BY

PROF.P. VIJAYARAGHAVAN’S UNIT

ELAVAZHAGAN.B

AN INTERESTING CASE OF CYANOSIS

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

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.

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.

.

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.

.

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

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.

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

Personal h/0: mixed diet. Not an alcoholic unmarried.

Family history: Her younger brother died of

AML.

summary

A case of non cirrhotic portal fibrosis recurrent hemetamesis post splenectomy status recurrent tb lymphadenitis recurrent malarial fever breathlessness platypnoea

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

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.

Impression

NON CIRRHOTIC PORTAL FIBROSIS POST SPLENECTOMY STATUS EXTRA PULMONARY TB ? HEPATOPULMONARY SYNDROME

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

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

UGG ABDOMEN: liver 15.5cm/ altered echo/surface

nodular/periportal echogenecity. other structures normal

Serum AFP: 2ngs

CT chest: normal.

O2 saturation in supine position

In erect posture

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.

ECHO

CONTRAST ECHO

Final diagnosis

NON-CIRRHOTIC PHT OF 10 YEARS DURATION

POST SPLENECTOMY STATUSHEPATOPULMONARY SYNDROME

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 )

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.

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

Bacterial translocation

Increased TNF alfa over expression

Adherence of macrophages in alveoli

Inos

NO production

Heme oxygenase CO production.

Increased VEGF angiogenesis.

Pulmonary vasodilatation/angiogenesis

Dilatation of precapillary/capillary pul.circulation

Hypoxemia

Hepato pulmonary syndrome

How ?

How ?

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.

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.

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

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

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.

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.

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.

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:drserif@dicle.edu.tr © 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.

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]

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