Hilary M DuBrock1, Michael J Krowka2, Kimberly A Forde3, Karen Krok4, Mamta Patel3, Tiffany Sharkoski3, Michael Sprys3, Grace Lin5, Jae K Oh5, Carl D Mottram2, Paul D Scanlon2, Michael B Fallon6, Steven M Kawut3. 1. Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN. Electronic address: dubrock.hilary@mayo.edu. 2. Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN. 3. Center for Clinical Epidemiology and Biostatistics and the Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. 4. Division of Gastroenterology, Department of Medicine, Penn State Milton S. Hershey Medical Center, Hershey, PA. 5. Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. 6. Department of Medicine, University of Arizona, Phoenix, AZ.
Abstract
BACKGROUND: Intrapulmonary vascular dilatations (IPVD) frequently are detected in patients with liver disease by the delayed appearance of microbubbles at contrast-enhanced echocardiography. IPVD with an elevated alveolar-arterial (A-a) gradient define hepatopulmonary syndrome (HPS); however, the importance of IPVD in the absence of abnormal gas exchange is unknown. We aimed to determine the clinical impact of IPVD in patients with liver disease. METHODS: We performed a cross-sectional study within the Pulmonary Vascular Complications of Liver Disease 2 Study, a multicenter, prospective cohort study of patients being evaluated for liver transplant. We excluded patients with obstructive or restrictive lung disease, HPS, or intracardiac shunting. We compared patients with and those without IPVD. RESULTS: Forty-six patients with IPVD and 81 patients without IPVD were included. Patients with IPVD were more likely to have autoimmune hepatitis and less likely to have cryptogenic cirrhosis and hepatocellular carcinoma. Patients with IPVD had higher Child-Pugh scores (6 [interquartile range (IQR), 5-7] vs 5 [IQR, 4-7]; P = .04), possibly higher Model for End-Stage Liver Disease scores (14.5 [IQR, 11.6-15.8] vs 12.2 [IQR, 9.4-15.5]; P = .06), higher PaO2 levels (97.9 [IQR, 92.0-103.0] vs 89.0 [IQR, 82.0-96.9] mm Hg; P < .001), and lower A-a gradients (9.9 [IQR, 6.2-13.5] vs 14.9 [IQR, 9.0-21.8] mm Hg; P < .001). Symptoms and quality of life were similar between the groups. CONCLUSIONS: Autoimmune hepatitis and increased liver disease severity were associated with the presence of IPVD, which was characterized by higher PaO2 levels. Future studies to better characterize IPVD pathogenesis and the relationship of IPVD to HPS are warranted.
BACKGROUND:Intrapulmonary vascular dilatations (IPVD) frequently are detected in patients with liver disease by the delayed appearance of microbubbles at contrast-enhanced echocardiography. IPVD with an elevated alveolar-arterial (A-a) gradient define hepatopulmonary syndrome (HPS); however, the importance of IPVD in the absence of abnormal gas exchange is unknown. We aimed to determine the clinical impact of IPVD in patients with liver disease. METHODS: We performed a cross-sectional study within the Pulmonary Vascular Complications of Liver Disease 2 Study, a multicenter, prospective cohort study of patients being evaluated for liver transplant. We excluded patients with obstructive or restrictive lung disease, HPS, or intracardiac shunting. We compared patients with and those without IPVD. RESULTS: Forty-six patients with IPVD and 81 patients without IPVD were included. Patients with IPVD were more likely to have autoimmune hepatitis and less likely to have cryptogenic cirrhosis and hepatocellular carcinoma. Patients with IPVD had higher Child-Pugh scores (6 [interquartile range (IQR), 5-7] vs 5 [IQR, 4-7]; P = .04), possibly higher Model for End-Stage Liver Disease scores (14.5 [IQR, 11.6-15.8] vs 12.2 [IQR, 9.4-15.5]; P = .06), higher PaO2 levels (97.9 [IQR, 92.0-103.0] vs 89.0 [IQR, 82.0-96.9] mm Hg; P < .001), and lower A-a gradients (9.9 [IQR, 6.2-13.5] vs 14.9 [IQR, 9.0-21.8] mm Hg; P < .001). Symptoms and quality of life were similar between the groups. CONCLUSIONS:Autoimmune hepatitis and increased liver disease severity were associated with the presence of IPVD, which was characterized by higher PaO2 levels. Future studies to better characterize IPVD pathogenesis and the relationship of IPVD to HPS are warranted.
Authors: Michael J Krowka; Michael B Fallon; Steven M Kawut; Valentin Fuhrmann; Julie K Heimbach; Michael A E Ramsay; Olivier Sitbon; Ronald J Sokol Journal: Transplantation Date: 2016-07 Impact factor: 4.939
Authors: Ilaria Lenci; Ace Alvior; Tommaso Maria Manzia; Luca Toti; James Neuberger; Richard Steeds Journal: J Am Soc Echocardiogr Date: 2008-11-20 Impact factor: 5.251
Authors: Elizabeth R Rochon; Michael J Krowka; Sonja Bartolome; Gustavo A Heresi; Todd Bull; Kari Roberts; Anna Hemnes; Kimberly A Forde; Karen L Krok; Mamta Patel; Grace Lin; Megan McNeil; Nadine Al-Naamani; Beth L Roman; Paul B Yu; Michael B Fallon; Mark T Gladwin; Steven M Kawut Journal: Am J Respir Crit Care Med Date: 2020-06-15 Impact factor: 21.405