM, Gowda ST, Justino H, Spicer DE, Anderson RH. Other malformations of the ventricular outflow tracts. In: Wernovsky G, editor. Anderson’s pediatric cardiology. Philadelphia: Elsevier; 2019. p. 945–62.

    Google Scholar 

  13. van Son JA, Edwards WD, Danielson GK. Pathology of coronary arteries, myocardium, and great arteries in supravavular aortic stenosis. Report of five cases with implications for surgical treatment. J Thorac Cardiovasc Surg. 1994;108:21–8.

    Article  PubMed  Google Scholar 

  14. Manderfield LJ, High FA, Engleka KA, et al. Notch activation of Jagged1 contributes to the assembly of the arterial wall. Circulation. 2012;125:314–23.

    Article  PubMed  Google Scholar 

  15. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019;53:1891916.

    Article  Google Scholar 

  17. Simon MA, Pinsky MR. Right ventricular dysfunction and failure in chronic pressure overload. Cardiol Res Pract. 2011;2011:568095.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Wren C, Oslizok P, Bull C. Natural history of supravalvar aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol. 1990;15:1625–30.

    Article  CAS  PubMed  Google Scholar 

  19. Mainwaring RD, Collins RT, Macmillen KL, et al. Pulmonary artery reconstruction after failed pulmonary artery stents. Ann Thorac Surg. 2020;110:949–65.

    Article  PubMed  Google Scholar 

  20. Raaijmakers R, Noordam C, Noonan JA, et al. Are ECG abnormalities in Noonan syndrome characteristic for the syndrome? Eur J Pediatr. 2008;167:1363–7.

    Article  CAS  PubMed  Google Scholar 

  21. Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with X-ray angiography. Circulation. 2002;106:473–8.

    Article  PubMed  Google Scholar 

  22. Kang SL, Armstrong A, Krings G. Three-dimensional rotational angiography in congenital heart disease: present status and evolving future. Congenit Heart Dis. 2019;14:1046–57.

    Article  PubMed  Google Scholar 

  23. Kim YM, Yoo SJ, Choi JY, et al. Natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis in Williams syndrome. Cardiol Young. 1999;9:37–41.

    Article  CAS  PubMed  Google Scholar 

  24. Kushner T, Halperin JL, Nair AP, et al. Peripheral pulmonary artery stenosis masquerading as pulmonary hypertension: a diagnostic and therapeutic challenge. Vasc Med. 2012;17:235–8.

    Article  PubMed  Google Scholar 

  25. Trivedi KR, Benson LN. Interventional strategies in the management of peripheral pulmonary artery stenosis. J Interv Cardiol. 2003;16:171–88.

    Article  PubMed  Google Scholar 

  26. Geggel RL, Gauvreau K, Lock JE. Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome. Circulation. 2001;103:2165–70.

    Article  CAS  PubMed  Google Scholar 

  27. Cunningham JW, McElhinney DB, Gauvreau K, et al. Outcomes after primary transcatheter therapy in infants and young children with severe bilateral peripheral pulmonary artery stenosis. Circ Cardiovasc Interv. 2013;6:460–7.

    Article  PubMed  Google Scholar 

  28. Rhodes JF, Lane GK, Mesia CI, et al. Cutting balloon angioplasty for children with small-vessel pulmonary artery stenosis. Catheter Cardiovasc Interv. 2002;55:73–7.

    Article  PubMed  Google Scholar 

  29. Bergersen LJ, Perry SB, Lock JE. Effect of cutting balloon angioplasty on resistant pulmonary artery stenosis. Am J Cardiol. 2003;91:185–9.

    Article  PubMed  Google Scholar 

  30. Bergersen L, Jenkins KJ, Gavreau K, et al. Follow-up results of cutting balloon angioplasty used to relieve stenoses in small pulmonary arteries. Cardiol Young. 2005;15:605–10.

    Article  PubMed  Google Scholar 

  31. Bergersen L, Gauvreau K, Justino H, et al. Randomized trial of cutting balloon compared with high-pressure angioplasty for the treatment of resistant pulmonary artery stenosis. Circulation. 2011;124:2388–96.

    Article  PubMed  Google Scholar 

  32. Halzer RJ, Gauvreau K, Kreutzer J, et al. Balloon angioplasty and stenting of branch pulmonary arteries: adverse events and procedural characteristics: results of a multi-institutional registry. Circ Cardiovasc Interv. 2011;4:287–96.

    Article  Google Scholar 

  33. Mainwaring RD, Hanley FL. Surgical techniques for repair of peripheral pulmonary artery stenosis. Semin Thorac Surg. 2017;29:198–205.

    Google Scholar 

  34. Martin E, Mainwaring RD, Collins RT, et al. Surgical repair of peripheral pulmonary artery stenosis in patients without Williams or Alagille syndromes. Semin Thorac Cardiovasc Surg. 2020;32(4):973–9.

    Article  PubMed  Google Scholar 

  35. Knutsen R, Beeman SC, Broekelmann TJ, et al. Minoxidil improves vascular compliance, restores cerebral blood flow and laters extracellular matrix gene expression in a model of chronic vascular stiffness. Am J Physiol Heart Circ Physiol. 2018;315:H18–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Fraentzel O. Ein fall von abnormer communicatoin der aorta mit der arteria pulmonalis. Virchows Arch Pathol Anat. 1868;43:420.

    Article  Google Scholar 

  37. Keane JF, Maltz D, Bernhard WF, et al. Anomalous origin of one pulmonary artery from the ascending aorta. Diagnostic, physiological and surgical considerations. Circulation. 1974;50(3):588–94.

    Article  CAS  PubMed  Google Scholar 

  38. Garg P, Talwar S, Kothari SS, et al. The anomalous origin of the branch pulmonary artery from the ascending aorta. Interact Cardovasc Thorac Surg. 2012;15:86–92.

    Article  Google Scholar 

  39. Patel RJ, Zakir RM, Sethi V, et al. Unrepaired tetralogy of Fallot with right hemitruncus in an adult: a rare case. Tex Heart Inst J. 2007;34:250–1.

    PubMed  PubMed Central  Google Scholar 

  40. Liu H, Juan YH, Chen J, et al. Anomalous origin of one pulmonary artery branch from the aorta: role of MDCT angiography. Am J Roentgenol. 2015;204:979–87.

    Article  Google Scholar 

  41. Fontana GP, Spach MS, Effmann EL, Sabiston DC. Origin of the right pulmonary artery for the ascending aorta. Ann Surg. 1987;206:102–13.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Goldstein BH, Bergersen L, Powell AJ, et al. Long-term outcome of surgically repaired unilateral anomalous pulmonary artery origin. Pediatr Cardiol. 2010;31:944–51.

    Article  PubMed  Google Scholar 

  43. Kutsche L, Van Mierop LH. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol. 1988;61:850–6.

    Article  CAS  PubMed  Google Scholar 

  44. Benamer H, Sluysmans T, Sidi D, et al. Right pulmonary artery arising from the proximal ascending aorta. A model of reflex pulmonary hypertension of the left lung? Arch Mal Coeur Vaiss. 1991;84:639–46.

    CAS  PubMed  Google Scholar 

  45. Yamaki S, Suzuki Y, Ishizawa E, et al. Isolated aortic origin of right pulmonary artery: report of a case with special reference to pulmonary vascular disease in the left and right lungs. Chest. 1983;83:575–8.

    Article  CAS  PubMed  Google Scholar 

  46. Zeng S, Zhou Q, Zhou J, Peng Q. Fetal isolated anomalous origin of right pulmonary artery from aorta. Am J Perinatol Rep. 2015;5:e80–1.

    Article  Google Scholar 

  47. Ma J, Zhang Y, Wang Y, Zhao L. Prenatal two- and three-dimensional echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta: case report and literature review. J Clin Ultrasound. 2020;48:423–7.

    Article  PubMed  Google Scholar 

  48. Kirkpatrick SE, Girod DA, King H. Aortic origin of the right pulmonary artery: surgical repair without a graft. Circulation. 1967;36:777–82.

    Article  CAS  PubMed  Google Scholar 

  49. Garg P, Talwar S, Kothari S, et al. The anomalous origin of the branch pulmonary artery from the ascending aorta. Interact Cardiovasc Thorac Surg. 2012;15:86–92.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Abu-Sulaiman RM, Hashmi A, McCrindle BW, et al. Anomalous origin of one pulmonary artery from the ascending aorta: 36 years’ experience from one centre. Cardiol Young. 1998;8:449–54.

    Article  CAS  PubMed  Google Scholar 

  51. Dong S, Yan J, Xu H, et al. The surgical treatment of anomalous origin of one pulmonary artery from the ascending aorta. J Cardiothorac Surg. 2019;14:82.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Glaevecke H, Doehle W. Ueber eine seltene angeborene anomalie der pulmonalarterie. Munch Med Wochenschr. 1897;44:950–3.

    Google Scholar 

  53. Xie J, Juan Y, Wang Q, et al. Evaluation of left pulmonary artery sling, associated cardiovascular anomalies, and surgical outcomes using cardiovascular computed tomography angiography. Sci Rep. 2017;7:40042.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Yu JM, Liao CP, Ge S, et al. The prevalence and clinical impact of pulmonary artery sling on school-aged children: a large-scale screening study. Pediatr Pulmonol. 2008;43:656–61.

    Article  PubMed  Google Scholar 

  55. Berdon WE, Baker DH, Wung JT, et al. Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex. Radiology. 1984;152:57–64.

    Article  CAS  PubMed  Google Scholar 

  56. Gikonyo BM, Jue KI, Edwards JE. Pulmonary vascular sling: report of seven cases and review of the literature. Pediatr Cardiol. 1989;10:81–9.

    Article  CAS  PubMed  Google Scholar 

  57. Backer CL, Mavroudis C, Gerber ME, Holinger LD. Tracheal surgery in children: an 18-year review of four techniques. Eur J Cardiothorac Surg. 2001;19:777–84.

    Article  CAS  PubMed  Google Scholar 

  58. Fiore AC, Brown JW, Weber TR, Turrentine MW. Surgical treatment of pulmonary artery sling and tracheal stenosis. Ann Thorac Surg. 2005;79:38–46.

    Article  PubMed  Google Scholar 

  59. Backer CL, Russell HM, Kaushal S, et al. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2012;143:144–51.

    Article  PubMed  Google Scholar 

  60. Moreno F, Garcia-Guereta L, Benito F, et al. Aberrant left pulmonary artery with tracheal stenosis without vascular sling. Pediatr Cardiol. 1991;12:44–5.

    Article  CAS  PubMed  Google Scholar 

  61. Pu WT, Chung T, Hoffer FA, et al. Diagnosis and management of agenesis of the right lung and left pulmonary artery sling. Am J Cardiol. 1996;78:723–7.

    Article  CAS  PubMed  Google Scholar 

  62. Wells TR, Gwinn JL, Landing BH, Stanley P. Reconsideration of the anatomy of sling left pulmonary artery: the association of one form with bridging bronchus and imperforate anus. J Pediatr Surg. 1988;23:892–8.

    Article  CAS  PubMed  Google Scholar 

  63. Cohen SR, Landing BH. Tracheal stenosis and bronchial abnormalities associated with pulmonary artery sling. Ann Otol Rhinol Laryngol. 1976;85:582–90.

    Article  CAS  PubMed  Google Scholar 

  64. Manning PB, Rutter MJ, Lisec A, et al. One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children. J Thorac Cardiovasc Surg. 2011;141:155–61.

    Article  PubMed  Google Scholar 

  65. Ishii Y, Miyamoto T, Nakajima K, et al. Abnormal cardiac axis as a prenatal marker of left pulmonary artery sling. Pediatr Int. 2016;58:158–61.

    Article  PubMed  Google Scholar 

  66. Yorioka H, Kasamatsu A, Kanzaki H. Prenatal diagnosis of fetal left pulmonary artery sling. Ultrasound Obstet Gynecol. 2011;37:245–6.

    Article  CAS  PubMed  Google Scholar 

  67. Speggiorin S, Durairaj S, Mimic B, Corno AF. Modeling of airways in congenital heart defects. Front Pediatr. 2016;4:116.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Harada A, Shimojima N, Shimotakahara A, et al. Surgical indication for congenital tracheal stenosis complicated by pulmonary artery sling. J Thorac Dis. 2019;11:5474–9.

    Article  PubMed  PubMed Central  Google Scholar 

  69. Potts WJ, Holinger PH, Rosenblum AH. Anomalous left pulmonary artery causing obstruction to right main bronchus: report of a case. J Am Med Assoc. 1954;155:1409–11.

    Article  CAS  PubMed  Google Scholar 

  70. Yong MS, Zhu MZ, Bell D, et al. Long-term outcomes of surgery for pulmonary artery sling in children. Eur J Cardiothorac Surg. 2019;56:369–76.

    Google Scholar 

  71. Huang SC, Wu ET, Wang CC, et al. Surgical management of pulmonary artery sling: trachea diameter and outcomes with or without tracheoplasty. Pediatr Pulmonol. 2012;47:903–8.

    Article  PubMed  Google Scholar 

  72. Hofferberth SC, Watters K, Rahbar R, et al. Evolution of surgical approaches in the management of congenital tracheal stenosis: single-center experience. World J Pediatr Congenit Heart Surg. 2016;7:16–24.

    Article  PubMed  Google Scholar 

  73. Sade RM, Rosenthal A, Fellows K, Castaneda AR. Pulmonary artery sling. J Thorac Cardiovasc Surg. 1975;69:333–46.

    Article  CAS  PubMed  Google Scholar 

  74. Yong MS, d’Udekem Y, Brizard CP, et al. Surgical management of pulmonary artery sling in children. J Thorac Cardiovasc Surg. 2013;145:1033–9.

    Article  PubMed  Google Scholar 

  75. Yong MS, d’Udekem Y, Robertson CF, et al. Tracheal repair in children: reduction of mortality with advent of slide tracheoplasty. ANZ J Surg. 2014;84:748–54.

    Article  PubMed  Google Scholar 

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Correspondence to Craig E. Fleishman .

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Fleishman, C.E. (2023). Anomalies of the Pulmonary Arteries. In: Abdulla, Ri., et al. Pediatric Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-030-42937-9_61-1

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  • DOI: https://doi.org/10.1007/978-3-030-42937-9_61-1

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