Highlighting Hepatopulmonary Syndrome: An Important Consideration in Decompensated Cirrhosis [Letter]

I would like to commend the authors for their insightful review of ”Management Options in Decompensated Cirrhosis”.¹ Their review provides valuable guidance on addressing various aspects of this complex condition. However, I would like to highlight the importance of including hepatopulmonary syndrome (HPS) as a critical consideration in the management of decompensated cirrhosis.

Hepatopulmonary syndrome (HPS) is a progressive condition associated with worse prognosis in individuals with chronic liver disease. Compared to those without HPS, patients with this syndrome experience a higher mortality rate even when adjusted for factors such as liver disease severity, age, sex, and liver transplantation status.² Patients with hepatopulmonary syndrome (HPS) might either show no symptoms or experience dyspnea. Platypnea, which refers to difficulty in breathing that worsens when sitting or standing, is a classic but not universally present symptom of HPS. Additionally, patients frequently exhibit position-dependent hypoxemia, particularly orthodeoxia, in which oxygen levels drop when in an upright position. During physical examinations, patients with hepatopulmonary syndrome (HPS) typically have clear lung fields. However, if hypoxemia has been present for a long time, they may also show signs such as digital clubbing and/or cyanosis.³ The mortality rate is more than twice as high in patients with HPS.⁴ Treatment options for hepatopulmonary syndrome (HPS) involve starting long-term oxygen therapy for patients with severe HPS (PaO2 <60 mmHg). Liver transplantation (LT) is the only treatment that can resolve HPS. In many countries, patients with severe HPS are granted MELD. Survival rates after liver transplantation are excellent at specialized centers, even in those with severe HPS.⁵

Timely diagnosis of hepatopulmonary syndrome is crucial as it significantly affects management strategies and treatment outcomes. Early identification enables prompt initiation of appropriate interventions such as oxygen therapy and prioritization for liver transplantation, thereby improving patient prognosis and survival rates.

Therefore, it is important to maintain a high level of suspicion for HPS in decompensated cirrhosis as it is frequently overlooked or misdiagnosed.

Disclosure

The author declares no conflicts of interest in this communication.

References

1. Shah NL, Banaei YP, Hojnowski KL, Cornella SL. Management options in decompensated cirrhosis. Hepat Med. 2015;7:43–50. doi:10.2147/HMER.S62463

2. Yi HM, Wang GS, Yi SH, Yang Y, Cai CJ, Chen GH. Prospective evaluation of postoperative outcome after liver transplantation in hepatopulmonary syndrome patients. Chin Med J. 2009;122(21):2598–2602.

3. Fallon MB, Krowka MJ, Brown RS, et al. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology. 2008;135(4):1168–1175. doi:10.1053/j.gastro.2008.06.038

4. Schenk P, Schöniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Müller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology. 2003;125(4):1042–1052. doi:10.1016/s0016-5085(03)01207-1

5. Iyer VN, Swanson KL, Cartin-Ceba R, et al. Hepatopulmonary syndrome: favorable outcomes in the MELD exception era. Hepatology. 2013;57(6):2427–2435. doi:10.1002/hep.26070

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