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

BMJ Open Respiratory Research
Claire L ShovlinJames E Jackson

Abstract

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. 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. 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 Rese...Continue Reading

Citations

Nov 17, 2017·Thorax·Claire L ShovlinUNKNOWN British Thoracic Society
Jan 19, 2019·QJM : Monthly Journal of the Association of Physicians·F GaweckiC L Shovlin
Feb 6, 2019·Orphanet Journal of Rare Diseases·Elisabetta BuscariniUNKNOWN VASCERN-HHT
Jun 25, 2020·Journal of Clinical Medicine·Shamaita Majumdar, Justin P McWilliams
Apr 4, 2021·International Journal of Molecular Sciences·Sala BofaridMarco C Post

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