Alharbi S, Alturaif N, Mostafa Y, Alfhaid A, Albenmousa A, Alghamdi S. Pulmonary hypertension post-liver transplant: A case report. World J Gastrointest Surg 2024; 16(12): 3875-3880 [DOI: 10.4240/wjgs.v16.i12.3875]
Corresponding Author of This Article
Noura Alturaif, MBBS, Attending Doctor, Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Almaather, Riyadh 11211, Saudi Arabia. f1500892@kfshrc.edu.sa
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastrointest Surg. Dec 27, 2024; 16(12): 3875-3880 Published online Dec 27, 2024. doi: 10.4240/wjgs.v16.i12.3875
Pulmonary hypertension post-liver transplant: A case report
Sami Alharbi, Noura Alturaif, Yehia Mostafa, Abdullah Alfhaid, Ali Albenmousa, Saad Alghamdi
Sami Alharbi, Noura Alturaif, Yehia Mostafa, Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
Abdullah Alfhaid, Ali Albenmousa, Saad Alghamdi, Department of Liver and Small Bowel Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
Co-first authors: Sami Alharbi and Noura Alturaif.
Author contributions: Alharbi S and Alturaif N equally written, edited, and reviewed the manuscript; Mostafa Y, Alfhaid A, Albenmousa A, equally edited and reviewed the manuscript; Alghamdi S reviewed, edited and approved the final manuscript.
Informed consent statement: Verbal informed consent was obtained from the patient.
Conflict-of-interest statement: All authors have no conflicts of interest to disclose, and they have all signed the copyright license agreement.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Noura Alturaif, MBBS, Attending Doctor, Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Almaather, Riyadh 11211, Saudi Arabia. f1500892@kfshrc.edu.sa
Received: August 26, 2024 Revised: September 26, 2024 Accepted: October 21, 2024 Published online: December 27, 2024 Processing time: 94 Days and 15.8 Hours
Abstract
BACKGROUND
Liver transplantation (LTx) is vital in patients with end-stage liver disease, with metabolic dysfunction-associated steatotic liver disease being the most common indication. Primary sclerosing cholangitis (PSC) is an important indication. Portopulmonary hypertension, associated with portal hypertension, poses a significant perioperative risk, making pretransplant screening essential.
CASE SUMMARY
We report the case of a 41-year-old woman with PSC who developed severe pulmonary hypertension years after a successful LTx. She presented with worsening dyspnea on exertion and presyncope. Diagnostic evaluation confirmed severe precapillary pulmonary hypertension without evidence of recurrent portal hypertension. Initial management with Sildenafil and Macitentan led to a significant improvement in her symptoms, exercise capacity, and biomarkers. This case highlights the rare development of de novo pulmonary hypertension in a liver transplant recipient without recurrent portal hypertension, possibly linked to autoimmune processes or primary liver disease itself. The patient’s positive response to the combination therapy underscores the importance of prompt diagnosis and aggressive management.
CONCLUSION
In conclusion, pulmonary arterial hypertension post-LTx is a rare but serious complication with a poor prognosis, necessitating further research to better understand its mechanisms and to develop effective strategies for prevention and treatment.
Core Tip: We present a young patient, four years post-liver transplant for primary sclerosing cholangitis, who developed World Health Organization group I pulmonary arterial hypertension (PAH). Isolated PAH following liver transplantation is an exceptionally rare condition with an unclear pathology. While we believe it should not be considered idiopathic in the absence of portal hypertension or known etiology, we suspect the prior liver disease or current transplant may contribute to its development. Early recognition and timely intervention are essential for optimizing outcomes. This case highlights the need for vigilance in managing rare post-transplant complications and underscores the importance of further research.
