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Lung transplantation
Long-term outcomes of patients with pulmonary arteriovenous malformations considered for lung transplantation, compared with similarly hypoxaemic cohorts
  1. Claire L Shovlin1,2,
  2. Elisabetta Buscarini3,
  3. J Michael B Hughes4,
  4. David J Allison5 and
  5. James E Jackson6
  1. 1 NHLI Vascular Science, Imperial College London, London, UK
  2. 2 Respiratory Medicine, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK
  3. 3 Gastroenterology Department, and VASCERN HHT European Reference Centre, Maggiore Hospital, ASST Crema, Crema, Italy
  4. 4 NHLI Respiratory Sciences, Imperial College London, London, UK
  5. 5 Department of Imaging, Imperial College Healthcare NHS Trust, London, UK
  6. 6 Department of Imaging, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK
  1. Correspondence to Professor Claire L Shovlin; c.shovlin{at}imperial.ac.uk

Abstract

Introduction Pulmonary arteriovenous malformations (PAVMs) may not be amenable to treatment by embolisation or surgical resection, and many patients are left with significant hypoxaemia. Lung transplantation has been undertaken. There is no guidance on selection criteria.

Methods To guide transplantation listing assessments, the outcomes of the six patients who had been considered for transplantation were compared with a similarly hypoxaemic patient group recruited prospectively between 2005 and 2016 at the same UK institution.

Results Six patients had been formally considered for lung transplantation purely for PAVMs. One underwent a single lung transplantation for diffuse PAVMs and died within 4 weeks of surgery. The other five were not transplanted, in four cases at the patients’ request. Their current survival ranges from 16 to 27 (median 21) years post-transplant assessment.

Of 444 consecutive patients with PAVMs recruited between 2005 and 2016, 42 were similarly hypoxaemic to the ‘transplant-considered’ cohort (SaO2 <86.5%). Hypoxaemic cohorts maintained arterial oxygen content (CaO2) through secondary erythrocytosis and higher haemoglobin. The ‘transplant-considered’ cohort had similar CaO2 to the hypoxaemic comparator group, but higher Medical Research Council (MRC) dyspnoea scores (p=0.023), higher rates of cerebral abscesses (p=0.0043) and higher rates of venous thromboemboli (p=0.0009) that were evident before and after the decision to list for transplantation.

Conclusions The non-transplanted patients demonstrated marked longevity. Symptoms and comorbidities were better predictors of health than oxygen measurements. While a case-by-case decision, weighing survival estimates and quality of life will help patients in their decision making, the data suggest a very strong case must be made before lung transplantation is considered.

  • Lung Transplantation
  • Ambulatory Oxygen Therapy
  • Thoracic Surgery
  • Systemic disease and lungs
  • Rare lung diseases
  • Paediatric Lung Disaese
  • Imaging/CT MRI etc

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bmjresp-2017-000198

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    Footnotes

    • Contributors CLS assessed and treated patients (1999–to date), set up the prospective characterisation series, performed the notes evaluations, statistical evaluations, and figure generation, and wrote the manuscript. EB assessed and treated patients. DJA assessed and treated patients (1984–1999), developed interventional treatment modalities and referred one case for lung transplantation. JMBH assessed and treated patients (1988–1999), developed physiological assessment modalities and referred two cases for lung transplantation. JEJ assessed and treated patients (1985–to date) and developed interventional treatment modalities. All authors contributed to and approved the final version of this manuscript.

    • Funding Funding was received from patient family donations, particularly the Donald Ralph and Thomas Ralph Memorial Fund. Professor Shovlin and Dr Jackson also acknowledge support from the NIHR Imperial Biomedical Research Centre.

    • Competing interests None declared.

    • Ethics approval This study was conducted in accordance with the amended Declaration of Helsinki. The Hammersmith and Queen Charlottes Local Research Ethics Committee (LREC 2000/5764) approved the protocol.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data sharing statement The authors agree with the principles of data sharing: anonymised data will be provided on reasonable request.