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First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia

Received: 12 May 2021    Accepted: 2 June 2021    Published: 15 June 2021
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Abstract

Cleidocranial dysplasia is a rare, autosomal dominant disease that is associated with clavicular absence or hypoplasia. Permanent pacemakers are most commonly implanted using percutaneous subclavian venous access. The clavicle is typically used as a bony landmark to guide venous access. Transvenous pacemaker implantation in the setting of clavicular hypoplasia, resection or other anomalies has not been described in literature. This is the first case report of a patient with clavicular absence undergoing transvenous permanent pacemaker implantation. This patient has a rare condition called cleidocranial dysplasia resulting in the congenital absence of his clavicles, along with other skeletal abnormalities. Cardiac anomalies are not associated with this disorder. This patient presented for permanent pacemaker placement in the setting of trifascicular block, symptomatic intermittent second-degree Mobitz type II atrioventricular block, and syncope. Using intra-procedural subclavian venography and intraprocedural Sonosite ultrasound imaging to identify vascular anatomy and surrounding anatomic landmarks, this patient underwent successful placement of a dual chamber transvenous pacemaker. Images from the intraprocedural venogram and the post-procedure chest x-ray illustrate the anatomy in this patient with congenital absence of the clavicles. This case has important implications in subclavian access and pacemaker placement in patients with clavicular abnormalities that may include absent, deformed, or resected clavicles.

Published in Cardiology and Cardiovascular Research (Volume 5, Issue 2)
DOI 10.11648/j.ccr.20210502.17
Page(s) 94-96
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Pacemaker, Cleidocranial Dysplasia, Clavicle

References
[1] Cleidocranial dysplasia. Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. Accessed March 28, 2021. https://rarediseases.info.nih.gov/diseases/6118/cleidocranial-dysplasia.
[2] Paul SA, Simon SS, Karthik AK, Chacko RK, Savitha S. (2015) A review of clinical and radiological features of cleidocranial dysplasia with a report of two cases and a dental treatment protocol. Journal of Pharmacy & Bioallied Sciences, 7 (6), 428-432.
[3] Farrow E, Nicot R, Wiss A, Laborde A, Ferri J. (2018) Cleidocranial dysplasia: a review of clinical, radiological, genetic implications and a guidelines proposal. Journal of Cardiofacial Surgery, 29 (2), 382-389.
[4] Kutilek S, Machytka R, Munzar P. (2019) Cleidocranial dysplasia. Sudanese Journal of Paediatrics, 19 (2), 165-168.
[5] Mundlos S. (1999) Cleidocranial dysplasia: clinical and molecular genetics. Journal of Medical Genetics, 36 (3), 177-182.
[6] Azevedo Almeida LC, Faraj de Lima FB, Matushita H, Valenca MM, Ferreira Castro TL, de Mendonca RN. (2020) Cleidocranial dysplasia, a rare skeletal disorder with failure of the cranial closure: case-based update. Child's Nervous System, 36, 2913-2918.
[7] Dhiman NK, Singh AK, Sharma NK, Jaiswara C. (2014) Cleidocranial dysplasia. National Journal of Maxillofacial Surgery, 5 (2), 206-208.
[8] Kusumoto FM, Schoenfeld MH, Barrett C, Edgerton JR, Ellenbogen KA, Gold MR, Goldschlager NF, Hamilton RM, Joglar JA, Kim RJ, Lee R, Marine JE, McLeod CJ, Oken KR, Patton KK, Pellegrini CN, Selzman KA, Thompson A, Varosy PD. (2019) 2018 ACC/AHA/HRS guideline on the evaluation and management of patients with bradycardia and cardiac conduction delay: a report of the American College of Cardiology/American Heart Association task force on clinical practice guidelines and the Heart Rhythm Society. Circulation, 140 (8), e382-e482.
[9] Migliore F, Curnis A, Bertaglia E. (2016) Axillary vein technique for pacemaker and implantable defibrillator leads implantation: a safe and alternative approach? Journal of Cardiovascular Medicine, 17 (4), 309-313.
[10] Sharma G, Boopathy Senguttuvan N, Thachil A, Leong D, Naik N, Yadav R, Juneja R, Bahl VK. (2012) A comparison of lead placement through the subclavian vein technique with fluoroscopy-guided axillary vein technique for permanent pacemaker insertion. Canadian Journal of Cardiology, 28 (5), 542-546.
[11] Parsonnet V, Roelke M. (1999) The cephalic vein cutdown versus subclavian puncture for pacemaker/ICD lead implantation. Pacing and Clinical Electrophysiology, 22 (5), 695-697.
[12] Tjong FVY, Reddy VY. (2017) Permanent leadless cardiac pacemaker therapy: a comprehensive review. Circulation, 135 (15), 1458-1470.
[13] Della Rocca DG, Gianni C, Di Biase L, Natale A, Al-Ahmad A. (2018) Leadless pacemakers: state of the art and future perspectives. Cardiac Electrophysiology Clinics, 10 (1), 17-29.
[14] Albertini CM de Moraes, da Silva KR, Leal Filho JM da Motta, Crevelari ES, Filho MM, Carnevale FC, Costa R. (2018) Usefulness of preoperative venography in patients with cardiac implantable electronic devices submitted to lead replacement or device upgrade procedures. Arquivos Brasileiros de Cardiologia 111 (5), 686-696.
[15] Brass P, Hellmich M, Kolodziej L, Schick G, Smith AF. (2015) Ultrasound guidance versus anatomical landmarks for subclavian or femoral vein catheterization. Cochrane Database of Systematic Reviews, 1 (1), CD011447.
[16] Lotlikar PP, Creanga AG, Singer SR. (2018) Clinical and radiological findings in a severe case of cleidocranial dysplasia. BMJ Case Reports, bcr2018226671.
Cite This Article
  • APA Style

    Krista Diane Niezwaag, Benjamin James Kotur, Andrew David Michaels. (2021). First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia. Cardiology and Cardiovascular Research, 5(2), 94-96. https://doi.org/10.11648/j.ccr.20210502.17

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    ACS Style

    Krista Diane Niezwaag; Benjamin James Kotur; Andrew David Michaels. First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia. Cardiol. Cardiovasc. Res. 2021, 5(2), 94-96. doi: 10.11648/j.ccr.20210502.17

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    AMA Style

    Krista Diane Niezwaag, Benjamin James Kotur, Andrew David Michaels. First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia. Cardiol Cardiovasc Res. 2021;5(2):94-96. doi: 10.11648/j.ccr.20210502.17

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  • @article{10.11648/j.ccr.20210502.17,
      author = {Krista Diane Niezwaag and Benjamin James Kotur and Andrew David Michaels},
      title = {First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia},
      journal = {Cardiology and Cardiovascular Research},
      volume = {5},
      number = {2},
      pages = {94-96},
      doi = {10.11648/j.ccr.20210502.17},
      url = {https://doi.org/10.11648/j.ccr.20210502.17},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ccr.20210502.17},
      abstract = {Cleidocranial dysplasia is a rare, autosomal dominant disease that is associated with clavicular absence or hypoplasia. Permanent pacemakers are most commonly implanted using percutaneous subclavian venous access. The clavicle is typically used as a bony landmark to guide venous access. Transvenous pacemaker implantation in the setting of clavicular hypoplasia, resection or other anomalies has not been described in literature. This is the first case report of a patient with clavicular absence undergoing transvenous permanent pacemaker implantation. This patient has a rare condition called cleidocranial dysplasia resulting in the congenital absence of his clavicles, along with other skeletal abnormalities. Cardiac anomalies are not associated with this disorder. This patient presented for permanent pacemaker placement in the setting of trifascicular block, symptomatic intermittent second-degree Mobitz type II atrioventricular block, and syncope. Using intra-procedural subclavian venography and intraprocedural Sonosite ultrasound imaging to identify vascular anatomy and surrounding anatomic landmarks, this patient underwent successful placement of a dual chamber transvenous pacemaker. Images from the intraprocedural venogram and the post-procedure chest x-ray illustrate the anatomy in this patient with congenital absence of the clavicles. This case has important implications in subclavian access and pacemaker placement in patients with clavicular abnormalities that may include absent, deformed, or resected clavicles.},
     year = {2021}
    }
    

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    T1  - First Case Report of Transvenous Pacemaker Placement in a Patient with Congenital Absence of the Clavicles Due to Cleidocranial Dysplasia
    AU  - Krista Diane Niezwaag
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    AB  - Cleidocranial dysplasia is a rare, autosomal dominant disease that is associated with clavicular absence or hypoplasia. Permanent pacemakers are most commonly implanted using percutaneous subclavian venous access. The clavicle is typically used as a bony landmark to guide venous access. Transvenous pacemaker implantation in the setting of clavicular hypoplasia, resection or other anomalies has not been described in literature. This is the first case report of a patient with clavicular absence undergoing transvenous permanent pacemaker implantation. This patient has a rare condition called cleidocranial dysplasia resulting in the congenital absence of his clavicles, along with other skeletal abnormalities. Cardiac anomalies are not associated with this disorder. This patient presented for permanent pacemaker placement in the setting of trifascicular block, symptomatic intermittent second-degree Mobitz type II atrioventricular block, and syncope. Using intra-procedural subclavian venography and intraprocedural Sonosite ultrasound imaging to identify vascular anatomy and surrounding anatomic landmarks, this patient underwent successful placement of a dual chamber transvenous pacemaker. Images from the intraprocedural venogram and the post-procedure chest x-ray illustrate the anatomy in this patient with congenital absence of the clavicles. This case has important implications in subclavian access and pacemaker placement in patients with clavicular abnormalities that may include absent, deformed, or resected clavicles.
    VL  - 5
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Author Information
  • Department of Cardiology, Idaho College of Osteopathic Medicine, Meridian, Idaho, the United States

  • Department of Cardiology, Saint Alphonsus Medical Center, Nampa, Idaho, the United States

  • Department of Cardiology, Idaho College of Osteopathic Medicine, Meridian, Idaho, the United States

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