INTRODUCTION:
Pulmonary arteriovenous malformations (PAVMs) may not be
amenable to treatment by embolization or surgical resection, and
many patients are left with significant hypoxemia. Lung transplantation has been undertaken
.
T
here 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 to a similarly hypoxemic patient group recruited
prospectively
between
2005
-
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 c
ases at the patients’ request
.
Their current survival
ranges from 16
-
27 (median 21) years post transplant assessment.
Of
444 consecutive patients with PAVMs recruited between 2005
-
2016, 42 were similarly hypoxemic
to the
“
transplant
-
considered
”
coho
rt (
SaO
2
<86.5%).
H
ypoxemic cohorts maintained arterial oxygen
content through secondary erythrocytosis and higher haemoglobin.
Th
e
“transplant
-
considered”
cohort
had
similar CaO
2
to the hypoxemic comparator group,
but
higher MRC dyspnea 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
. Symp
toms and co
-
morbidities 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.