Long-term outcomes of patients with pulmonary arteriovenous malformations considered for lung transplantation, compared with similarly hypoxaemic cohorts

Claire L Shovlin; Elisabetta Buscarini; J Michael B Hughes; David J Allison; James E Jackson

doi:10.1136/bmjresp-2017-000198

Long-term outcomes of patients with pulmonary arteriovenous malformations considered for lung transplantation, compared with similarly hypoxaemic cohorts

BMJ Open Respir Res. 2017 Oct 13;4(1):e000198. doi: 10.1136/bmjresp-2017-000198. eCollection 2017.

Authors

Claire L Shovlin^{1

2}, Elisabetta Buscarini³, J Michael B Hughes⁴, David J Allison⁵, James E Jackson⁶

Affiliations

¹ NHLI Vascular Science, Imperial College London, London, UK.
² Respiratory Medicine, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
³ Gastroenterology Department, and VASCERN HHT European Reference Centre, Maggiore Hospital, ASST Crema, Crema, Italy.
⁴ NHLI Respiratory Sciences, Imperial College London, London, UK.
⁵ Department of Imaging, Imperial College Healthcare NHS Trust, London, UK.
⁶ Department of Imaging, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, 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.

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