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Hemodynamic and Clinical Response to Liver Transplantation in Children and Young Adults POPH Patients

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Abstract

Portopulmonary hypertension is an intractable form of pulmonary hypertension. Although liver transplantation is recommended for patients who respond poorly to treatments, the mechanisms by which liver transplantation improves pulmonary hypertension remain unclear. The present study investigated these mechanisms by retrospectively evaluating patients’ data. This study retrospectively evaluated echocardiography and catheterization data before and after liver transplantation in 12 patients who underwent liver transplantation from 2001 to 2019. The 12 patients included one male and 11 females, of median age at liver transplantation of 10 years, 2 months. Nine patients underwent liver transplantation for congenital biliary atresia and three for portal vein aplasia or hypoplasia. Mean pulmonary arterial pressure was 44.1 ± 8.1 mmHg at the first cardiac catheter examination, 35.3 ± 7.8 mmHg before liver transplantation, and 29.5 ± 9.3 mmHg 6 months after liver transplantation. Pulmonary artery pressure was reduced by treatments of pulmonary hypertension and by liver transplantation. Pulmonary vascular resistance did not differ before and after liver transplantation, whereas the cardiac index decreased significantly, indicating that the significant reduction in mean pulmonary artery pressure was due to a decrease in cardiac index. Decreased cardiac index was thought to result from improvements in hyperdynamic conditions due to increased (normalized) systemic vascular resistance. Liver transplantation likely suppresses shear stress on pulmonary arteries, preventing further damage by hyper-circulation. A longer-term evaluation is required to determine the effect of improving pulmonary artery remodeling.

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Acknowledgements

The authors thank all the patients involved in this study, as well as Dr Hiraku Doi, a previous chief of the Pediatric Cardiology Department at Kyoto University Hospital, for valuable advice.

Funding

The study in this manuscript was not supported by any funding. All the treatments described were covered by Japan's national health insurance.

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Authors and Affiliations

  1. Department of Pediatrics, Graduate School of Medicine, Kyoto University, 54 Kawahara-Cho, Shogoin, Sakyo-Ku, Kyoto City, Kyoto, 606-8507, Japan

    Shiro Baba, Kentaro Akagi, Koichi Matsuda, Takuya Hirata & Junko Takita

  2. Department of Pediatric Surgery, Graduate School of Medicine, Kyoto University, 54 Kawahara-Cho, Shogoin, Sakyo-Ku, Kyoto City, Kyoto, 606-8507, Japan

    Eri Ogawa, Tatsuya Okamoto & Hideaki Okajima

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  1. Shiro Baba
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  2. Eri Ogawa
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Contributions

Shiro Baba collected all data and analyzed it, and wrote the main manuscript text. Eri Ogawa, Tatsuya Okamoto, and Hideaki Okajima performed liver transplantation for all the involved patients and collected data. Kentaro Akagi, Koichi Matsuda, and Takuya Hirata analyzed the data and gave comments for the manuscript. Junko Takita supervised and commented dor the writing the manuscript. All authors reviewed the manuscript and accepted the submission.

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Correspondence to Shiro Baba.

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Baba, S., Ogawa, E., Akagi, K. et al. Hemodynamic and Clinical Response to Liver Transplantation in Children and Young Adults POPH Patients. Pediatr Cardiol 45, 1142–1150 (2024). https://doi.org/10.1007/s00246-023-03121-0

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