Hepatopulmonary Syndrome and Liver Transplantation: A Recent Review of the Literature

Abstract

A severe and common pulmonary vascular complication of liver disease is hepatopulmonary syndrome (HPS). It is a triad of liver dysfunction and/or portal hypertension, intrapulmonary vascular dilatations, and increased alveolar-arterial oxygen gradient. Prevalence varies according to various study groups from 4%-47%. While the most common presenting symptom of HPS is dyspnea, it is usually asymptomatic, and thus all liver transplant candidates should be screened for its presence. Pulse oximetry is a useful screening method, but arterial blood gas examination is the gold standard. If there is an abnormal P (A-a)O 2 gradient, microbubble transthoracic echocardiography should be done for diagnosis. Outcome is unpredictable, and there is currently no effective medical therapy. The only effective therapy is considered to be liver transplantation. Complete resolution of HPS after liver transplantation is seen within a year in most HPS patients.

Clinical efficacy of transjugular intrahepatic portosystemic shunt in the treatment of hepatopulmonary syndrome

“…[ 11 , 17 ] Furthermore, TIPS and liver transplantation lead to satisfactory outcomes. [ 9 , 27 ] In the present study, only group A displayed short-term complications after TIPS; nonetheless, the study was not designed to address the safety issue of the TIPS approach. In addition, the use of TIPS for HPS is yet controversial.…”

Dyspnoea, Cyanosis and Digital Clubbing in a 28-Year-Old Patient as a Result of Hepatopulmonary Syndrome

“…The development of HPS has been described in the course of the Budd-Chiari syndrome, in portal hypertension without any chronic liver disease, as well as in liver diseases without concomitant cirrhosis and portal hypertension [5,6]. The syndrome is defined as the concurrence of a triad of symptom: liver damage which may be concomitant with portal hypertension, hypoxemia resulting from the disorders in the ventilation to perfusion ratio, and thickening of the intrapulmonary www.journals.viamedica.pl vascular bed [2,7]. Pathophysiology of disorders in the pulmonary circulation has not been fully clarified yet; nevertheless, there is a body of evidence confirming the role of mediators with vasoactive properties, such as nitric oxide (NO), or the tumour necrosis factor a (TNF-a).…”

“…The role of accumulation of macrophages stimulated by the increase of the concentration of endotoxins caused by the deterioration of the liver functions has been postulated, as well. Substances with the angiogenic potential produced by macrophages cause the increase of the number of capillaries and stimulate intervascular connections [3,5,7].…”

“…The results obtained are contradictory, as of today there are no clear-cut criteria that would allow to isolate a group of patients with HPS obtaining measurable benefits by the application of the vascular anastomosis. [14][15][16] As a palliative procedure, embolisaton of thickened vessels or local resections of the pulmonary tissue that contains them is adopted [7]. In connection with the values obtained in gasometry in the patient described herein, the patient was directly qualified to lung transplantation as the target treatment.…”

Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome

“…In pediatrics, the prevalence of HPS has been documented as 4–29% in children with cirrhosis or severe portal hypertension, although this may be an underestimation [ 5 ]. HPS is a progressive disease that is unlikely to resolve without liver transplantation [ 6 ]. Aside from supplemental oxygen, liver transplant is the only treatment option for HPS patients [ 7 ].…”