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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; 16:3875-3880. [PMID: 39734439 PMCID: PMC11650223 DOI: 10.4240/wjgs.v16.i12.3875] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/26/2024] [Revised: 09/26/2024] [Accepted: 10/21/2024] [Indexed: 11/27/2024] Open
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.
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Affiliation(s)
- Sami Alharbi
- Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
| | - Noura Alturaif
- Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
| | - Yehia Mostafa
- Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
| | - Abdullah Alfhaid
- Department of Liver and Small Bowel Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
| | - Ali Albenmousa
- Department of Liver and Small Bowel Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
| | - Saad Alghamdi
- Department of Liver and Small Bowel Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia
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Leatheng C, Khatskevich K, Erno J, Riemer EC. Unlikely Death From Esophageal Variceal Hemorrhage in a Post-Orthotopic Liver Transplant Patient. Acad Forensic Pathol 2024; 14:142-145. [PMID: 39534450 PMCID: PMC11552032 DOI: 10.1177/19253621241278889] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2023] [Accepted: 08/05/2024] [Indexed: 11/16/2024]
Abstract
Gastrointestinal bleeding is one of the complications seen after a liver transplant with a poor prognosis and low survival. The most common cause of gastrointestinal bleeding after a liver transplant is ulcers followed by hemorrhagic enteritis and portal hypertensive lesions. We present a case of a middle-aged male, with improving liver function labs and imaging, who passed away from large-volume hematemesis approximately 2 months after a liver transplant. On autopsy, the cause of death was concluded to be from acute upper gastrointestinal hemorrhage due to esophageal variceal bleeding.
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Affiliation(s)
- Chanbormey Leatheng
- Chanbormey Leatheng, MD, Department of Internal Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH,
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Moon HH. Refractory Ascites with Intrahepatic Portal Thrombosis after Living Donor Liver Transplantation Successfully Treated by Splenic Artery Embolization and Apixaban (Case Report). KOSIN MEDICAL JOURNAL 2021. [DOI: 10.7180/kmj.2021.36.2.187] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
Abstract
Refractory ascites is a rare complication after liver transplantation, and its incidence ranges from 5% to 7%. A 56-yearold man diagnosed with HBV-LC with massive ascites underwent living donor liver transplantation. After transplantation, more than 1000 ml/day of ascites was steadily drained until two weeks after LT. CT showed intrahepatic Rt. portal vein thrombosis and many remnant collaterals with splenomegaly. We decided to embolize the proximal splenic artery and use apixaban to reduce portal flow and resolve the intrahepatic portal thrombosis. One day after splenic artery embolization, the patient's ascites dramatically decreased. Three days later, he was discharged from the hospital. Three months later, a follow-up liver CT showed resolution of thrombosis and no ascites. Splenic artery embolization was an effective and safe procedure for portal flow modulation in portal hyertension. Apixaban was effective for partial portal vein thrombosis in a liver transplant recipient.
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Hartl L, Elias J, Prager G, Reiberger T, Unger LW. Individualized treatment options for patients with non-cirrhotic and cirrhotic liver disease. World J Gastroenterol 2021; 27:2281-2298. [PMID: 34040322 PMCID: PMC8130039 DOI: 10.3748/wjg.v27.i19.2281] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2021] [Revised: 03/19/2021] [Accepted: 04/25/2021] [Indexed: 02/06/2023] Open
Abstract
The obesity pandemic has led to a significant increase in patients with metabolic dysfunction-associated fatty liver disease (MAFLD). While dyslipidemia, type 2 diabetes mellitus and cardiovascular diseases guide treatment in patients without signs of liver fibrosis, liver related morbidity and mortality becomes relevant for MAFLD's progressive form, non-alcoholic steatohepatitis (NASH), and upon development of liver fibrosis. Statins should be prescribed in patients without significant fibrosis despite concomitant liver diseases but are underutilized in the real-world setting. Bariatric surgery, especially Y-Roux bypass, has been proven to be superior to conservative and/or medical treatment for weight loss and resolution of obesity-associated diseases, but comes at a low but existent risk of surgical complications, reoperations and very rarely, paradoxical progression of NASH. Once end-stage liver disease develops, obese patients benefit from liver transplantation (LT), but may be at increased risk of perioperative infectious complications. After LT, metabolic comorbidities are commonly observed, irrespective of the underlying liver disease, but MAFLD/NASH patients are at even higher risk of disease recurrence. Few studies with low patient numbers evaluated if, and when, bariatric surgery may be an option to avoid disease recurrence but more high-quality studies are needed to establish clear recommendations. In this review, we summarize the most recent literature on treatment options for MAFLD and NASH and highlight important considerations to tailor therapy to individual patient's needs in light of their risk profile.
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Affiliation(s)
- Lukas Hartl
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna A-1090, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna A-1090, Austria
| | - Joshua Elias
- Cambridge Institute of Therapeutic Immunology and Infectious Disease, Jeffrey Cheah Biomedical Centre, University of Cambridge, Cambridge CB2 0AW, United Kingdom
- Division of Gastroenterology and Hepatology, Department of Medicine, University of Cambridge, Addenbrooke’s Hospital, Cambridge CB2 0QQ, United Kingdom
| | - Gerhard Prager
- Division of Visceral Surgery, Department of General Surgery, Medical University of Vienna, Vienna A-1090, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna A-1090, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna A-1090, Austria
| | - Lukas W Unger
- Cambridge Institute of Therapeutic Immunology and Infectious Disease, Jeffrey Cheah Biomedical Centre, University of Cambridge, Cambridge CB2 0AW, United Kingdom
- Division of Visceral Surgery, Department of General Surgery, Medical University of Vienna, Vienna A-1090, Austria
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García-Pagán JC, Saffo S, Mandorfer M, Garcia-Tsao G. Where does TIPS fit in the management of patients with cirrhosis? JHEP Rep 2020; 2:100122. [PMID: 32671331 PMCID: PMC7347999 DOI: 10.1016/j.jhepr.2020.100122] [Citation(s) in RCA: 75] [Impact Index Per Article: 15.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Revised: 04/20/2020] [Accepted: 05/11/2020] [Indexed: 02/06/2023] Open
Abstract
In this review, we summarise the current knowledge on the indications and contraindications of transjugular intrahepatic portosystemic shunt (TIPS) placement for the treatment of the complications of portal hypertension in cirrhosis, specifically variceal haemorrhage and ascites. Moreover, we discuss the role of TIPS for the treatment of portal vein thrombosis (PVT) and the prevention of complications after extrahepatic surgery ('preoperative TIPS') in patients with cirrhosis. The position of TIPS in the treatment hierarchy depends on the clinical setting and on patient characteristics. In acute variceal haemorrhage, preemptive TIPS is indicated in patients at a high risk of failing standard therapy, that is those with a Child-Pugh score of 10-13 points or Child-Pugh B with active bleeding at endoscopy, although the survival benefit in the latter group still remains to be established. Non-preemptive TIPS is a second-line therapy for the prevention of recurrent variceal haemorrhage and for the treatment of ascites. Of note, TIPS may also improve sarcopenia. Contraindications to TIPS placement, independent of clinical setting, include very advanced disease (Child-Pugh >13 points), episodes of recurrent overt hepatic encephalopathy without an identifiable precipitating factor, heart failure, and pulmonary hypertension. In patients with PVT, TIPS placement not only controls complications of portal hypertension, but also promotes portal vein recanalisation. Although the severity of portal hypertension correlates with poor outcomes after extrahepatic surgery, there is no evidence to recommend preoperative TIPS placement.
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Key Words
- ACLF, acute-on-chronic liver failure
- AKI, acute kidney injury
- ARR, absolute risk reduction
- AVB, acute variceal bleeding
- Ascites
- BNP, brain natriuretic peptide
- BRTO, balloon-occluded retrograde transvenous obliteration
- Bleeding
- CHF, chronic heart failure
- CLD, chronic liver disease
- CSPH, clinically significant portal hypertension
- Cirrhosis
- EVL, endoscopic variceal ligation
- GOV, gastro-oesophageal varices
- HCC, hepatocellular carcinoma
- HE, hepatic encephalopathy
- HVPG, hepatic venous pressure gradient
- Haemorrhage
- ICA, International Club of Ascites
- IGV, isolated gastric varices
- INR, international normalised ratio
- ISMN, isosorbide mononitrate
- LVP+A, LVP with albumin
- LVP, large-volume paracenteses
- MELD, model for end-stage liver disease
- NNT, number needed to treat
- NSBB, non-selective beta blocker
- OS, overall survival
- PCI, percutaneous coronary intervention
- PFTE, polytetrafluoroethylene
- PLT, platelet count
- PSE, portosystemic encephalopathy
- PV, portal vein
- PVT, portal vein thrombosis
- Portal hypertension
- Portal vein thrombosis
- RA, refractory ascites
- RCTs, randomised controlled trials
- SBP, spontaneous bacterial peritonitis
- SEMS, self-expandable metallic stent
- TFS, transplant-free survival
- TIPS, transjugular intrahepatic portosystemic shunt
- Transjugular intrahepatic portosystemic shunt
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Affiliation(s)
- Juan Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Lab, Liver Unit, Hospital Clínic, Barcelona, Spain
- Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
- Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Madrid, Spain
| | - Saad Saffo
- Section of Digestive Diseases, Yale School of Medicine, New Haven, CT, USA
| | - Mattias Mandorfer
- Barcelona Hepatic Hemodynamic Lab, Liver Unit, Hospital Clínic, Barcelona, Spain
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Guadalupe Garcia-Tsao
- Section of Digestive Diseases, VA-Connecticut Healthcare System, West Haven, CT, USA
- Section of Digestive Diseases, Yale School of Medicine, New Haven, CT, USA
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