Hepatopulmonary syndrome history and symptoms: Difference between revisions

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==History and Symptoms==
==History and Symptoms==
*The majority of patients with hepatopulmonary syndrome (80 percent) initially present with their liver disease symptoms. The remaining 20 percent present with pulmonary symptoms. The most complication and presentation is insidiously progressive shortness of breath (dyspnea) at rest or upon exertion.
*The majority of patients with hepatopulmonary syndrome (80 percent) initially present with their liver disease symptoms. The remaining 20 percent present with pulmonary symptoms. The most complication and presentation is insidiously progressive shortness of breath (dyspnea) at rest or upon exertion.<ref name="pmid14708947">Lima BL, França AV, Pazin-Filho A, Araújo WM, Martinez JA, Maciel BC et al. (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=14708947 Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome.] ''Mayo Clin Proc'' 79 (1):42-8. [http://dx.doi.org/10.4065/79.1.42 DOI:10.4065/79.1.42] PMID: [https://pubmed.gov/14708947 14708947]</ref>


*The hallmark of hepatopulmonary syndrome is platypnea (dyspnea in the upright position which is improved in supine position), and orthodeoxia (a drop of  4mmHg in PaO2 or  5% in saturation when moving from the supine to the standing position). Almost 90 percent of patients with HPS present with this specific finding. <ref name="pmid18509123">Rodríguez-Roisin R, Krowka MJ (2008) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18509123 Hepatopulmonary syndrome--a liver-induced lung vascular disorder.] ''N Engl J Med'' 358 (22):2378-87. [http://dx.doi.org/10.1056/NEJMra0707185 DOI:10.1056/NEJMra0707185] PMID: [https://pubmed.gov/18509123 18509123]</ref>
*The hallmark of hepatopulmonary syndrome is platypnea (dyspnea in the upright position which is improved in supine position), and orthodeoxia (a drop of  4mmHg in PaO2 or  5% in saturation when moving from the supine to the standing position). Almost 90 percent of patients with HPS present with this specific finding. <ref name="pmid18509123">Rodríguez-Roisin R, Krowka MJ (2008) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18509123 Hepatopulmonary syndrome--a liver-induced lung vascular disorder.] ''N Engl J Med'' 358 (22):2378-87. [http://dx.doi.org/10.1056/NEJMra0707185 DOI:10.1056/NEJMra0707185] PMID: [https://pubmed.gov/18509123 18509123]</ref>
<ref name="pmid11003635">Fallon MB, Abrams GA (2000) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11003635 Pulmonary dysfunction in chronic liver disease.] ''Hepatology'' 32 (4 Pt 1):859-65. [http://dx.doi.org/10.1053/jhep.2000.7519 DOI:10.1053/jhep.2000.7519] PMID: [https://pubmed.gov/11003635 11003635]</ref>
<ref name="pmid11003635">Fallon MB, Abrams GA (2000) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11003635 Pulmonary dysfunction in chronic liver disease.] ''Hepatology'' 32 (4 Pt 1):859-65. [http://dx.doi.org/10.1053/jhep.2000.7519 DOI:10.1053/jhep.2000.7519] PMID: [https://pubmed.gov/11003635 11003635]</ref>
<ref name="pmid14762853">Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=14762853 Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database.] ''Liver Transpl'' 10 (2):174-82. [http://dx.doi.org/10.1002/lt.20016 DOI:10.1002/lt.20016] PMID: [https://pubmed.gov/14762853 14762853]</ref>
<ref name="pmid14762853">Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C et al. (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=14762853 Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database.] ''Liver Transpl'' 10 (2):174-82. [http://dx.doi.org/10.1002/lt.20016 DOI:10.1002/lt.20016] PMID: [https://pubmed.gov/14762853 14762853]</ref><ref name="pmid891282">Kennedy TC, Knudson RJ (1977) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=891282 Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis.] ''Chest'' 72 (3):305-9. [http://dx.doi.org/10.1378/chest.72.3.305 DOI:10.1378/chest.72.3.305] PMID: [https://pubmed.gov/891282 891282]</ref><ref name="pmid27326810">Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=27326810 International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.] ''Transplantation'' 100 (7):1440-52. [http://dx.doi.org/10.1097/TP.0000000000001229 DOI:10.1097/TP.0000000000001229] PMID: [https://pubmed.gov/27326810 27326810]</ref>
 
<ref name="pmid8101797">Krowka MJ, Dickson ER, Cortese DA (1993) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8101797 Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue.] ''Chest'' 104 (2):515-21. [http://dx.doi.org/10.1378/chest.104.2.515 DOI:10.1378/chest.104.2.515] PMID: [https://pubmed.gov/8101797 8101797]</ref><ref name="pmid15828054">Swanson KL, Wiesner RH, Krowka MJ (2005) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=15828054 Natural history of hepatopulmonary syndrome: Impact of liver transplantation.] ''Hepatology'' 41 (5):1122-9. [http://dx.doi.org/10.1002/hep.20658 DOI:10.1002/hep.20658] PMID: [https://pubmed.gov/15828054 15828054]</ref><ref name="pmid1465744">Rodríguez-Roisin R, Agustí AG, Roca J (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1465744 The hepatopulmonary syndrome: new name, old complexities.] ''Thorax'' 47 (11):897-902. [http://dx.doi.org/10.1136/thx.47.11.897 DOI:10.1136/thx.47.11.897] PMID: [https://pubmed.gov/1465744 1465744]</ref>
<ref name="pmid891282">Kennedy TC, Knudson RJ (1977) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=891282 Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis.] ''Chest'' 72 (3):305-9. [http://dx.doi.org/10.1378/chest.72.3.305 DOI:10.1378/chest.72.3.305] PMID: [https://pubmed.gov/891282 891282]</ref>
<ref name="pmid27326810">Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al. (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=27326810 International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.] ''Transplantation'' 100 (7):1440-52. [http://dx.doi.org/10.1097/TP.0000000000001229 DOI:10.1097/TP.0000000000001229] PMID: [https://pubmed.gov/27326810 27326810]</ref>
<ref name="pmid8101797">Krowka MJ, Dickson ER, Cortese DA (1993) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=8101797 Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue.] ''Chest'' 104 (2):515-21. [http://dx.doi.org/10.1378/chest.104.2.515 DOI:10.1378/chest.104.2.515] PMID: [https://pubmed.gov/8101797 8101797]</ref>
<ref name="pmid15828054">Swanson KL, Wiesner RH, Krowka MJ (2005) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=15828054 Natural history of hepatopulmonary syndrome: Impact of liver transplantation.] ''Hepatology'' 41 (5):1122-9. [http://dx.doi.org/10.1002/hep.20658 DOI:10.1002/hep.20658] PMID: [https://pubmed.gov/15828054 15828054]</ref>
<ref name="pmid1465744">Rodríguez-Roisin R, Agustí AG, Roca J (1992) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=1465744 The hepatopulmonary syndrome: new name, old complexities.] ''Thorax'' 47 (11):897-902. [http://dx.doi.org/10.1136/thx.47.11.897 DOI:10.1136/thx.47.11.897] PMID: [https://pubmed.gov/1465744 1465744]</ref>


*Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis.
*Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis.

Revision as of 14:25, 5 September 2019

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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

The hallmark of hepatopulmonary syndrome is platypnea and orthodeoxia. A positive history of liver disease and dyspnea is suggestive of hepatopulmonary syndrome. Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis

History and Symptoms

  • The majority of patients with hepatopulmonary syndrome (80 percent) initially present with their liver disease symptoms. The remaining 20 percent present with pulmonary symptoms. The most complication and presentation is insidiously progressive shortness of breath (dyspnea) at rest or upon exertion.[1]
  • The hallmark of hepatopulmonary syndrome is platypnea (dyspnea in the upright position which is improved in supine position), and orthodeoxia (a drop of 4mmHg in PaO2 or 5% in saturation when moving from the supine to the standing position). Almost 90 percent of patients with HPS present with this specific finding. [2]

[3] [4][5][6] [7][8][9]

  • Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis.

History

Patients with hepatopulmonary syndrome may have a positive history of:

  • liver disease
  • Dyspnea
  • Unexplained decreased O2 Saturation below 96 percent.
  • Platypnea
  • Orthodeoxia

Common Symptoms

Common symptoms of hepatopulmonary syndrome include:

  • Dyspnea
  • Platypnea
  • Clubbing
  • Cyanosis
  • Symptoms of liver disease like:
  • Spider nevi

Less Common Symptoms

Less common symptoms of hepatopulmonary syndrome include

  • Clubbing
  • Cyanosis

References

  1. ↑ Lima BL, França AV, Pazin-Filho A, Araújo WM, Martinez JA, Maciel BC et al. (2004) Frequency, clinical characteristics, and respiratory parameters of hepatopulmonary syndrome. Mayo Clin Proc 79 (1):42-8. DOI:10.4065/79.1.42 PMID: 14708947
  2. ↑ 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
  3. ↑ 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
  4. ↑ 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
  5. ↑ 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
  6. ↑ 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
  7. ↑ 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
  8. ↑ 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
  9. ↑ 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

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