PANCREAS, BILIARY TRACT, AND LIVER Diagnosis of Hepatopulmonary Syndrome in a Large Integrated Health System Shoma Bommena, * ,‡ Richard D. Gerkin, * ,‡ Sumit Agarwal, * ,‡ Sarah Raevens, § Marilyn K. Glassberg, * ,‡ and Michael B. Fallon* ,‡ *Department of Medicine, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona; ‡ Department of Internal Medicine, Banner University Medical Center-Phoenix, Phoenix, Arizona; and § Department of Internal Medicine, Ghent University, Ghent, Belgium BACKGROUND & AIMS: 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. METHODS: 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. De- mographics and cardiopulmonary testing closest to the time of HPS diagnosis were recorded. HPS was defined using standard criteria. RESULTS: 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 pul- monary 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 dis- ease and were more often diagnosed in transplant centers. CONCLUSIONS: 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. Keywords: Arterial Oxygenation; Intrapulmonary Vascular Dilatation; Pulmonary Hypertension; Hypoxemia. H epatopulmonary syndrome (HPS), the most common pulmonary vascular complication of liver disease, develops when vascular dilation and remodeling in the alveolar microcirculation causes impaired gas exchange. 1-4 It is found in 5%–30% of pa- tients being evaluated for liver transplantation, and its presence doubles mortality and adversely influences the quality of life. 5,6 In patients with cirrhosis, intra- pulmonary vascular dilation (IPVD) detected on contrast- enhanced transthoracic echocardiography (CE TTE) accompanied by abnormal arterial oxygenation in the absence of other causes for altered oxygenation confirm the diagnosis of HPS. 7 Symptoms and signs of HPS are generally nonspecific. Slowly progressive dyspnea at rest or on exertion is the most common symptom. 8 Signs, including digital clubbing and cyanosis, are found in advanced disease, and ortho- deoxia and platypnea are uncommon. 6 Moreover, ascites, hepatic hydrothorax, sarcopenia, and intrinsic cardiopul- monary disease may also contribute to respiratory symptoms and may coexist with IPVD and HPS, necessi- tating a high index of suspicion to make the diagnosis. 9,10 Data on the diagnosis of HPS made in patients with cirrhosis outside of the transplant evaluation setting are limited. We evaluated how often and how accurately HPS Abbreviations used in this paper: A-a, alveolar-arterial gradient; ABG, arterial blood gas analysis; CE TTE, contrast-enhanced transthoracic echocardiography; COPD, chronic obstructive pulmonary disease; HPS, hepatopulmonary syndrome; ICD, International Classification of Diseases; IPVD, intrapulmonary vascular dilatation; LV, left ventricle; MELD, Model for End-Stage Liver Disease; PFT, pulmonary function test; POPH, por- topulmonary hypertension. Most current article © 2021 by the AGA Institute. Published by Elsevier, Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/). 1542-3565 https://doi.org/10.1016/j.cgh.2020.09.050 Clinical Gastroenterology and Hepatology 2021;19:2370–2378
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