Hepatopulmonary syndrome epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]
Overview
Neither specific etiology nor severity of cirrhosis have been found to be correlated with the incidence or severity of hepatopulmonary syndrome. Hepatopulmonary syndrome occurs in both males and females, in children and adults, and in people of all ethnic backgrounds. Even patients with non-cirrhotic portal hypertension with normal synthetic liver function (e.g. nodular regenerative hyperplasia) may develop HPS, nonetheless, cirrhosis remains the most common cause of HPS.
The most common cause of cirrhosis in the United States is chronic and heavy alcohol use, while the most common cause of cirrhosis worldwide and in Asian countries is the hepatitis virus. The gender that is most commonly affected by cirrhosis varies, depending upon the etiology. The incidence of cirrhosis increases with age; the median age of diagnosis of cirrhosis due to alcoholic liver disease is 52 years. The median age of diagnosis of cryptogenic/NAFLD/NASH cirrhosis is 60 years.
Epidemiology and Demographics
Incidence
- There is no report of HPS prevalence worldwide.[1]
Prevalence
- There is no report of HPS prevalence worldwide.
- Among cirrhotic subjects awaiting orthotopic liver transplantation, approximately 70% complain of dyspnea, 34-47% have intrapulmonary vascular dilatations (IPVDs), and 5-32% have diagnosed HPS.[2]
- Nevertheless, the prevalence of cirrhosis as the most important risk factor of hepatopulmonary syndrome is higher in:
- Non-Hispanic blacks
- Individuals below the poverty line
- Mexican Americans
- Areas with high illiteracy rates
- Chronic and heavy alcohol use is responsible for more than half of the cases of cirrhosis in the United States.[3]
Case-fatality rate/Mortality rate
- In [year], the incidence of hepatopulmonary syndrome is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
- The case-fatality rate/mortality rate of hepatopulmonary syndrome is approximately [number range].
Age
- Patients of all age groups may develop hepatopulmonary syndrome. Age plays no role in the prediction of likelihood of developing HPS in a given patient with cirrhosis.[4]
- Nevertheless, the incidence of cirrhosis increases with age;
- The median age of diagnosis of cirrhosis due to alcoholic liver disease is 52 years.
- The median age of diagnosis of cryptogenic/NAFLD/NASH cirrhosis is 60 years.
Race
- There is no racial predilection to hepatopulmonary syndrome.
Gender
- Hepatopulmonary syndrome affects men and women equally.
- Regarding cirrhosis, the gender that is most commonly affected by cirrhosis varies, depending upon the etiology.
Region
- Hepatopulmonary syndrome is a rare disease without any specific geographical distribution.
- Nevertheless, cirrhosis as the main of hepatopulmonary syndrome has different etiologies in different countries.
Developed Countries
- Chronic and heavy alcohol use is responsible for more than half of the cases of cirrhosis in the United States.
Developing Countries
- Chronic hepatitis B is the most common cause of cirrhosis worldwide, especially South-East Asia, but is less common in the United States.
References
- ↑ 1.0 1.1 Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database. Liver Transpl 10 (2):174-82. DOI:10.1002/lt.20016 PMID: 14762853
- ↑ Martínez GP, Barberà JA, Visa J, Rimola A, Paré JC, Roca J et al. (2001) Hepatopulmonary syndrome in candidates for liver transplantation. J Hepatol 34 (5):651-7. PMID: 11434610
- ↑ Scaglione S, Kliethermes S, Cao G, Shoham D, Durazo R, Luke A, Volk ML (2015). "The Epidemiology of Cirrhosis in the United States: A Population-based Study". J. Clin. Gastroenterol. 49 (8): 690–6. doi:10.1097/MCG.0000000000000208. PMID 25291348.
- ↑ Rodríguez-Roisin R, Krowka MJ (2008) Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med 358 (22):2378-87. DOI:10.1056/NEJMra0707185 PMID: 18509123
- ↑ Fallon MB, Abrams GA (2000) Pulmonary dysfunction in chronic liver disease. Hepatology 32 (4 Pt 1):859-65. DOI:10.1053/jhep.2000.7519 PMID: 11003635
- ↑ Kennedy TC, Knudson RJ (1977) Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 72 (3):305-9. DOI:10.1378/chest.72.3.305 PMID: 891282
- ↑ Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 100 (7):1440-52. DOI:10.1097/TP.0000000000001229 PMID: 27326810
- ↑ Krowka MJ, Dickson ER, Cortese DA (1993) Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest 104 (2):515-21. DOI:10.1378/chest.104.2.515 PMID: 8101797
- ↑ Swanson KL, Wiesner RH, Krowka MJ (2005) Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 41 (5):1122-9. DOI:10.1002/hep.20658 PMID: 15828054
- ↑ Rodríguez-Roisin R, Agustí AG, Roca J (1992) The hepatopulmonary syndrome: new name, old complexities. Thorax 47 (11):897-902. DOI:10.1136/thx.47.11.897 PMID: 1465744