LETTERS TO THE EDITOR
Reply
TO THE EDITOR:
We thank Rammohan et al. for the comment on our
article. As the authors highlight, the reported rates of
hepatic artery thrombosis (HAT) in propionic acide-
mia (PA) patients who undergo liver transplantation
(LT) are higher than in other indications of LT.(1) As
described in the article, the rate of HAT in our series
was 33% (2/6), which is similar to rates reported by
other groups.(1,2) The overall incidence of HAT in the
last 10 years in our center for non-PA indications was
3.5% (4/113): 3.7% (3/81) in whole liver graft trans-
plantation and 3.1% (1/32) in living donor liver trans-
plantation (LDLT).
In the 2 HATs described in our series of trans-
planted PA patients, no anastomotic stenosis cases
were observed in the interventional radiologist room.
A third patient presented a vasospasm of the hepatic
artery. Although technical issues cannot be completely
ruled out, the higher incidence of HAT observed in
all the reported series requires the assessment of other
physiopathological explanations.
Considering the alterations observed in our patients
in the thromboelastography with normal coagulation
parameters, it could be hypothesized that some propio-
nate metabolites may interfere in the patient’s hemosta-
sis. Although there is not any evidence in the literature
supporting arterial wall compliance abnormalities in PA
patients, we highlight in the text the subjective feeling
Abbreviations: APOLT, auxiliary partial orthotopic liver
transplantation; HAT, hepatic artery thrombosis; LDLT, living
donor liver transplantation; LT, liver transplantation; PA, propionic
acidemia.
Address reprint requests to Jesús Quintero, M.D., Pediatric Hepatology
and Liver Transplant Department, Hospital Universiatri Vall
d’Hebron, Passeig de la Vall d’Hebron 119-129 08035, Barcelona,
Spain. Telephone: 0034-93-48-31-40; FAX: 93-247-61-12;
E-mail: 38633jqb@gmail.com
Received October 23, 2018; accepted October 23, 2018.
Copyright © 2018 by the American Association for the Study of Liver
Diseases.
View this article online at wileyonlinelibrary.com.
DOI 10.1002/lt.25389
Potential conflict of interest: Nothing to report.
178 | Letters to the editor
of the surgical team that the consistency of the arterial
wall was lower than normal. Moreover, in 2 patients,
a vasospasm without thrombosis was detected during
the surgical procedure. One of them was completely
solved using intra-arterial vasodilators, and the other
one finally developed HAT 36 hours after the first pro-
cedure. Taking into account these observations, the use
of continuous venovenous hemodiafiltration to remove
procoagulant metabolites or more aggressive anticoag-
ulant prophylaxis could be assessed in these patients.
In our experience, when the inherent predisposition to
HAT is joined with complex vascular reconstruction, as
is usually seen in LDLT, the rate of thrombosis increases.
Given the high rate of HAT observed, the use of
the arterial conduit directly from the recipient aorta
has been proposed as an alternative in our center. This
approach has been adopted taking into account the
experience in Alagille syndrome, another indication
for LT related to higher rates of HAT.(3)
As stated by Rammohan et al., auxiliary partial
orthotopic liver transplantation (APOLT) can be a
good treatment option for selected noncirrhotic inborn
errors of metabolism. This technique has potential
benefits over the conventional orthotopic liver trans-
plantation (OLT) as part of the native liver is left in
place and can function as a back-up if the donor graft
fails. Moreover, by avoiding the anhepatic phase, the
risk of metabolic decompensations during the surgery
is diminished.(4) Moreover, the possibility of perform-
ing gene therapy in the future is left open. Although
the possibility to perform APOLT in this type of
patients is currently being evaluated at our institution,
other negative aspects must be taken into account.
Mainly, PA patients seem to require a significantly
higher amount of liver volume to avoid metabolic
decompensation compared with patients with other
inborn errors of metabolism. For this reason, APOLT
receptors still have a risk of severe decompensations
which could lead to a severe deterioration of their
quality of life. In our series, no patient has presented
decompensation either during the surgery or during
follow-up, presenting stabilization or improvement in
the neurologic impairment in all patients 1 year after
LT. It will be very important to know the metabolic
evolution of PA patients receptors of APOLT in terms
of toxic metabolites, neurological evolution, and num-
ber/severity of metabolic crises.
Liver Transplantation, Vol. 25, No. 1, 2019
Many aspects remain unresolved in the indication
of the transplant and the perioperative management of
PA patients. The low frequency of the disease and the
heterogeneity in follow-up make it difficult to general-
ize the results. Multicenter studies that involve a meta-
bolic team, pediatric hepatologist, and LT surgeons are
necessary to answer these questions.
Jesús Quintero, M.D.1
Cristina Molera, M.D. 6
Javier Juamperez, M.D.1
Susanna Redecillas, M.D. 2
Silvia Meavilla, M.D. 6
Raquel Nuñez, M.D. 2
Camila García-Volpe, M.D.6
Mireia del Toro, M.D., Ph.D. 3
Ángels Garcia-Cazorla, M.D., Ph.D.7
Juan Ortega, M.D.4
Óscar Segarra, M.D., Ph.D. 2
Javier Martin de Carpi, M.D., Ph.D. 6
Itxarone Bilbao, M.D., Ph.D. 5
Ramon Charco, M.D., Ph.D. 5
1 Pediatric Hepatology and Liver Transplant
Department  Champion M, et al. Auxiliary liver transplantation for propionic
2 Pediatric Gastroenterology, Hepatology, and
Nutrition Unit 
Letters to the Editor
3 Pediatric Neurology Unit 
4 Pediatric Intensive Care Unit 
5 HPB Surgery and Transplants Department 
Hospital Universiatri Vall d’Hebron
Barcelona, Spain
6 Pediatric Gastroenterology Hepatology and
Nutrition Unit 
7 Pediatric Neurology Unit 
Hospital Sant Joan de Déu
Esplugues de Llobregat, Spain
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1) Vara R, Turner C, Mundy H, Heaton ND, Rela M, Mieli-Vergani
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2) Charbit-Henrion F, Lacaille F, McKiernan P, Girard M, de
Lonlay P, Valayannopoulos V, et al. Early and late complications
after liver transplantation for propionic acidemia in children: a
two centers study. Am J Transplant 2015;15:786-791.
3) Kohaut J, Pommier R, Guerin F, Pariente D, Jacquemin E,
Martelli H, Branchereau S. Abdominal arterial anomalies in
children with alagille syndrome: surgical aspects and outcomes
of liver transplantation. J Pediatr Gastroenterol Nutr 2017;64:
888-891.
4) Rela M, Battula N, Madanur M, Mieli-Vergani G, Dhawan A,
acidemia: a 10-year follow-up. Am J Transplant 2007;7:2200-
2203.
LETTERS TO THE EDITOR | 179