Pulmonary arterial hypertension is treated with pulmonary vasodilator therapy, anything from oral PDE5 inhibitors or endophilin receptor antagonist all the way up to parenteral prostacycline for our most severe patients. We have an abundance of drugs that we use for pulmonary hypertension in general. Michael, as you said, these all consequences of portal hypertension that's happened with cirrhosis. And now I feel like that thinking is evolved and I've certainly seen patients who have this dysfunctional vascular regulation, an association of cirrhosis and actually have both conditions.
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Episode notes
This week we are joined by one of our Associate Editors, Tess Litchman, as well as two guest experts to discuss two disease states that involve both the liver and the lung. Join us as we go through how to differentiate portopulmonary hypertension and hepatopulmonary syndrome.
Meet the Guests
Tess Litchman is a senior resident at Beth Israel Deaconess Medical Center and is one of the Associate Editors for PulmPEEPs. Tess will be continuing her training as a Pulmonary and Critical Care Medicine fellow at Brigham and Women’s Hospital next year.
Tyler Peck is an Instructor in Medicine at Beth Israel Deaconess Medical Center in the Division of Pulmonary and Critical Care Medicine. Tyler’s clinical and research interests are in pulmonary vascular disease and pulmonary hypertension.
Michael Curry is an Associate Professor of Medicine at Beth Israel Deaconess Medical Center and Section Chief of the Hepatology Department at BIDMC.
Further Readings and References
Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome–a liver-induced lung vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185. PMID: 18509123
Krowka MJ, Fallon MB, Kawut SM, et al. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 2016; 100:1440.
Peppas, S., Nagraj, S., Koutsias, G., Kladas, M., Archontakis-Barakakis, P., Schizas, D., Giannakoulas, G., Palaiodimos, L., & Kokkinidis, D. G. (2022). Portopulmonary Hypertension: A Review of the Current Literature. Heart, lung & circulation, 31(9), 1191–1202. https://doi.org/10.1016/j.hlc.2022.04.056
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