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Living-donor liver transplantation for moderate or severe porto-pulmonary hypertension accompanied by pulmonary arterial hypertension: a single-centre experience over 2 decades in Japan

  • Original article
  • Published:
Journal of Hepato-Biliary-Pancreatic Sciences

Abstract

Background

Candidates for orthotopic liver transplantation (OLT) often have porto-pulmonary hypertension (PPHTN) with pulmonary arterial hypertension (PAH). Poor outcomes of PPHTN contraindicate OLT. There are no guidelines for living-donor liver transplantation (LDLT) in PPHTN patients.

Methods

We present our experiences of LDLT in six patients with moderate or severe PPHTN, along with our institutional guidelines. Three had liver cirrhosis and three were non-cirrhotic. Catheterization studies were undertaken before, during and after LDLT, and the mean pulmonary arterial pressure (mPAP), cardiac output (CO), pulmonary vascular resistance and total peripheral resistance (TPR) were monitored.

Results

The results showed significant differences in CO and TPR between cirrhotic and non-cirrhotic patients before, during and after LDLT. Cirrhotic patients showed systemic hyperdynamic state. Two cirrhotic patients showed poor responses to pre-transplant treatment, and continued to have increased PAH and poor clinical courses after LDLT. LDLT has an advantage of flexible timing of LT. Currently in our institution, PPHTN patients with mPAP <40 mmHg are registered for LDLT after treatment and catheterization. However, LDLT is performed when mPAP is ≤35 mmHg, leading to improved outcomes.

Conclusion

PPHTN patients with well-controlled PAH, or secondary PAH resulting from porto-systemic shunts, may be appropriate candidates for LDLT after careful considerations.

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Abbreviations

ACR:

Acute cellular rejection

AM:

Abernethy malformation

AVCT:

Acute volume challenge test

BSA:

Body surface area

BV:

Blood volume

CBA:

Congenital biliary atresia

CI:

Cardiac index

CO:

Cardiac output

DDLT:

Deceased-donor liver transplantation

ET-1:

Endothelin-1

GRWR:

Graft-to-recipient weight ratio

HPS:

Hepato-pulmonary syndrome

ICU:

Intensive care unit

LDLT:

Living-donor liver transplantation

LNB:

Liver needle biopsy

NO:

Nitric oxide

OLT:

Orthotopic liver transplantation

MELD:

Model for end-stage liver disease

mAP:

Mean arterial pressure

mPAP:

Mean pulmonary arterial pressure

PELD:

Pediatric end-stage liver disease

PAH:

Pulmonary arterial hypertension

PCWP:

Pulmonary capillary wedge pressure

PGI2 :

Prostaglandin I2

POD:

Postoperative day

PPHTN:

Porto-pulmonary hypertension

PVR:

Pulmonary vascular resistance

RV:

Right ventricle

SPT:

Steroid pulse therapy

TPR:

Total peripheral resistance

UNOS:

United Network for Organ Sharing

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Acknowledgments

We are grateful to Masafumi Ueno (Department of Cardiology, University of Florida College of Medicine, Shands Jacksonville Medical Center, FL 32209, USA). This work was partially supported by a grant to T. Hori from the Uehara Memorial Foundation (no. 200940051, Tokyo 171-0033, Japan). The authors have no financial conflicts of interest to declare.

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Authors and Affiliations

  1. Divisions of Hepato-Pancreato-Biliary and Transplant Surgery, Department of Surgery, Kyoto University Hospital, 54 Shogoinkawara-cho Sakyo-ku, Kyoto, 606-8507, Japan

    Eri Ogawa & Shinji Uemoto

  2. Division of Transplant Surgery, Department of Transplantation, Mayo Clinic in Florida, Jacksonville, FL, 32224, USA

    Tomohide Hori

  3. Division of Pediatric Cardiology, Department of Pediatrics, Kyoto University Hospital, Kyoto, 606-8507, Japan

    Hiraku Doi

  4. Department of Critical Care Medicine and Anesthesia, Kyoto University Hospital, Kyoto, 606-8507, Japan

    Hajime Segawa

Authors
  1. Eri Ogawa
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  2. Tomohide Hori
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  3. Hiraku Doi
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  4. Hajime Segawa
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  5. Shinji Uemoto
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Corresponding authors

Correspondence to Tomohide Hori or Shinji Uemoto.

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Ogawa, E., Hori, T., Doi, H. et al. Living-donor liver transplantation for moderate or severe porto-pulmonary hypertension accompanied by pulmonary arterial hypertension: a single-centre experience over 2 decades in Japan. J Hepatobiliary Pancreat Sci 19, 638–649 (2012). https://doi.org/10.1007/s00534-011-0453-y

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  • DOI: https://doi.org/10.1007/s00534-011-0453-y

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