The hepatopulmonary syndrome

Abstract

IntroductionThe hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution.AimTo update clinical manifestation, diagnosis and treatment of this entity.MethodA literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013.ResultsHepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied.ConclusionThe severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic.

Correlations of MMP‐2 and MMP‐9 gene polymorphisms with the risk of hepatopulmonary syndrome in cirrhotic patients: A case‐control study

“…The diagnostic characteristics of HPS include the presence of portal hypertension or liver disease, an elevated age‐adjusted alveolar‐arterial oxygen gradient (AaPO 2 ), and evidence of intrapulmonary vasodilatation [9,28]. Clubbed fingers and distal cyanosis when accompanied by portal hypertension or liver disease are also classified as red flags and considered to be significant symptoms [4,29].…”

“…Hepatopulmonary syndrome (HPS) is characterized by vasodilatation and portopulmonary hypertension which results from vasoconstriction [2,3]. It was well‐acknowledged as a significant vascular complication in lungs because of systemic hypoxemia in patients with portal hypertension and cirrhosis [4]. And it has been revealed that HPS could elevate the mortality of patients suffering from liver cirrhosis, especially patients plagued by severe hypoxemia [5,6].…”

Hepatopulmonary Syndrome: A Brief Review

“…Moreover, digital clubbing was also more prevalent in a similar population (17 vs 7%, P = 0.03) [1]. Even though the aforementioned symptoms and clinical signs were not specific for the diagnosis of HPS, their presence may lead to an increased V/Q mismatch as the consequence of predominant vasodilation, especially in the basal parts of the lung [14,15]. Direct arteriovenous shunt and V/Q mismatch thus contributed to the occurrence of orthodeoxia.…”

Use of Orthodeoxia by Pulse Oximetry in the Detection of Hepatopulmonary Syndrome

“…This syndrome is currently defined by the presence of this trio: a) hepatic illness, b) hypoxemia (PaO 2 <60 mmHg and difference alveolararterial >15 mmHg) and c) pulmonary vasodilatation [1,2]. Within the physiotherapy of this syndrome we can find three main mechanisms for the hypoxia: intrapulmonary shorts right-left, faults in the capillary socket diffusion of oxygen and alterations in the alveolar perfusionventilation [3].…”