A tale of two entities: a rare presentation of hepatopulmonary syndrome associated with non-cirrhotic portal fibrosis

Priyadharshini V.; Syed Muhammad Humayl S.; Pasupuleti Namo Chengalwarayulu; Murugan K. R.; Shiva Malarvizhi S.; Aarav Joshua Paul; Sahasyaa Adalarasan; Samuel Dinesh A.; Umadevi T. B.

doi:10.18203/2320-6012.ijrms20263279

Authors

Priyadharshini V. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Syed Muhammad Humayl S. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Pasupuleti Namo Chengalwarayulu Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Murugan K. R. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Shiva Malarvizhi S. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Aarav Joshua Paul Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Sahasyaa Adalarasan Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Samuel Dinesh A. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
Umadevi T. B. Institute of Internal Medicine, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20263279

Keywords:

Hepatopulmonary syndrome, Non-cirrhotic portal fibrosis, Orthodeoxia, Intrapulmonary shunt, Liver transplantation

Abstract

Hepatopulmonary syndrome (HPS) is traditionally associated with cirrhosis and advanced chronic liver disease, whereas its occurrence in non-cirrhotic portal fibrosis (NCPF) is distinctly uncommon. We report a 21-year-old woman with NCPF, portal hypertension, and autoimmune hemolytic anemia who presented with progressive dyspnea, fatigue, and dry cough. Examination revealed clubbing and marked orthodeoxia, with oxygen saturation decreasing from 89% supine to 81% upright. Room-air arterial blood gas analysis showed a PaO₂ of 71.9 mmHg with hypocapnia. Contrast-enhanced echocardiography demonstrated delayed microbubble transit into the left atrium via the pulmonary veins, confirming an intrapulmonary right-to-left shunt consistent with HPS. Abdominal imaging demonstrated non-cirrhotic portal hypertension (NCPH) with periportal fibrosis, splenomegaly, and extensive portosystemic collaterals, with preserved hepatic function. The patient underwent right-lobe living-donor liver transplantation and subsequently showed marked clinical and respiratory improvement. This case highlights that HPS may develop in NCPF despite preserved hepatic function and underscores the diagnostic importance of orthodeoxia and intrapulmonary shunting in unexplained hypoxemia.

References

Fallon MB, Abrams GA. Pulmonary dysfunction in chronic liver disease. Hepatology. 2000;32:859-65.

Anand AC, Mukherjee D, Rao KS, Seth AK. Hepatopulmonary syndrome: prevalence and clinical profile. Indian J Gastroenterol. 2001;20:24-7.

Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol. 2002;17(5):526-34.

Sarin SK, Kumar A, Chawla YK, Baijal SS, Dhiman RK, Jafri W, et al. Members of the APASL Working Party on Portal Hypertension. Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int. 2007;1(3):398-413.

Chanda A, Mukherjee M, Mandal M, Hossain MS, Roy N. Face says it all: a curious case of non-cirrhotic portal fibrosis. Int J Adv Med. 2023;10(9):670-2.

Kaymakoglu S, Kahraman T, Kudat H, Demir K, Cakaloglu Y, Adalet I, et al. Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. Dig Dis Sci. 2003;48(3):556-60.

Sood V, Rajesh S, Lal BB, Rawat D, Alam S. Severe hepatopulmonary syndrome in an adolescent patient with non-cirrhotic portal fibrosis. ACG Case Rep J. 2016;3:e174.

Sari S, Oguz D, Sucak T, Dalgic B, Atasever T. Hepatopulmonary syndrome in children with cirrhotic and non-cirrhotic portal hypertension: a single-center experience. Dig Dis Sci. 2012;57(1):175-81.

Borkar VV, Poddar U, Kapoor A, Ns S, Srivastava A, Yachha SK. Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension. Liver Int. 2015;35(6):1665-72.

Maganty K, Ghanta R, Bejarano P, Weppler D, Tekin A, Moon J, et al. Liver transplantation for hepatopulmonary syndrome due to noncirrhotic portal hypertension. Transplant Proc. 2011;43(7):2814-6.

Khanna R, Sarin SK. Non-cirrhotic portal hypertension-diagnosis and management. J Hepatol. 2014;60:421-41.

Zhang M, Luo B, Chen SJ, Abrams GA, Fallon MB. Endothelin-1 stimulation of endothelial nitric oxide synthase in the pathogenesis of hepatopulmonary syndrome. Am J Physiol. 1999;277:G944-52.

Deibert P, Allgaier HP, Loesch S, Müller C, Olschewski M, Hamm H, et al. Hepatopulmonary syndrome in patients with chronic liver disease: role of pulse oximetry. BMC Gastroenterol. 2006;6:15.

Krasinskas AM, Eghtesad B, Kamath PS, Demetris AJ, Abraham SC. Liver transplantation for severe intrahepatic noncirrhotic portal hypertension. Liver Transpl. 2005;11:627-34.