Unlabelled: Hepatopulmonary syndrome (HPS) is a complication of portal hypertension (PH) defined by the presence of liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations (IPVD) producing a right to left intrapulmonary shunt. Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia may contraindicate LT. The use of transjugular intrahepatic portosystemic shunts (TIPS) could be effective in HPS, although available data is limited.
Aim: To report a clinical case of severe HPS treated sequentially with TIPS and LT.
Case Report: A 46 year old female cirrhotic patient presented with rapidly progressive dyspnea, hypoxemia (PaO2 60 mmHg, SaO2 92%) and increased alveolar-arterial oxygen gradient (A-a) (46 mmHg). She also had orthodeoxia (SaO2 87% in sitting position, but 91% in a prone position). A CT scan and pulmonary angiography were normal. Spirometric assessment showed a mild restrictive pattern and a desaturation was observed in a six-minute walking test. Contrast-enhanced echocardiography (CEE) showed intrapulmonary shunting. A HPS was diagnosed and liver transplantation was disregarded due to severe hypoxemia. The patient underwent TIPS placement. After four weeks, a significant improvement of dyspnea and a complete remission of orthodeoxia were seen. One year later, the patient was successfully transplanted. Interestingly, six months after LT, and in the absence of dyspnea, a new CEE showed persistent passing of bubbles to the left cavities.
Comments/conclusion: Persistent right-to-left shunt after TIPS placement and liver transplantation in spite of the improvement of pulmonary function tests suggests long-term persistence of structural changes in the pulmonary vascular tree after liver transplantation. Because of lack of data, it is not possible to recommend the routine use of TIPS as a part of the conventional management of HPS. However, in patients with severe hypoxemia TIPS placement can reasonably be used as a bridge towards transplantation.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Perioperative Variation of Plasma Copeptin and Its Association With Vasopressor Need During Liver Transplantation.
Transplant Proc
January 2025
Nişantaşı University Health Services Vocational School, Department of Anesthesia, Turkey.
Background: Vasopressor usage during liver transplant is related to decreased hepatic flow, graft failure, and mortality. We measured plasma Copeptin levels in liver transplant patients based on vasopressor requirements. We hypothesize that preoperative plasma copeptin measurement helps predict the vasopressor infusion requirement during liver transplantation in preoperative evaluation.
View Article and Find Full Text PDFKDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary.
Kidney Int
February 2025
Institute of Physiology, University of Zurich, Zurich, Switzerland; Division of Nephrology, Cliniques universitaires Saint-Luc, UCLouvain Medical School, Brussels, Belgium. Electronic address:
The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations.
View Article and Find Full Text PDFMetabolic Dysfunction-Associated Steatotic Liver Disease and the Cardiovascular System.
Trends Cardiovasc Med
January 2025
Department of Cardiology, Euroclinic Hospital, Athens, Greece; First Department of Cardiology, Athens University School of Medicine, Athens, Greece. Electronic address:
Metabolic dysfunction-associated steatotic liver disease (MASLD), previously termed nonalcoholic fatty-liver disease, is an important and rising health issue with a link with atherosclerotic cardiovascular (CV) disease (CVD), affecting ∼25-30% of the adults in the general population; in patients with diabetes, its prevalence culminates to ∼70%; its evolutive form, nonalcoholic steatohepatitis, is estimated to be the main cause of liver transplantation in the future. MASLD is a multisystem disease that affects, besides the liver, extra-hepatic organs and regulatory pathways; it raises the risk of type 2 diabetes mellitus (T2D), CVD, and chronic kidney disease; the disease may also progress to hepatocellular carcinoma. Its diagnosis requires hepatic steatosis and at least one cardiometabolic risk factor and the exclusion of both significant alcohol consumption and other competing causes of chronic liver disease.
View Article and Find Full Text PDFGender-Equity Model for Liver Allocation using Artificial Intelligence (GEMA-AI) for waiting list liver transplant prioritization.
Clin Gastroenterol Hepatol
January 2025
Department of Computer Science and Numerical Analysis, University of Córdoba, Córdoba, Spain. Campus Universitario de Rabanales, Albert Einstein Building. Ctra. N-IV, Km. 396. 14071, Córdoba, Spain; Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, Spain. Av. Menéndez Pidal, s/n, Poniente Sur, 14004 Córdoba, Spain.
Background & Aims: We aimed to develop and validate an artificial intelligence score (GEMA-AI) to predict liver transplant (LT) waiting list outcomes using the same input variables contained in existing models.
Methods: Cohort study including adult LT candidates enlisted in the United Kingdom (2010-2020) for model training and internal validation, and in Australia (1998-2020) for external validation. GEMA-AI combined international normalized ratio, bilirubin, sodium, and the Royal Free Glomerular Filtration Rate in an explainable Artificial Neural Network.
Recurrent Portal Hypertension after Liver Transplant: Impact on Survival and the Role of TIPS Creation in Management.
J Vasc Interv Radiol
January 2025
Mallinckrodt Institute of Radiology, Washington University, Vascular and Intrventional Radiology. Electronic address:
Introduction: Recurrent portal hypertension (PH) after liver transplant (LT) and its management are not well-studied. This study aims to evaluate the impact of transjugular intrahepatic portosystemic shunt (TIPS) on outcomes of recurrent PH.
Methods: From a cohort of 1846 LT recipients, 36 patients who underwent TIPS creation after LT were identified and considered as cases.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!