Introduction and objectives Pulmonary arteriovenous malformations (PAVMs) are abnormal vessels that provide anatomic right-to-left shunts, and frequently result in severe hypoxaemia. Lung transplantation is sometimes considered if PAVMs are not amenable to treatment by embolization or surgical resection.
Methods A retrospective review was performed of patients with PAVMs assessed between 1999 and 2016 at a single UK institution. Characteristics of those considered and referred for lung transplantation assessment were examined.
Results Between May 1999 and July 2016, 707 patients with PAVMs were reviewed. Six were or had been formally considered for lung transplantation purely for PAVMs, 4 as adults (3 at our institution between 1989–1995), 2 as children. Ages ranged from 2–47 (median 22) years. Four were from the UK, two had been assessed in non UK countries. All had hereditary haemorrhagic telangiectasia (HHT). Three had suffered a cerebral abscess due to their PAVMs. The adults had undergone maximal embolisation of PAVMs, and the children were considered to have untreatable disease. The median SaO2 was 79% on air.
One adult underwent lung transplantation overseas and died peri-operatively. The other five were not transplanted, in four cases at the patients’ request. Currently, their survival post assessment for lung transplant ranges from 15 to 27 (median 22) years, with four of the five still alive.
All five maintained arterial oxygen content by marked secondary erythrocytotic responses when not iron deficient. Four had subsequent embolisation treatments. One had three successful pregnancies, and another is working full time with no supplementary oxygen. However, two had a cerebral abscess (one fatal); three had deep vein thromboses; two experienced long term disabling pain attributed to hypertrophic pulmonary osteoarthropathy in one; and three have exercise limitation requiring supplementary oxygen. Antiplatelet therapy was recommended for two due to ischaemic strokes; one remains anticoagulated 16 years after the insertion of a caval filter; and one is regularly evaluated for hyperviscosity symptoms that would precipitate isovolaemic venesection.
Conclusion Very long term stability is evident in the five patients who did not receive a lung transplant. The cohort remain at high risk of cerebral abscess and other complications of right-to-left shunting.
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