open access

Vol 86, No 4 (2018)
CASE REPORTS
Published online: 2018-08-15
Submitted: 2018-04-10
Accepted: 2018-08-14
Get Citation

2-year follow-up of Lung transplantation as a treatment of Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease) – a case report

Magdalena Latos, Magdalena Ryba, Elżbieta Lazar, Marek Ochman
DOI: 10.5603/ARM.a2018.0031
·
Pubmed: 30110120
·
Adv Respir Med 2018;86(4):202-204.

open access

Vol 86, No 4 (2018)
CASE REPORTS
Published online: 2018-08-15
Submitted: 2018-04-10
Accepted: 2018-08-14

Abstract

Hemorrhagic telangiectasia (HHT) is a disease of initially mild course - manifesting with recurrent nosebleeds and increased
fatigue. Nevertheless, its progression can deteriorate patient’s health. Solid organ transplantation becomes the only therapeutic
option to save a life.
Case series describes a 19-year-old female patient who was diagnosed with HHT and qualified for lung transplantation. She
met the Curacao criteria for HHT (¾). Her health deteriorated significantly to the point of the referral to Department of Cardiac,
Vascular and Endovascular Surgery and Transplantology. Due to her condition, she was qualified for lung transplantation as one
diagnosed with pulmonary arteriovenous malformations and then transplanted at the age of 17. A direct postoperative period was
complicated by HSV2 infection of the wound. 18 months after the procedure, the patient underwent acute cholangitis. The presence
of portal and systemic fistulas was noted and the final diagnosis of HHT was made. Despite the fact that proper diagnosis
was made posttransplant, it was a good treatment. The patient is currently 2 years after the lung transplantation and feels good.
Lung transplantation is a viable therapeutic option for patients with HHT as there are reports of other patients who have benefited
from lung transplantation after other therapeutic options were exhausted.

Abstract

Hemorrhagic telangiectasia (HHT) is a disease of initially mild course - manifesting with recurrent nosebleeds and increased
fatigue. Nevertheless, its progression can deteriorate patient’s health. Solid organ transplantation becomes the only therapeutic
option to save a life.
Case series describes a 19-year-old female patient who was diagnosed with HHT and qualified for lung transplantation. She
met the Curacao criteria for HHT (¾). Her health deteriorated significantly to the point of the referral to Department of Cardiac,
Vascular and Endovascular Surgery and Transplantology. Due to her condition, she was qualified for lung transplantation as one
diagnosed with pulmonary arteriovenous malformations and then transplanted at the age of 17. A direct postoperative period was
complicated by HSV2 infection of the wound. 18 months after the procedure, the patient underwent acute cholangitis. The presence
of portal and systemic fistulas was noted and the final diagnosis of HHT was made. Despite the fact that proper diagnosis
was made posttransplant, it was a good treatment. The patient is currently 2 years after the lung transplantation and feels good.
Lung transplantation is a viable therapeutic option for patients with HHT as there are reports of other patients who have benefited
from lung transplantation after other therapeutic options were exhausted.

Get Citation

Keywords

lung transplantation, hereditary hemorrhagic telangiectasia, transplantation, arteriovenous malformation

About this article
Title

2-year follow-up of Lung transplantation as a treatment of Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease) – a case report

Journal

Advances in Respiratory Medicine

Issue

Vol 86, No 4 (2018)

Pages

202-204

Published online

2018-08-15

DOI

10.5603/ARM.a2018.0031

Pubmed

30110120

Bibliographic record

Adv Respir Med 2018;86(4):202-204.

Keywords

lung transplantation
hereditary hemorrhagic telangiectasia
transplantation
arteriovenous malformation

Authors

Magdalena Latos
Magdalena Ryba
Elżbieta Lazar
Marek Ochman

References (10)
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  2. Plauchu H, de Chadarévian JP, Bideau A, et al. Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet. 1989; 32(3): 291–297.
  3. Rozenberg D, Leek E, Faughnan ME. Prevalence and nature of dyspnea in patients with hereditary hemorrhagic telangiectasia (HHT). Respir Med. 2015; 109(6): 768–777.
  4. Garcia-Tsao G, Korzenik JR, Young L, et al. Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2000; 343(13): 931–936.
  5. Misra MV, Mullen MP, Vargas SO, et al. Bilateral lung transplant for hereditary hemorrhagic telangiectasia in a pediatric patient. Pediatr Transplant. 2012; 16(8): E364–E367.
  6. Shin JiH, Park SJ, Ko GY, et al. Embolotherapy for pulmonary arteriovenous malformations in patients without hereditary hemorrhagic telangiectasia. Korean J Radiol. 2010; 11(3): 312–319.
  7. Shin J, Park S, Ko GY, et al. Embolotherapy for pulmonary arteriovenous malformations in patients without hereditary hemorrhagic telangiectasia. Kor J Radiol. 2010; 11(3): 312.
  8. Angriman F, Ferreyro BL, Wainstein EJ, et al. Pulmonary arteriovenous malformations and embolic complications in patients with hereditary hemorrhagic telangiectasia. Arch Bronconeumol. 2014; 50(7): 301–304.
  9. Fukushima H, Mitsuhashi T, Oto T, et al. Successful lung transplantation in a case with diffuse pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. Am J Transplant. 2013; 13(12): 3278–3281.
  10. Svetliza G, De la Canal A, Beveraggi E, et al. Lung transplantation in a patient with arteriovenous malformations. J Heart Lung Transplant. 2002; 21(4): 506–508.

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