Frequency, Clinical Characteristics, and Respiratory Parameters of Hepatopulmonary Syndrome

doi:10.4065/79.1.42

Mayo Clinic Proceedings

Volume 79, Issue 1, January 2004, Pages 42-48

https://doi.org/10.4065/79.1.42 Get rights and content

Objective

To determine the frequency and the clinical characteristics of hepatopulmonary syndrome (HPS) in cirrhotic candidates for orthotopic liver transplantation and to identify the major respiratory parameters predictive of the presence of changes in arterial oxygenation.

Patients and Methods

Patients underwent transthoracic contrast-enhanced echocardiography, pulmonary scintigraphy, pulmonary function test with diffusing capacity of lung for carbon monoxide (Dlco), and measurement of arterial blood gases.

Results

Fifty-six patients were studied. Twenty-five patients (45%) presented with intrapulmonary vascular dilatations, but only 9 (16%) fulfilled the criteria for HPS. The clinical or demographic characteristics considered did not differ in the patients with and without HPS. The Dlco value was significantly lower in patients with HPS (P=.01). However, 32 (80%) of 40 patients with low Dlco values did not fulfill the criteria for HPS. An alveolar arterial oxygen gradient (AaPo₂) of more than 20 mm Hg showed a higher diagnostic accuracy (91%) in the assessment of HPS than did the Dlco of less than 80% predicted (41%) and the AaPo₂ of more than 15 mm Hg (71%).

Conclusions

The AaPo₂ proved to be a more reliable index than Pao₂ and Dlco for the determination of changes in arterial oxygenation in HPS. The Dlco does not seem to be a good marker for HPS screening. Intrapulmonary vascular dilatations were frequent, even in patients who did not fulfill the criteria for HPS.

Section snippets

PATIENTS AND METHODS

The diagnosis of hepatic cirrhosis (HC) was based on clinical and laboratory criteria and on histologic analysis when available. Hepatopulmonary syndrome was defined by the presence of HC and IPVDs detected by transthoracic contrast-enhanced echocardiography (CEE) and of disorders of arterial oxygenation represented by a Pao₂ of less than 70 mm Hg or an AaPo₂ of more than 20 mm Hg.¹ The study was approved by the Ethics Committee of the University Hospital of the Faculty of Medicine of Ribeirão

RESULTS

Sixty consecutive cirrhotic patients who were candidates for OLT were evaluated prospectively from August 2001 to August 2002. Fifty-six patients (93%) concluded the protocol. The exclusion of 4 patients was due to an inadequate acoustic window for transthoracic CEE in 2 patients, to the death of 1 patient before the conclusion of the protocol, and to the impossibility of performing pulmonary function evaluation in 1 patient.

Twenty-five (45%) of the 56 patients had IPVDs as detected by

DISCUSSION

Until recently, it was difficult to define HPS because no consensus existed among investigators about the diagnostic criteria for the syndrome. Although doubts still persist today, some aspects have been better defined. In the current study, which used the criteria recommended by Krowka and Cortese,¹⁵ 16% of the cirrhotic patients assessed for OLT were found to have HPS, a frequency similar to that reported in the literature, which ranges from 4% to 32%3, 16, 17, 18 according to the criteria

CONCLUSIONS

The frequency of HPS is high among cirrhotic patients who are candidates for OLT. Although IPVDs are frequent among candidates for OLT, most patients do not fulfill the criteria for HPS. The Dlco value does not seem to be a good marker for HPS screening because it is reduced in patients both with and without HPS. We emphasize the role of AaPo₂ as a more reliable index than Pao₂ (even with higher cutoff levels) and of Dlco in the determination of changes in arterial oxygenation in cirrhotic

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Hepatopulmonary syndrome (HPS) is a condition frequently associated with severe liver disease, where the presence of intrapulmonary vascular dilations result in shunting and abnormal arterial oxygenation [1]. The incidence of HPS in patients with cirrhosis is estimated to be 10–30% [2–4]. In the past, HPS has been considered a relative contraindication to liver transplantation (LT) [5].
Isolated cases of extracorporeal membrane oxygenation (ECMO) in patients with hepatopulmonary syndrome (HPS) undergoing liver transplantation (LT) have been reported with increasing frequency. We aimed to systemically review and synthesize the available literature on ECMO use in this population.
A systematic literature review of the PubMed, Web of Science, Cochrane Library, and Embase databases (end-of-search date: November 14, 2021) was conducted in accordance with the PRISMA statement. Eligible studies presented clinical parameters and outcomes of adult or pediatric patients with HPS receiving ECMO support at the time of, or following, LT.
Sixteen studies from 4 continents reporting on 17 patients who were initiated on ECMO prior to (n = 2), during (n = 1) or after LT (n = 14) were included. Nine of the 16 studies were published between 2019 and 2021. The median pre-LT PaO₂ was 38.0 mmHg (IQR 35.0–52.0). The median time from LT to ECMO initiation was 7 days (IQR, 3–12). Six patients (50%, n = 6 of 12) were extubated post-LT, before deterioration, development of refractory hypoxemia, and initiation of ECMO. Most patients were cannulated with a venovenous configuration (75%, n = 12 of 16). Most patients cannulated with a venoarterial or veno-arterial-venous strategy (75%, n = 3 of 4) had concurrent hemodynamic instability. The median total time on ECMO was 13 days (IQR 10–29). Using linear regression, for patients cannulated postoperatively, each day between LT and ECMO initiation was associated with a 3.5-day increase in total ECMO duration (95%CI: 2.23–4.73, p < 0.001, R² = 73.7%). The median postoperative intensive care unit length of stay was 40 days (IQR, 37–61) and hospital length of stay was 59.5 days (IQR 42–77). 82.4% of patients (14 of 17) survived to discharge.
ECMO is feasible in patients with HPS undergoing LT and appears to be associated with better outcomes compared to other causes of cardiopulmonary failure in LT patients. As the volume of experience grows, ECMO may become a central part of perioperative support in LT patients with severe HPS.
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The hepatopulmonary syndrome (HPS) is defined as the triad of liver disease, intrapulmonary vascular dilatation, and abnormal gas exchange, and is found in 10–32% of patients with liver disease. Liver transplantation is the only known cure for HPS, but patients can develop severe posttransplant hypoxemia, defined as a need for 100% inspired oxygen to maintain a saturation of ≥85%. This complication is seen in 6–21% of patients and carries a 45% mortality. Its management requires the application of specific strategies targeting the underlying physiologic abnormalities in HPS, but awareness of these strategies and knowledge on their optimal use is limited. We reviewed existing literature to identify strategies that can be used for this complication, and developed a clinical management algorithm based on best evidence and expert opinion. Evidence was limited to case reports and case series, and we determined which treatments to include in the algorithm and their recommended sequence based on their relative likelihood of success, invasiveness, and risk. Recommended therapies include: Trendelenburg positioning, inhaled epoprostenol or nitric oxide, methylene blue, embolization of abnormal pulmonary vessels, and extracorporeal life support. Availability and use of this pragmatic algorithm may improve management of this complication, and will benefit from prospective validation.
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Original ArticleFrequency, Clinical Characteristics, and Respiratory Parameters of Hepatopulmonary Syndrome

Objective

Patients and Methods

Results

Conclusions

Section snippets

PATIENTS AND METHODS

RESULTS

DISCUSSION

CONCLUSIONS

Chest

Gastroenterology

Clin Chest Med

Surg Clin North Am

Liver Transpl

J Hepatol

J Am Soc Echocardiogr

Am J Cardiol

Chest

Mayo Clin Proc

Am J Med

Am Heart J

Hepatology

Mayo Clin Proc

Am J Med

J Hepatol

Chest

Am J Cardiol

Original Article
Frequency, Clinical Characteristics, and Respiratory Parameters of Hepatopulmonary Syndrome