A Case of Hepato-Pulmonary Syndrome
-
Upload
stanley-medical-college-department-of-medicine -
Category
Health & Medicine
-
view
1.823 -
download
6
Transcript of A Case of Hepato-Pulmonary Syndrome
![Page 1: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/1.jpg)
BY
PROF.P. VIJAYARAGHAVAN’S UNIT
ELAVAZHAGAN.B
AN INTERESTING CASE OF CYANOSIS
![Page 2: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/2.jpg)
HISTORYA 35 year old female, born of
consanguineous marriage was apparently normal till her 18 yrs of age.
IN 1998: pt developed recurrent bouts of hemetamesis for which pt admitted and evaluated. Pt had splenomegaly and esophageal varices.
USG shows normal liver size and echotexture
![Page 3: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/3.jpg)
CONTD..
Viral markers were negative.
Slit lamp examination shows no k f ring.
1998- 2001 Due to recurrent hemetamesis patient
underwent sclerotherapy around 13 times over a period of 3 years.
![Page 4: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/4.jpg)
2001: A massive bout of hemetamesis occurred in. Around 5 units of blood tansfusion done. Sengstaken blackmore tried.
Pt underwent splenectomy and devascularisation done.with prophylactic immunisation for capsulated organisms.
Liver biopsy shows fibrosis in periportal area and ductular proliferation suggestive of non cirrhotic portal fibrosis.
.
![Page 5: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/5.jpg)
Over NEXT 10 years
Pt gave history of 2- 3 episodes of hematamesis over ten years with conservative management. OGD shows gastric varices in serial examination.
Pt had recurrent episodes of fever. Two episodes showed plasmodiumvivax. She took treatment for that intermittently.
h/o recurrent diarrhoea. Colonoscopy normal.
.
![Page 6: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/6.jpg)
h/o extra pulmonary tuberculosis.
She had recurrent TB lymphadenitis. She took anti tubercular therapy in 2002, 2003 and 2010.
In last therapy pt started on liver protective SEO regimen, due to development of drug induced hepatitis
![Page 7: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/7.jpg)
Now admitted for fever for past 10 days. Low grade intermittent.
On probing history pt complains of - breathlessness for past four years,
occurs on prolonged standing or sitting position. Feels comfortable in lying down position.
Elargement of finger tips over nails over past 3 years.
![Page 8: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/8.jpg)
c/o cough , non productiveNO h/o chest pain , palpitation h/o swelling of legs h/o reduced urine output h/o abdominal distension, pain h/o rashes
![Page 9: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/9.jpg)
Personal h/0: mixed diet. Not an alcoholic unmarried.
Family history: Her younger brother died of
AML.
![Page 10: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/10.jpg)
summary
A case of non cirrhotic portal fibrosis recurrent hemetamesis post splenectomy status recurrent tb lymphadenitis recurrent malarial fever breathlessness platypnoea
![Page 11: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/11.jpg)
ExaminationPt conscious oriented afebrile thin built, moderately nourished conjunctiva congested no icterus, central cyanosis present b/l pan digital clubbing grade 3 cervical lymphadenopathy present. no pedal edema
![Page 12: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/12.jpg)
Systemic examination:CVS: S1 S2 heard, no murmurs.RS: b/l NVBS heard, no added sounds.P/A soft, non tender, dilated veins present, no organomegaly no free fluid.CNS: NFND.
![Page 13: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/13.jpg)
Impression
NON CIRRHOTIC PORTAL FIBROSIS POST SPLENECTOMY STATUS EXTRA PULMONARY TB ? HEPATOPULMONARY SYNDROME
![Page 14: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/14.jpg)
InvestigationsCBC: tc-9000 RFT: BS- 98mgs dc- p56%l40% e4% urea-30mgs% hb- 15gms creatinine- 0.8 pcv- 45% electrolytes: na-
135meq plt- 3 lak k- 3.5 esr- 15/45 cl-108 hco3-
22.XRAY CHEST ; normal
ECG: normal
![Page 15: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/15.jpg)
LFT: t.b-1.3 mgs d.b-0.4 sgot-40u/l sgpt-38u/l alk.p- 130 s. protein-5.8gms albumin-3.0 gms
VIRAL MARKERS :negative
![Page 16: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/16.jpg)
UGG ABDOMEN: liver 15.5cm/ altered echo/surface
nodular/periportal echogenecity. other structures normal
Serum AFP: 2ngs
CT chest: normal.
![Page 17: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/17.jpg)
O2 saturation in supine position
![Page 18: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/18.jpg)
In erect posture
![Page 19: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/19.jpg)
ABG
CHRONIC RESPIRATORY ALKALOSIS.
PaO2 IN SUPINE
54.2mmhg,
PaO2 IN ERECT
43.9mmhg
ORTHODEOXIA
Here both Pco2 and Po2 are low .
So, high will be the alveolar arterial gradient.
![Page 20: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/20.jpg)
ECHO
![Page 21: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/21.jpg)
CONTRAST ECHO
![Page 22: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/22.jpg)
![Page 23: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/23.jpg)
![Page 24: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/24.jpg)
Final diagnosis
NON-CIRRHOTIC PHT OF 10 YEARS DURATION
POST SPLENECTOMY STATUSHEPATOPULMONARY SYNDROME
![Page 25: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/25.jpg)
Hepatopulmonary syndrome
In cirrhosis and portal hypertension the micro vascular alteration in pulmonary vascular structures causes impairment of gas exchange results in HPS.
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.
(Aapo2=15mmhg or 20mmhg in pt older than 64 years )
![Page 26: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/26.jpg)
Grading
Mild – Pao2>80mmhgModerate- Pao2 61-80mmhgSevere-Pao2 50-60Very severe- <50mmhg
Also divided as type 1 and type 2, based on angiographic appearance
Increased mortality on comparing with those without HPS.
![Page 27: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/27.jpg)
Pathophysiology Cirrhosis/ portal hypertension
Hepatocyte,cholangiocyte injury
Increased TGF-B
increased release of endothelin 1/ endothelin b receptor
increased Endothelial NO synthetase
Increased NO production
![Page 28: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/28.jpg)
Bacterial translocation
Increased TNF alfa over expression
Adherence of macrophages in alveoli
Inos
NO production
![Page 29: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/29.jpg)
Heme oxygenase CO production.
Increased VEGF angiogenesis.
Pulmonary vasodilatation/angiogenesis
Dilatation of precapillary/capillary pul.circulation
Hypoxemia
Hepato pulmonary syndrome
![Page 30: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/30.jpg)
How ?
![Page 31: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/31.jpg)
How ?
![Page 32: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/32.jpg)
Beyond rules
HPS also occurs in non cirrhotic/ post hepatitic portal
hyper tension,
Ischemic hepatitis
Chronic hepatitis in absence of cirrhosis.
Reference given at the end of presentation.
![Page 33: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/33.jpg)
Signs and symptoms of HPS:PlatypneaOrthodeoxiaInsidious, slow progression of dyspneaClubbing cyanosisCough.
Clubbing /hypoxemia(<60mmhg) – highly suggestive
Presence/ severity of HPS not correlate with degree of hepatic dysfunction.
![Page 34: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/34.jpg)
OXYGEN SATURATION
≥ 96 % < 96 %
T T E CONTRAST T T E
( + ) (- )
ABG EXCLUDES LUNG DISEASE A B G EXCLUDES
LUNG DISEASE
HYPOXEMIA
H P S
![Page 35: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/35.jpg)
Contrast echo is the most sensitive test.
Rule out intrinsic cardiopulmonary disease by xray, ct chest, pulmonary function test.
In those with associated copd, we have to find out the cause of hypoxia by
technetium labelled macro aggregated albumin scan
![Page 36: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/36.jpg)
Treatment Those with preserved hepatic synthetic function
who have hypoxemia treat symptomatically.
Medical: oxygenation, pentoxiphylline, garlic preparation.
almitrine, bismesylate, somatostatin analog ,
oral norfloxacin.
Interventional : pulmonary angiography with embolisation
TIPS – to reduce portal pressure.
![Page 37: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/37.jpg)
Transplant Liver transplantation reverses
hepatopulmonary syndrome.
But the mortality after that, higher on comparing with without HPS.
MELD exception points can be given to HPS with Pao2<60,there by increasing their priority status for transplantation.
![Page 38: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/38.jpg)
Reference:Hepatopulmonary syndrome in
noncirrhotic portal hypertensive patients.
Kaymakoglu S, Kahraman T, Kudat H, Demir K, Cakaloglu Y, Adalet I, Dincer D, Besisik F, Boztas G, Sözen AB, Mungan Z, Okten A.
SourceDivision of Gastroenterology, Istanbul
Medical Faculty, Istanbul University, Istanbul, Turkey.
![Page 39: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/39.jpg)
A severe (type II ) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine S¸. Yilmaz1*, M. Dursum1, F. Canoruç1, K. Bayan1, A. Karabulut2, H. Akay3 Departments of 1Gastroenterology, 2Cardiology and 3Radiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey, *corresponding author: tel.: +90412 2488001, fax: +90412 2488523, e-mail:[email protected] © 2005 Van Zuiden Communications B.V. All rights reserved. Its incidence in patients with liver cirrhosis is about 10%,2 and in the literature this rate is 10 to 20% in patients who
are candidates to liver transplantation.3,4 A few cases of noncirrhotic portal hypertension (NCPH) complicated
by HPS have been published.5,6 Therefore the cirrhosis
is not a strict criterion for HPS identification. We present
here a patient with a classical presentation of severe HPS,
which was caused by idiopathic portal hypertension, and we discuss the effect of paroxetine therapy on this syndrome.
![Page 40: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/40.jpg)
AbstractHepatopulmonary syndrome has yet not been
sufficiently assessed in noncirrhotic portal hypertension . 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.
PMID: 12757170 [PubMed - indexed for MEDLINE]
![Page 41: A Case of Hepato-Pulmonary Syndrome](https://reader036.fdocuments.in/reader036/viewer/2022062404/554b5b91b4c905793d8b4ef9/html5/thumbnails/41.jpg)
Thank you