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

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.

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Study flow chart. The cohort of 330 patients who first presented to our institution between 1984 and 2005 included five individuals considered for lung transplantation, compared with only one from the cohort of 445 who were first assessed between 2005 and 2016. ‡It was not possible to compare the presentation values of the 1984–2005 cohort to the final lung transplant-considered group due to the changes in clinic assessments over the period. In particular, routine dyspnoea and iron (ferritin) assessments changed in 2005, as described in references and . Cohorts A and B were categorised by SaO2: cohort A had resting SaO2 on air of ≥86.5%, cohort B <86.5%.
Figure 2
Figure 2
Dyspnoea and oxygenation parameters. The five cases considered for transplant, as assessed at their most recent follow-up, compared with 29 similarly hypoxaemic cases who were a subgroup of cohort B in whom presentation clinic assessments were rigorously evaluated between 2005 and 2010 (to generate blinded MRC dyspnoea scores as described in references and 61). (A) Modified MRC dyspnoea scale. (B) SaO2 and (C) CaO2. Box plots indicate median (line) and IQR, error bars represent two SD. Note that the comparator population were able to benefit from subsequent treatments. p Values were calculated by Mann-Whitney U test.
Figure 3
Figure 3
Kaplan-Meier comparisons of cerebral abscess risks. (A) The five cases considered for transplant (LTx-considered), compared with the full comparator cohort of 444 patients (cohorts A+B). T=0 represents the start of the follow-up after consideration for transplant (LTx-considered, at median age 21.5 years) or 22 years for the comparator cohort of 444 patients. (B) The same five cases considered for transplant (LTx-considered), compared with the comparably hypoxaemic comparator cohort of 42 patients (cohort B). Again, T=0 represents the start of follow-up after consideration for transplant (LTx-considered, at median age 21.5 years) or 22 years for the comparator cohort of 42 patients.

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