A Case of Hepato-Pulmonary Syndrome

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A Case of Hepato-Pulmonary Syndrome

  1. 1. BY PROF.P. VIJAYARAGHAVAN’S UNIT ELAVAZHAGAN.B AN INTERESTING CASE OF CYANOSIS
  2. 2. HISTORY <ul><li>A 35 year old female, born of consanguineous marriage was apparently normal till her 18 yrs of age. </li></ul><ul><li>IN 1998: pt developed recurrent bouts of hemetamesis for which pt admitted and evaluated. Pt had splenomegaly and esophageal varices. </li></ul><ul><li>USG shows normal liver size and echotexture </li></ul>
  3. 3. CONTD.. <ul><li>Viral markers were negative. </li></ul><ul><li>Slit lamp examination shows no k f ring. </li></ul><ul><li>1998- 2001 </li></ul><ul><li>Due to recurrent hemetamesis patient underwent sclerotherapy around 13 times over a period of 3 years. </li></ul>
  4. 4. <ul><li>2001: A massive bout of hemetamesis occurred in. Around 5 units of blood tansfusion done. Sengstaken blackmore tried. </li></ul><ul><li>Pt underwent splenectomy and devascularisation done.with prophylactic immunisation for capsulated organisms. </li></ul><ul><li>Liver biopsy shows fibrosis in periportal area and ductular proliferation suggestive of non cirrhotic portal fibrosis . </li></ul><ul><li>. </li></ul>
  5. 5. Over NEXT 10 years <ul><li>Pt gave history of 2- 3 episodes of hematamesis over ten years with conservative management. OGD shows gastric varices in serial examination. </li></ul><ul><li>Pt had recurrent episodes of fever. Two episodes showed plasmodiumvivax. She took treatment for that intermittently. </li></ul><ul><li>h/o recurrent diarrhoea. Colonoscopy normal. </li></ul><ul><li>. </li></ul>
  6. 6. <ul><li>h/o extra pulmonary tuberculosis. </li></ul><ul><li>She had recurrent TB lymphadenitis. She took anti tubercular therapy in 2002, 2003 and 2010. </li></ul><ul><li>In last therapy pt started on liver protective SEO regimen, due to development of drug induced hepatitis </li></ul>
  7. 7. <ul><li>Now admitted for fever for past 10 days. Low grade intermittent. </li></ul><ul><li>On probing history pt complains of - </li></ul><ul><li>breathlessness for past four years, occurs on prolonged standing or sitting position. Feels comfortable in lying down position. </li></ul><ul><li>Elargement of finger tips over nails over past 3 years. </li></ul>
  8. 8. <ul><li>c/o cough , non productive </li></ul><ul><li>NO </li></ul><ul><li>h/o chest pain , palpitation </li></ul><ul><li>h/o swelling of legs </li></ul><ul><li>h/o reduced urine output </li></ul><ul><li>h/o abdominal distension, pain </li></ul><ul><li>h/o rashes </li></ul>
  9. 9. <ul><li>Personal h/0 : </li></ul><ul><li>mixed diet. Not an alcoholic </li></ul><ul><li>unmarried. </li></ul><ul><li>Family history: </li></ul><ul><li>Her younger brother died of AML. </li></ul>
  10. 10. summary <ul><li>A case of non cirrhotic portal fibrosis </li></ul><ul><li>recurrent hemetamesis </li></ul><ul><li>post splenectomy status </li></ul><ul><li>recurrent tb lymphadenitis </li></ul><ul><li>recurrent malarial fever </li></ul><ul><li>breathlessness </li></ul><ul><li>platypnoea </li></ul>
  11. 11. Examination <ul><li>Pt conscious </li></ul><ul><li>oriented </li></ul><ul><li>afebrile </li></ul><ul><li>thin built, moderately nourished </li></ul><ul><li>conjunctiva congested </li></ul><ul><li>no icterus, </li></ul><ul><li>central cyanosis present </li></ul><ul><li>b/l pan digital clubbing grade 3 </li></ul><ul><li>cervical lymphadenopathy present. </li></ul><ul><li>no pedal edema </li></ul>
  12. 12. Systemic examination: <ul><li>CVS: </li></ul><ul><li>S1 S2 heard, no murmurs. </li></ul><ul><li>RS: </li></ul><ul><li>b/l NVBS heard, no added sounds. </li></ul><ul><li>P/A </li></ul><ul><li>soft, non tender, </li></ul><ul><li>dilated veins present, </li></ul><ul><li>no organomegaly </li></ul><ul><li>no free fluid. </li></ul><ul><li>CNS: NFND. </li></ul>
  13. 13. Impression <ul><li>NON CIRRHOTIC PORTAL FIBROSIS </li></ul><ul><li>POST SPLENECTOMY STATUS </li></ul><ul><li>EXTRA PULMONARY TB </li></ul><ul><li>? HEPATOPULMONARY SYNDROME </li></ul>
  14. 14. Investigations <ul><li>CBC: tc-9000 RFT: BS- 98mgs </li></ul><ul><li>dc- p56%l40% e4% urea-30mgs% </li></ul><ul><li>hb- 15gms creatinine- 0.8 </li></ul><ul><li>pcv- 45% electrolytes: na-135meq </li></ul><ul><li>plt- 3 lak k- 3.5 </li></ul><ul><li>esr- 15/45 cl-108 </li></ul><ul><li>hco3-22. </li></ul><ul><li>XRAY CHEST ; normal </li></ul><ul><li>ECG: normal </li></ul>
  15. 15. <ul><li>LFT: t.b-1.3 mgs </li></ul><ul><li>d.b-0.4 </li></ul><ul><li>sgot-40u/l </li></ul><ul><li>sgpt-38u/l </li></ul><ul><li>alk.p- 130 </li></ul><ul><li>s. protein-5.8gms </li></ul><ul><li>albumin-3.0 gms </li></ul><ul><li>VIRAL MARKERS :negative </li></ul>
  16. 16. <ul><li>UGG ABDOMEN : </li></ul><ul><li>liver 15.5cm/ altered echo/surface nodular/ periportal echogenecity. </li></ul><ul><li>other structures normal </li></ul><ul><li>Serum AFP: 2ngs </li></ul><ul><li>CT chest: normal. </li></ul>
  17. 17. O2 saturation in supine position
  18. 18. In erect posture
  19. 19. ABG <ul><li>CHRONIC RESPIRATORY ALKALOSIS . </li></ul><ul><li>PaO2 IN SUPINE </li></ul><ul><li>54.2mmhg, </li></ul><ul><li>PaO2 IN ERECT </li></ul><ul><li>43.9mmhg </li></ul><ul><li>ORTHODEOXIA </li></ul><ul><li>Here both Pco2 and Po2 are low . </li></ul><ul><li>So, high will be the alveolar arterial gradient . </li></ul>
  20. 20. ECHO
  21. 21. CONTRAST ECHO
  22. 24. Final diagnosis <ul><li>NON-CIRRHOTIC PHT OF 10 YEARS DURATION </li></ul><ul><li>POST SPLENECTOMY STATUS </li></ul><ul><li>HEPATOPULMONARY SYNDROME </li></ul>
  23. 25. Hepatopulmonary syndrome <ul><li>In cirrhosis and portal hypertension the micro vascular alteration in pulmonary vascular structures causes impairment of gas exchange results in HPS. </li></ul><ul><li>Also defined as widened age corrected alveolar arterial oxygen gradient on room air in the presence or absence of hypoxemia as a result of intra pulmonary vasodilatation. </li></ul><ul><li>(Aapo2=15mmhg or 20mmhg in pt older than 64 years ) </li></ul>
  24. 26. Grading <ul><li>Mild – Pao2>80mmhg </li></ul><ul><li>Moderate- Pao2 61-80mmhg </li></ul><ul><li>Severe-Pao2 50-60 </li></ul><ul><li>Very severe- <50mmhg </li></ul><ul><li>Also divided as type 1 and type 2, based on angiographic appearance </li></ul><ul><li>Increased mortality on comparing with those without HPS. </li></ul>
  25. 27. Pathophysiology <ul><li>Cirrhosis/ portal hypertension </li></ul><ul><li>Hepatocyte,cholangiocyte injury </li></ul><ul><li>Increased TGF-B </li></ul><ul><li>increased release of endothelin 1/ endothelin b receptor </li></ul><ul><li>increased Endothelial NO synthetase </li></ul><ul><li>Increased NO production </li></ul>
  26. 28. <ul><li>Bacterial translocation </li></ul><ul><li>Increased TNF alfa over expression </li></ul><ul><li>Adherence of macrophages in alveoli </li></ul><ul><li>Inos </li></ul><ul><li>NO production </li></ul>
  27. 29. <ul><li>Heme oxygenase CO production. </li></ul><ul><li>Increased VEGF angiogenesis. </li></ul><ul><li>Pulmonary vasodilatation/angiogenesis </li></ul><ul><li>Dilatation of precapillary/capillary pul.circulation </li></ul><ul><li>Hypoxemia </li></ul><ul><li>Hepato pulmonary syndrome </li></ul>
  28. 30. How ?
  29. 31. How ?
  30. 32. Beyond rules <ul><li>HPS also occurs in </li></ul><ul><li>non cirrhotic/ post hepatitic portal hyper tension, </li></ul><ul><li>Ischemic hepatitis </li></ul><ul><li>Chronic hepatitis in absence of cirrhosis. </li></ul><ul><li>Reference given at the end of presentation. </li></ul>
  31. 33. Signs and symptoms of HPS: <ul><li>Platypnea </li></ul><ul><li>Orthodeoxia </li></ul><ul><li>Insidious, slow progression of dyspnea </li></ul><ul><li>Clubbing </li></ul><ul><li>cyanosis </li></ul><ul><li>Cough. </li></ul><ul><li>Clubbing /hypoxemia(<60mmhg) – highly suggestive </li></ul><ul><li>Presence/ severity of HPS not correlate with degree of hepatic dysfunction. </li></ul>
  32. 34. <ul><li>OXYGEN SATURATION </li></ul><ul><li>≥ 96 % < 96 % </li></ul><ul><li>T T E CONTRAST T T E </li></ul><ul><li>( + ) (- ) </li></ul><ul><li>ABG EXCLUDES LUNG DISEASE A B G EXCLUDES </li></ul><ul><li>LUNG DISEASE </li></ul><ul><li>HYPOXEMIA </li></ul><ul><li>H P S </li></ul>
  33. 35. <ul><li>Contrast echo is the most sensitive test . </li></ul><ul><li>Rule out intrinsic cardiopulmonary disease by xray, ct chest, pulmonary function test. </li></ul><ul><li>In those with associated copd, we have to find out the cause of hypoxia by </li></ul><ul><li>technetium labelled macro aggregated albumin scan </li></ul>
  34. 36. Treatment <ul><li>Those with preserved hepatic synthetic function who have hypoxemia treat symptomatically. </li></ul><ul><li>Medical: oxygenation , pentoxiphylline, garlic preparation. </li></ul><ul><li>almitrine, bismesylate, somatostatin analog , </li></ul><ul><li>oral norfloxacin. </li></ul><ul><li>Interventional : pulmonary angiography with embolisation </li></ul><ul><li>TIPS – to reduce portal pressure. </li></ul>
  35. 37. Transplant <ul><li>Liver transplantation reverses hepatopulmonary syndrome. </li></ul><ul><li>But the mortality after that, higher on comparing with without HPS. </li></ul><ul><li>MELD exception points can be given to HPS with Pao2<60,there by increasing their priority status for transplantation. </li></ul>
  36. 38. Reference: <ul><li>Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. </li></ul><ul><li>Kaymakoglu S , Kahraman T , Kudat H , Demir K , Cakaloglu Y , Adalet I , Dincer D , Besisik F , Boztas G , Sözen AB , Mungan Z , Okten A . </li></ul><ul><li>Source </li></ul><ul><li>Division of Gastroenterology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey. </li></ul>
  37. 39. <ul><li>A severe (type II ) hepatopulmonary syndrome </li></ul><ul><li>in a patient with idiopathic portal hypertension </li></ul><ul><li>and treatment with paroxetine </li></ul><ul><li>S¸. Yilmaz1*, M. Dursum1, F. Canoruç1, K. Bayan1, A. Karabulut2, H. Akay3 </li></ul><ul><li>Departments of 1Gastroenterology, 2Cardiology and 3Radiology, Faculty of Medicine, Dicle University, </li></ul><ul><li>Diyarbakir, Turkey, *corresponding author: tel.: +90412 2488001, fax: +90412 2488523, </li></ul><ul><li>e-mail:drserif@dicle.edu.tr </li></ul><ul><li>© 2005 Van Zuiden Communications B.V. All rights reserved. </li></ul><ul><li>Its incidence in patients with liver cirrhosis is about 10%,2 </li></ul><ul><li>and in the literature this rate is 10 to 20% in patients who </li></ul><ul><li>are candidates to liver transplantation .3,4 A few cases of </li></ul><ul><li>noncirrhotic portal hypertension (NCPH) complicated by </li></ul><ul><li>HPS have been published.5,6 Therefore the cirrhosis is not </li></ul><ul><li>a strict criterion for HPS identification. We present here a </li></ul><ul><li>patient with a classical presentation of severe HPS, which </li></ul><ul><li>was caused by idiopathic portal hypertension, and we discuss </li></ul><ul><li>the effect of paroxetine therapy on this syndrome. </li></ul>
  38. 40. Abstract <ul><li>Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension </li></ul><ul><ul><li>. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram . Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13-62%) and a decreased diffusion capacity of carbon monoxide (40-79%), and 7 of them were hypoxemic (PaO2, 31.6-69.8 mm Hg ). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome. </li></ul></ul><ul><li>PMID: 12757170 [PubMed - indexed for MEDLINE] </li></ul>
  39. 41. Thank you

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