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| Case of the Week Thursday 3/1/2006 |
Presented by Dr. Hany Shehab .
Hepato-Pulmonary Syndrome
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Staff round presentation
Unit of Professor Dr. Afaf Farrag
Professor Dr. Magdy El Serafy.
Professor Dr. Yasser El Boraey.
Dr. Shereen Hunter.
Dr. Mohammad Mahmoud.
Dr. Hanan Abdel Hafez.
Dr. Yahia El Sharif.
Dr. Alaa Haseeb.
Dr. Mohammad El Said.
Resident:Hany Shehab.
Resident: Mahmoud Abdo.
Personal history:
Saieed M. Soliman, 53 years old.
A father of seven, the youngest is 15 years old.
Owner of a fuel station.
Born and living in El sharkia.
History of contact with canal water, and parenteral anti-shcistosomal therapy .
He used to have hubble-bubble 5 times/day for 12 years, then stopped 1 year ago because of his present illness.
Complaint:
Breathlessness
Present history:
The condition started 3 years ago with a gradual onset and progressive course of dyspnoea (G1 → G3), palpitation, cough and expectoration.The sputum was of moderate amounts ,whitish and has started to be blood-tinged one month ago, with no diurnal or positional variation.
All these symptoms are precipitated by effort and relieved by rest.
There is history of mild weight loss but no anorexia.
No orthopnea, chest pain or wheezes.
No history of abdominal distension, right hypochondrial pain or LL edema.
No history of fever,night sweating, or perception of body swellings.
No skin rashes or joint pains.
No symptoms suggestive of liver cell failure.
Past history:
Diabetic for 4 years on glibenclamide(Daonil) tab once daily.
No history of specific drug intake in the last 6 months.
No history of blood transfusion.
Family history:
No similar condition in the family.
Negative consanguinity.
General examination:
The patient is fully conscious, cooperative, of average intelligence and lying comfortably in bed.
Vital signs:
Bp: 120/80.
Resting pulse: 90 b.p.m,normal character ,average volume ,equal on both sides.
Temp.: 37 ° C , afebrile all through the hospital stay.
1st degree clubbing.
Central cyanosis evident from :
Injected conjunctivae.
Inner surface of the lips.
Under surface of the tongue.
Finger tips.
No jaundice.
No lymphadenopathy.
Neck veins are not congested.
No palmar erythema ,spider naevi or flapping tremors.
No L.L edema
Chest examination
Shape: symmetrically elliptical.
Respiration: chest wall moves freely with respiration at a rate of 18 /min,regular and no use of accessory muscles of respiration.
Trachea : central.
No chest wall tenderness or palpable rhonchi and normal T.V.F .
Normal vesicular breathing and no additional sounds.
Cardiological examination
Normal shape of the precordium.
The cardiac apex is seen and felt in the left 5th intercostal space midclavicular line,localized ,no special character and no thrill.
Normal first and second heart sounds.
No additional sounds and no murmurs.
Abdominal examination:
Inspection:
Normal shape.
Right subcostal angle .
No divarication of the recti .
Umbilicus normal in site and shape, with no impulse on cough & no pigmentations.
No visible veins, hernias, pigmentations or visible peristalsis.
Scar of previous appendicectomy, 5 cm in length, healing by 2ry intention.
Liver:
Upper border : 5th space in Rt MCL .
Lower border:
Rt lobe: 3 cm below costal margin (with deep inspiration)
Lt lobe: 10 cm by light percussion below the xiphisternal junction.
- It is firm in consistency, with smooth surface, sharp edge and not tender or pulsating.
Spleen: not felt, and resonant Traube ’ s area.
No ascites detected clinically.
Fundus examination revealed :
Multiple small peripheral hemorrhages.
Normal maculae and discs.
Summary
A 53-year-old male with:
- dysnoea
-couph
-expectoration
-palpitation
-haemoptysis(of recent onset)
-central cyanosis
-clubbing
-mild hepatomegaly
D.D of central cyanosis
Pulmonary causes :
COPD ( the most common)
Bronchial asthma
Pneumonia
Pulmonary infarction
Interstitial pulmonary fibrosis (IPF)
Granulomatous lung disease
Pulmonary shunts (hepatopulmonary syndrome).
Cardiac causes:
-congenital cyanotic heart disease
-Eisenmenger ’ s syndrome
-acute pulmonary oedema
haemogobinopathies:
-methemoglobinemia
-sulphemoglobinemia
Investigations
Urinalysis
Normal apart from ca-oxalate ++.
CBC
RBCs: 5.47 x 106 /uL.
HGB: 17.8 g/dL.
HCT: 49.5 %.
MCV: 90.5 fL.
MCH: 32.5 pg.
PLT: 94 x 103 /uL.
WBCs: 5900 /uL.
B: 0 %
E: 1 %
St. : 1%
Seg.: 46%
L.: 45 %
M.: 7 %
ESR: 1st hour 35.
Liver biochemical profile:
Total Bilirubin: 1.56 mg/dl.
AST: 84 U (N: 0-32)
ALT: 63 U (N: 0-32)
ALP: 82 U (N: 0-104)
Total proteins: 9.1 g/dl
Albumin: 3 g/dl
Globulins: 6.1 g/dl
PC: 53 %
PT: 18.8 sec.
INR: 1.67
HBsAg: Negative.
Hbs Ab : Negative.
Hb core Total : Reactive.
Hb Core IgM : Negative.
HCV Ab: Positive.
Serum Protein Electrophoresis
(SPEP)
Renal functions:
Urea: 25 mg/dl.
S. Creatinine: 0.58 mg/dl.
Na : 144 mmol/l.
K : 4.4 mmol/l.
Fasting blood sugar: 105 mg/dl
Postprandial blood sugar: 155 mg/dl.
On diet therapy and glibenclamide
Chest x-ray
Normal .
Spiral CT chest
No evidence of hilar or mediastinal lymph node enlargement,masses or calcification.
No pulmonary alveolar or interstitial opacities are seen with normal areation of both lungs.
No evidence of pleural effusion ,pleural thickening or masses.
Normal cardiac size and shape with no gross abnormality of the cardiac chambres.No pericardial effusion.
Normal appearance of the thoracic aorta and the great vessels.
Normal appearance of the chest wall and dorsal vertebrae
The upper abdominal cuts show ?small G.B stone for further evaluation .
Comment: Normal CT chest
Pulmonary function tests
E.C.G
Right B.B.B
D.D of RBBB
Congenital heart disease:
-ASD
-VSD
-Fallot ’ s tetralogy
-pulmonary stenosis
Myocardial disease:
-cardiomyopathy
-Acute myocardial infarction
-conduction system fibrosis
Pulmonary disease:
-cor pulmonale
-recurrent pulmonary embolism
Normal variant in 1% of population
Echocardiography:
Normal internal dimensions, and global contractility of the left ventricle.
No regional wall motion abnormalities at rest study.
Normal other cardiac chambers and valves.
No intracardiac masses or thrombi.
No coarctation of the aorta.
No pericardial effusion.
Abdominal ultrasound:
Liver: average-sized right lobe(13.3 cm), mildly enlarged left lobe(11.5 cm), showing parenchymal coarseness with finely irregular surface, hepatic veins are attenuated, no focal lesions or IHBR dilatation, PV = 12 mm.
G.B: average-sized but thickened wall, with few small calculi inside measuring 4-6 mm in diameter. CBD is not dilated.
Spleen: Average-sized(10 cm), homogenous echopattern.
Kidneys: normal.
Pancreas : free.
No sizeable lymphadenopathy .
No ascites.
Conclusion:
Liver cirrhosis.
Calcular gall bladder.
Upper endoscopy:
Esophagus: 3 cords of grade 1-2 esophageal varices.
Stomach: mucosa of the fundus, body and antrum is hyperaemic and edematous with mosaic pattern. There are several scattered antral erosions.
Pyloric ring: Normal .
Duodenum: free down to D2.
Conclusion:
G1-2 esophageal varices.
Portal hypertensive gastropathy.
Arterial Blood Gases
(A.B.Gs)
While pt → flat:
7.45
25.2
47
17
86%
While pt → sitting:
7.44
23
38
15.8
77%
Contrast-enhanced echocardiography
(CEEC)
Contrast study by injecting hand-agitated saline was done.
The contrast was seen filling the right atrium(R.A) then the right ventricle(R.V).
After 5 beats..
The contrast was seen
in
the left atrium ( L.A. )
and
the left ventricle (L.V.)
Technetium-labelled macro-aggregated albumin perfusion lung scan
The striking feature of this scan is the homogenous visualization of brain activity denoting right to left tracer shunt.
Both lungs portray uniform tracer distribution.
Comment
- Technetium-labelled macro-aggregated albumin should be trapped in normal pulmonary circculation.
- Tracer activity in the kidney,liver,spleen or thyroid is less specific than tracer activity in the brain for pulmonary capillary vasodilation and A-V shunting
- Technetium,released due to poor labelling,may exhibit tracer activity in any organ except the brain.
- Technetium cannot cross the blood-brain-barrier(BBB)
- Technetium bound to albumin can cross BBB.
- Technetium bound to albumin is present in systemic circulation only in case of pulmonay capillary vasodilation and pulmonary A-V shunting .
In the absence of intrinsic cardio-pulmonary disease..
The three criteria needed for the diagnosis are fulfilled:
1-Chronic liver disease.
2-Positive bubble study(CEEC)
3-Abnormal oxygenation(PaO2 <70 MMHG) & orthodeoxia.
Thank you
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