Diagnosis Of Hepatopulmonary Syndrome Research Articles

Hepatopulmonary Syndrome: A Comprehensive Review.

Hepatopulmonary syndrome (HPS) is defined by abnormally dilated blood vessels and shunts within the lungs, leading to impaired oxygen exchange. This condition results from intricate interactions between the liver, the gastrointestinal system, and the lungs. This complex system primarily affects pulmonary endothelial, immunomodulatory, and respiratory epithelial cells. Consequently, this contributes to pathological pulmonary changes characteristic of HPS. A classification system based on the severity of oxygen deficiency has been proposed for grading the physiological dysfunction of HPS. Contrast-enhanced echocardiography is considered the primary radiological evaluation for identifying abnormal blood vessel dilations within the lungs, which, combined with an elevated alveolar-arterial gradient, is essential for making the diagnosis. Liver transplantation is the sole effective definitive treatment that can reverse the course of the condition. Despite often being symptomless, HPS carries a significant risk of mortality before transplantation, regardless of the severity of liver disease. Meanwhile, there is varying data regarding survival rates following liver transplantation. The adoption of the model for end-stage liver disease (MELD) standard exception policy has notably improved the results for individuals with HPScompared to the period before MELD was introduced. This review offers a summary of the present understanding, highlighting recent advancements in the diagnosis and treatment of HPS. Furthermore, it aims to augment comprehension of the condition's fundamental mechanisms through insights derived from experimental models and translational research.

Diagnostic Potential of 99mTc-macroaggregated Albumin Scintigraphy in the Diagnosis of Hepatopulmonary Syndrome: Insights from Two Case Studies and Critical Review of Literature

Hepatopulmonary syndrome (HPS) is a rare pulmonary vascular complication of chronic liver disease characterized by dilatation of pulmonary capillaries leading to vascular shunting and systemic hypoxemia. Diagnosis of HPS requires documentation of intrapulmonary vasodilation (IPVD), the two most common imaging studies performed for the detection of IPVD include transthoracic contrast echocardiography (TTCE) and 99m-Tc-macroaggregated albumin scintigraphy (99mTc-MAA scan). TTCE has high sensitivity and thus, is the preferred initial investigation, while 99mTc-MAA scan is highly specific and plays an adjuvant role in diagnosis. 99mTc-MAA scan can, however, identify some cases of HPS not apparent on TTCE and can also quantify the shunt fraction. The current study describes the utility of 99mTc-MAA scan in the detection of IPVD in two suspected cases of HPS.

Favorable Outcomes of Liver Transplantation for Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS. Clinical data, including survival and postoperative efficacy, from patients with HPS from records as obtained over the period from January 1 to December 31, 2022. All records were from a waiting list for LT at the Beijing Friendship Hospital Affiliated with Capital Medical University. Among the 274 patients on the LT waiting list, 37 were diagnosed with HPS (13.50%) and were enrolled. Survival rates of patients with HPS receiving an LT were greater, whereas a statistically significant difference was obtained between patients with LT vs non-LT with moderate to severe HPS (P = .003). The overall time until death without LT was 4-72 days after their initial HPS diagnosis. Patients with HPS receiving an LT showed a significant improvement in the state of oxygenation after surgery (P = .001). Comprehensive preoperative screening of patients on the waiting list for LT is critical to identify those patients with HPS who would maximally benefit from LT. Survival rates of patients with moderate to severe HPS are significantly increased after LT, a procedure that should be performed as soon as possible in these patients with HPS.

Hepatopulmonary syndrome: diagnosis and treatment

Hepatopulmonary syndrome (HPS) is one of the lung diseases associated with liver cirrhosis and portal hypertension. The diagnosis is based on the triad: liver disease and portal hypertension, evidence of intrapulmonary vascular dilatation and impaired gas exchange. HPS impairs prognosis (23 % survival after 5 years) and patients’ quality of life, so early diagnosis and timely treatment are of great importance. Liver transplantation allows for regression of intrapulmonary vascular dilatation in almost 100 % of cases, normalization of gas exchange and improves a 5-year survival after transplantation from 76 to 87 %. This is the only treatment method indicated for patients with severe HPS, defined by an arterial partial pressure of oxygen (PaO2) below 60 mm Hg. However, in the face of a global shortage of transplants, it is necessary to develop medical therapies to delay or even defer liver transplantation. This goal seems possible due to the growing understanding of the HPS pathophysiology and the development of therapies targeting key mechanisms, mainly inflammatory and angiogenic. This article provides an overview of the clinical manifestations, diagnosis and treatment of HPS based on literature sources from the MEDLINE database on the PubMed platform.

Dyspnoea in liver cirrhosis: beyond the ordinary cardiopulmonary cause

BackgroundDyspnoea is a common presentation to the casualties, attributed mostly to cardiopulmonary diseases. Approach to dyspnoea consists of initial acute management to ensure airway patency, respiratory support, and hemodynamic stability, followed by identification of the underlying disease for a definite management to reduce short-term inpatient morbidity and mortality, and rehospitalization in long term. Awareness of the patient’s pre-existing medical conditions is a crucial clue to guide the attending doctor to the primary cause. Dyspnea in chronic liver disease can be due to concomitant cardiopulmonary diseases as well as pulmonary vascular congestion or abnormality associated with portal hypertension such as hepatopulmonary syndrome.Case presentationA 59-year-old man presented with a syncopal attack associated with breathlessness after physical exertion. He had alcohol associated liver cirrhosis for 2 years with a 6-month duration of platypnea and reduced effort tolerance. His physical examination revealed a cyanosed man with normal blood pressure and heart rate, without evidence of systemic and pulmonary fluid congestions. Chest radiography and computed tomography showed no evidence of pulmonary embolism and parenchymal disease and a normal echocardiography ruled out any cardiogenic cause. A diagnosis of hepatopulmonary syndrome was made after the appearance of microbubbles over left atrium at fourth to fifth cardiac cycle demonstrated by a transthoracic contrast-enhanced echocardiography to suggest an extracardiac intrapulmonary shunt.ConclusionsHPS is not an uncommon complication of chronic liver disease. Usual manifestation of HPS is platypnea which needs to be differentiated from orthopnoea associated with cardiopulmonary disease. Nevertheless, there is no effective therapeutic approach of HPS besides liver transplantation.

Abstract 11714: High-Output Heart Failure in Multiple Myeloma: Novel Approach to a Challenging Diagnosis

High-output heart failure (HOHF) is a rare complication of multiple myeloma and is believed to be related to arteriovenous shunting in osseous myeloma lesions. Timely diagnosis is crucial, as these patients respond best to aggressive treatment of the underlying malignancy. In this case, a 51-year-old man with multiple myeloma complicated by extensive osseous disease was admitted from oncology clinic for several weeks of worsening dyspnea, early satiety, and bilateral leg swelling. His exam was notable for signs of volume overload, and the BNP was elevated to 1444 pg/ml. An echocardiogram showed new right ventricular dysfunction. After the patient failed to respond to escalating doses of intravenous diuretics, right heart catheterization was performed and revealed elevated biventricular filling pressures with evidence of high cardiac output (Fick CO 17.1 LPM, Fick CI 7.9 LPM/m 2 ). In a novel diagnostic approach, 5.1 mCi of technetium-99m-labeled macroaggregated albumin was injected into the aorta at the time of left heart catheterization and then whole body planar images were obtained in anterior and posterior projections. The scan revealed evidence of lower extremity arteriovenous shunting with a proportion of 6% (Fig. 1). He was started on lenalidomide and pulse-dose dexamethasone with improvement in diuretic response and resolution of heart failure symptoms. A follow-up echocardiogram showed normalization of right ventricular size and function. He eventually underwent successful allogeneic stem cell transplant during the admission. Although Tc-99m MAA is routinely used in the diagnosis of hepatopulmonary syndrome, this is, to our knowledge, the first report of its use to investigate myeloma-related HOHF in the clinical setting. This technique was previously utilized successfully to experimentally correlate between shunting values and cardiac indices. It may be of use in a wider population of patients with HOHF in whom occult shunting is suspected.

Abstract P5-18-03: Hepatopulmonary syndrome with long term use of ado-trastuzumab emtansine (T-DM1)

Abstract Introduction: Hepatopulmonary syndrome (HPS) is characterized by hypoxemia, portal hypertension, and intrapulmonary shunting and manifests as dyspnea. The portal hypertension may be a result of cirrhosis or non-cirrhotic causes such as nodular regenerative hyperplasia of the liver that is associated with drug therapy. Progressive hypoxemia often occurs despite stable liver function. Ado-trastuzumab emtansine (T-DM1; KADCYLA) is an antibody-drug conjugate comprised of a humanized monoclonal antibody trastuzumab covalently linked to the cytotoxic agent maytansine. T-DM1 is prescribed in the postneoadjuvant setting in persons with residual invasive HER2-positive (HER2+) breast cancer (BC) as well as in persons with metastatic HER2+ BC who were previously treated with trastuzumab and a taxane. Liver toxicities including an asymptomatic, transient increase in transaminases and nodular regenerative hyperplasia that results in portal hypertension have been described; however, HPS secondary to noncirrhotic portal hypertension occurring with long-term exposure to T-DM1 has not been reported. We have identified patients (pts) with this infrequent, yet serious complication, and we aim to better characterize their clinical course to guide earlier detection and hopefully prevent irreversible hypoxic respiratory failure. Methods: Institutional search tools were utilized to identify pts at Mayo Clinic with HER2+ BC treated with T-DM1 in either the postneoadjuvant or metastatic setting. Electronic health records were manually reviewed for HPS criteria including 1) hypoxemia 2) portal hypertension and 3) intrapulmonary shunt/vasodilation confirmed by contrast echocardiogram or macroaggregated albumin lung perfusion scan. For pts meeting criteria for HPS, imaging was independently reviewed by a hepatologist to determine whether there was preexisting liver disease or if changes occurred after initiation of T-DM1. Results: We identified 259 pts with HER2+ BC treated with T-DM1 in the postneoadjuvant (n=70) or metastatic setting (n=185). Four pts met criteria for HPS (1.5%) while receiving T-DM1 for metastatic disease (Table 1; listed in chronological order of onset of HPS). Median age was 43 (range 35 - 53 years), median prior lines of treatment was 1.5 (range 0 - 7), and the median number of cycles of T-DM1 prior to discontinuation was 68.5 (range 51 - 90). All pts had evidence of left atrial enlargement on echocardiogram, and evidence of portal hypertension as determined by the presence of varices, and splenomegaly. Three pts had documented telangiectasias as well as Grade 1-2 elevations in serum transaminases and bilirubin. Elevations in hemoglobin were noted in 2 pts during treatment with T-DM1. All pts had a confirmed intrapulmonary shunt via contrast echocardiogram and a lung perfusion scan. Three pts discontinued T-DM1 after diagnosis of HPS and experienced some clinical improvement in hypoxia. One pt died two months after stopping T-DM1 due to progression of BC. Conclusion: HPS may occur in the setting of prolonged exposure to T-DM1 and can be associated with severe hypoxic respiratory failure. Further data are required to characterize the spectrum of liver injury that occurs with long-term use of T-DM1 to provide further guidance to clinicians as regards monitoring for these adverse effects. Patients who developed hepatopulmonary syndrome while on T-DM1Age, yrsSites of metastases# lines of prior txCycles of T-DM1Presenting sign or symptomsNon-contrast ECHO findingsSupplemental oxygenTransaminitis; hyperbilirubinemiaEvidence of portal hypertensionTelangiectasiasHgb prior to T-DM1Hgb end of txLung perfusion studyContrast ECHOPt 153LN, bone090DOE, hypoxemia, clubbingleft atrial enlargement (62 mg/m2)YesNovarices, splenomegalyNot documented12.9 g/dL10.7 g/dL13.5%Yes, small shuntPt 235LN, bone, liver751DOEleft atrial enlargement (54 mg/m2)YesGrade 1; Grade 2varices, splenomegaly; thrombocytopeniaYes, face and handsn/a11.9 g/dL24%Yes, large shuntPt 338LN, bone251Hyperbilirubinemiaborderline left atrial enlargementYesGrade 1; Grade 2varices, splenomegalyYes, trunk, extremities, face11.7 g/dL16.2 g/dL17.8%Yes, moderate shuntPt 448LN, liver186DOEleft atrial enlargement (49 mg/m2)NoGrade 1; Grade 2varices, splenomegalyYes, trunk12.5 g/dL16.1 g/dL7.9%Yes, large shunt Citation Format: Ally Higgins, Adham K Alkurashi, Patrick S Kamath, Vivek N Iyer, Ciara O'Sullivan, Tufia C Haddad. Hepatopulmonary syndrome with long term use of ado-trastuzumab emtansine (T-DM1) [abstract]. In: Proceedings of the 2021 San Antonio Breast Cancer Symposium; 2021 Dec 7-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2022;82(4 Suppl):Abstract nr P5-18-03.

A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report

Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on 99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (99mTc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on 99mTc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on 99mTc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term.

Hepatopulmonary syndrome: An update.

Hepatopulmonary syndrome (HPS) is characterized by defects in oxygenation caused by intra-pulmonary vasodilation occurring because of chronic liver disease, portal hypertension, or congenital portosystemic shunts. Clinical implications of portal hypertension are very well-known, however, awareness of its effect on multiple organs such as the lungs are less known. The presence of HPS in chronic liver disease is associated with increased mortality. Medical therapies available for HPS have not been proven effective and definitive treatment for HPS is mainly liver transplantation (LT). LT improves mortality for patients with HPS drastically. This article provides a review on the definition, clinical presentation, diagnosis, and management of HPS.

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease.

Pulmonary vascular disease is a frequent complication of chronic liver disease and portal hypertension, affecting up to 30% of patients. There are two distinct pulmonary vascular complications of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS affects 25% of patients with chronic liver disease and is characterized by intrapulmonary vasodilatation and abnormal arterial oxygenation. HPS negatively impacts quality of life and is associated with a 2-fold increased risk of death compared to controls with liver disease without HPS. Angiogenesis, endothelin-1 mediated endothelial dysfunction, monocyte influx, and alveolar type 2 cell dysfunction seem to play important roles in disease pathogenesis but there are currently no effective medical therapies. Fortunately, HPS resolves following liver transplant (LT) with improvements in hypoxemia. POPH is a subtype of pulmonary arterial hypertension (PAH) characterized by an elevated mean pulmonary arterial pressure and pulmonary vascular resistance in the setting of normal left-sided filling pressures. POPH affects 5% to 6% of patients with chronic liver disease. Although the pathogenesis has not been fully elucidated, endothelial dysfunction, inflammation, and estrogen signaling have been identified as key pathways involved in disease pathogenesis. POPH is typically treated with PAH targeted therapy and may also improve with liver transplantation in selected patients. This article highlights what is currently known regarding the diagnosis, management, pathobiology, and outcomes of HPS and POPH. Ongoing research is needed to improve understanding of the pathophysiology and outcomes of these distinct and often misunderstood pulmonary vascular complications of liver disease. © 2021 American Physiological Society. Compr Physiol 11:1-22, 2021.

S2660 A Case of Hepatopulmonary Syndrome Requiring Living Donor Liver Transplantation in a Patient With Nodular Regenerative Hyperplasia and Autoimmune Lymphoproliferative Syndrome-Associated Granulomatous Hepatitis Without Cirrhosis

Introduction: Nodular regenerative hyperplasia (NRH) is a non-cirrhotic cause of portal hypertension that can rarely lead to hepatopulmonary syndrome (HPS). We report on a patient with a rare underlying Autoimmune Lymphoproliferative Syndrome (ALPS), granulomatous hepatitis, and NRH who developed HPS requiring living donor liver transplantation (LDLT). Case Description/Methods: A 34-year-old male with history of ALPS and prior diagnosis of non-necrotizing granulomatous hepatitis with perisinusoidal fibrosis, presented to our clinic for evaluation of worsened liver enzyme elevations. He was asymptomatic with labs notable for ALT 145 U/L, AST 228 U/L, ALP 308 U/L, and total bilirubin 3.0 mg/dL. His liver enzymes did not improve with a trial of high dose steroids, and repeat liver biopsy was suggestive of nodular regenerative hyperplasia. He later developed signs of portal hypertension on imaging as well as esophageal varices on upper endoscopy, with recurrent bleed requiring Transjugular Intrahepatic Portosystemic Shunting (TIPSS) placement. He then developed chronic hypoxia. Echocardiogram with agitated saline study revealed right to left intrapulmonary shunting, consistent with a diagnosis of HPS. The patient’s symptoms quickly progressed with worsening hypoxia requiring continuous oxygen therapy, PaO2 was 48 on room air. He was listed for liver transplantation with exception points for HPS (native MELD-Na = 13, listed at 27). 183 days after listing, he underwent LDLT with complete resolution of hypoxia within 25 days post-transplant. The explant confirmed the absence of cirrhosis and the presence of granulomatous hepatitis and NRH (Figure 1). Discussion: HPS is most commonly associated with cirrhosis. This case highlights a rare instance of HPS in a patient without cirrhosis, but with NRH and concomitant granulomatous hepatitis. LDLT led to a rapid reversal of his HPS.Figure 1.: Histological section of the explanted native liver revealing subtle nodules without fibrous septa composed of hepatocytes arranged in plates 1-2 cells thick (*). Hepatocytes between nodules are compressed into thin, atrophic plates (arrows). (H&E, original magnification x200).

Hepatopulmonary Syndrome: A Nationwide Analysis of Epidemiological Trends and Outcomes From 2012 to 2018.

BackgroundThis study was designed to determine the epidemiological trends and adverse outcomes of hepatopulmonary syndrome (HPS).MethodsThis retrospective interrupted trend study analyzed data from the Nationwide Inpatient Sample (NIS) for the years 2012, 2014, 2016 and 2018 to identify adult (≥ 18 years) hospitalizations with a diagnosis of HPS. We highlighted epidemiological trends for HPS. Inpatient mortality, mean length of stay (LOS) and mean total hospital charge (THC) were estimated using multivariate regression trend analysis.ResultsWe observed an increase in the total number of HPS hospitalizations from 1,565 in 2012 to 2,495 in 2018, with mean age ranging from 55.8 to 58.1 years. There was a trend towards increasing hospitalizations (P-trend < 0.001) with increasing mean age (P-trend = 0.003) for HPS. Whites made up most of the study population. The inpatient mortality for HPS ranged from 12.4% to 12.6%, but there was no statistically significant trend for mortality (P-trend = 0.534) between 2012 and 2018. Additionally, there was no change in both mean LOS (P-trend = 0.545) and mean THC (P-trend = 0.534) for HPS for these years.ConclusionsHospitalizations and mean age for HPS were on the rise. Inpatient mortality ranged from 12.4% to 12.6%; however, a statistically significant trend for mortality was absent.

Hepatopulmonary syndrome is related to the development of acute-on-chronic liver failure and poor prognosis in cirrhotic patients

Long-term prospective data on hepatopulmonary syndrome (HPS) from a large number of patients, especially in Asian patients, are lacking. We evaluated the long-term prognosis of HPS and the development of acute-on-chronic liver failure (ACLF), and related factors. A total of 142 patients with cirrhosis who underwent saline-agitated contrast echocardiography for the diagnosis of HPS were enrolled and observed prospectively from 2014 to 2019. A total of 59 patients (41%) were diagnosed with HPS (24 grade 1, 23 grade 2, 12 grade 3). Thirty-eight and 37 patients died in the HPS and non-HPS groups, respectively (p < 0.01). The 5-year survival rate was 47% in the HPS group and 62% in the non-HPS group. In the Cox proportional hazards model, HPS and Model for End-stage Liver Disease (MELD) score ≥ 18, and Child-Turcotte-Pugh (CTP) class B/C were significant risk factors for mortality after adjusting for other risk factors (HPS hazard ratio [HR] = 1.9, p = 0.01; MELD score ≥ 18 HR = 2.3, p < 0.01; CTP class B/C HR = 2.9, p < 0.01). Compared to that in non-HPS group, the HPS group had a significantly higher incidence of ACLF during follow-up (p < 0.01) and more frequently presented with lung involvement of ACLF (p = 0.03). In the long-term follow-up cohort, patients with HPS showed poorer prognosis than that of patients without HPS. HPS was a risk factor for ACLF development independent of hepatic dysfunction, and lung involvement was significantly common than without ACLF.

Assessment of biventricular function in patients with hepatopulmonary syndrome.

Cardiac function impairment in the setting of hepatopulmonary syndrome (HPS) in patients with end stage liver disease remains an issue of debate. The current study evaluated possible correlations between HPS and biventricular systolic function in patients with decompensated cirrhosis. Consecutive liver transplantation candidates with stable decompensated cirrhosis were prospectively evaluated. HPS was defined as the presence of an elevated alveolar-arterial oxygen gradient and intrapulmonary vasodilatation, detected by contrast enhanced echocardiography. HPS severity was determined based on arterial blood oxygen pressure values, while shunt size was assessed with a semi-quantitative method. Demographic, clinical and laboratory parameters were also prospectively collected. In total, 130 patients (mean age 56.5, M/F: 94/36, MELD score 14.6 ± 5.6) were enrolled, of whom 45 (34.6%) fulfilled the criteria for HPS diagnosis (mild: 57.7%, moderate: 33.3%, severe 4.4% and very severe 4.4%). Significantly lower absolute left ventricular (LV) global longitudinal strain (GLS) values (- 21.6 ± 2.3 vs. - 22.6 ± 2.5%, p = 0.041) were measured in patients with HPS compared to cirrhotic patients without HPS, while there was no statistically significant difference regarding right ventricular GLS (- 22.1 ± 3.3 vs. - 23.2 ± 3.5%, p = 0.061) between the two groups. Lower LV ejection fraction values were also recorded in the HPS group (53.9 ± 3.5 vs. 56.3 ± 4.5%, p < 0.01). No other echocardiographic parameter was correlated to HPS. Intrapulmonary shunt grading was correlated to HPS classification (χ2 = 19.8, p < 0.01), with lower arterial oxygen values being recorded in higher stages of intrapulmonary shunt. In patients with cirrhosis, the presence of HPS is associated with worse LV contractile performance.

Echocardiographic diagnosis of hepatopulmonary syndrome: a valuable tool to remember

Echocardiographic diagnosis of hepatopulmonary syndrome: a valuable tool to remember

Reply to Comment on: Lung Perfusion Imaging with Technetium-99m-macroaggregated Albumin should be Combined with Contrast-enhanced Echocardiography for the Diagnosis of Hepatopulmonary Syndrome.

Reply to Comment on: Lung Perfusion Imaging with Technetium-99m-macroaggregated Albumin should be Combined with Contrast-enhanced Echocardiography for the Diagnosis of Hepatopulmonary Syndrome.

Diagnosis of Hepatopulmonary Syndrome in a Large Integrated Health System

Data on the accuracy of the diagnosis of hepatopulmonary syndrome (HPS) in cirrhosis is limited. We evaluated the clinical characteristics of patients with International Classification of Diseases (ICD) codes for hepatopulmonary syndrome (HPS) in a large integrated health system. A retrospective review of encounters was performed of all patients with ICD-9-CM and/or ICD-10-CM diagnosis of cirrhosis and HPS from 2014-2019 in a multi-state health system. Demographics and cardiopulmonary testing closest to the time of HPS diagnosis were recorded. HPS was defined using standard criteria. A total of 42,749 unique individuals with cirrhosis were identified. An ICD diagnosis of HPS was found in 194 patients (0.45%), of which 182 had clinically confirmed cirrhosis. 143 (78.5%) underwent contrast-enhanced transthoracic echocardiography, and 98 (54%) had delayed shunting. Among them, 61 patients had a documented arterial blood gas, with 53 showing abnormal oxygenation (A-a gradient of >15 mm Hg). 12 were excluded due to significant pulmonary function test abnormalities and abnormal oxygenation from other cardiopulmonary diseases. Ultimately, 41 (22.5%) fulfilled the criteria for HPS. When stratifying those with an ICD code diagnosis of HPS into HPS, no HPS and indeterminate HPS groups, based on standard diagnostic criteria for HPS, we found that the confirmed HPS patients had similar complications except for less portopulmonary hypertension, worse gas exchange, less cardiopulmonary disease and were more often diagnosed in transplant centers. The diagnosis of HPS by ICD code is made in an extremely small subset of a sizeable cirrhotic cohort. When made, only a minority of these patients meet diagnostic criteria. Our findings highlight the need for improved education and more effective screening algorithms.

Endothelin-1 level as a predictor of hepatopulmonary syndrome in liver cirrhosis.

Aim To determine the role of endothelin (ET)-1 in predicting hepatopulmonary syndrome (HPS) in patients with liver cirrhosis. Methods A cross sectional study involving 80 liver cirrhosis patients aged 18 years or older was conducted in Adam Malik General Hospital Medan, Indonesia between July 2017 and June 2018. HPS diagnosis was confirmed from the presence of liver cirrhosis, abnormal oxygenation, and intrapulmonary vascular dilatations (IPVD). ET-1 level was measured from serum sample using ELISA method. Patients with coexisting primary pulmonary pathology and intrinsic heart disease were active smokers, and those who declined to participate were excluded. Statistical analysis was conducted at 95% confidence interval. p<0.05 was considered significant. Results Majority the patients were male (56.3%) and had higher educational background (62.5%). Mean age of the patients was 51.3 (SD=12.6) years. The prevalence of HPS was 21.2%. The patients with HPS had higher ET-1 level compared to those without HPS (p<0.001). The patients with hepatic encephalopathy had 2.917 times higher risk for suffering from HPS, while the patients with Child Pugh score A had lower risk (0.738 times) for having HPS compared to subjects with Child Pugh score B and C. ET-1 level >187.5 mg/L had sensitivity and specificity for predicting HPS in subjects with liver cirrhosis of 82.35% and 81.25%, respectively. Conclusion ET-1 could be used as a promising marker for HPS in patients with liver cirrhosis. ET-1 level of >187.5 mg/l had a good accuracy in predicting HPS in liver cirrhosis patient.

Diagnosis and therapeutic strategies for hepatopulmonary syndrome

Hepatopulmonary syndrome (HPS) is a common pulmonary complication in patients with liver disease and / or portal hypertension, and is characterized by abnormal arterial oxygenation caused by intrapulmonary vascular dilatation. The pathogenesis of HPS is complex, with a low clinical early diagnosis rate and poor prognosis. HPS currently lacks effective therapeutic drugs; therefore, liver transplantation is the only fundamental treatment. This article summarizes the pathogenesis, clinical manifestations, diagnosis and treatment of HPS in order to further improve the level of clinical screening and diagnosis and treatment of HPS.

Comparison of Two Diagnostic Criteria for Hepatopulmonary Syndrome-High Prevalence in Biliary Atresia.

There is lack of clarity regarding the exact prevalence of hepatopulmonary syndrome (HPS) in pediatric liver diseases owing to lack of standardized diagnostic criteria. Thus, we aimed to do a comparative study of HPS with respect to its prevalence using the available diagnostic criteria. All consecutive children with biliary atresia (BA) and other chronic liver diseases (CLDs) were studied. Prevalence of HPS was compared using the 2 available criteria: demonstration of intrapulmonary vascular dilatation along with either alveolar-arterial oxygen difference (P [A-a] O2) on arterial blood gas analysis of more than 15 mmHg (criteria 1), or higher than age-appropriate calculated value for P (A-a) O2 (criteria 2). A total of 42 children in BA group and 62 in the non-BA CLD group were included. Using the criteria 1, the prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group, whereas using criteria 2, the prevalence was 48.1%: 61.9% in the BA group and 38.7% in the CLD group. Criteria 2 diagnosed 6 additional patients with HPS compared to criteria 1 (P value 0.405). BA subjects had higher risk (2.9-3 folds) of developing HPS compared to other CLDs. There is high prevalence of HPS in pediatric liver disease subjects. Age-appropriate formula for HPS diagnosis may be better applicable in pediatric population. BA subjects have a higher risk of developing HPS compared to other CLDs overall, irrespective of the severity of liver disease and/or portal hypertension.