Care of Patient with Hepatopulmonary Syndrome with Case Presentation

1. HEPATOPULMONARY
SYNDROME
Presented by,:
Mr. Albin Joseph,
Senior Staff Nurse, LTICU

2. INTRODUCTION:
Hepato-pulmonary Syndrome is the pulmonary complications in patient
with Liver disease .
The relationship between Liver and Lung was first noted by Fluckiger in (1884)
based on his observation of a woman with cirrhosis , cyanosis, and clubbed
digits.
Hepato-pulmonary syndrome (HPS) was coined in 1977 by Kennedy and
Knudson.

3. What is HPS ?
Triad
1.Liver disease
2.Widespread intrapulmonary vasodilatation
3.Gas exchange abnormality presenting with increased alveolar arterial oxygen
gradient while breathing room air
Liver disease
Intrapulmonary vascular
abnormalities
Impaired
oxygenation

4. • Adult – 4%- 47%
• Pediatrics – 9 %-20%
• Schenk P et al Gastroenterology. 2003;125(4):1042
– Prospective study
– N = 111
– Prevalence – 24%
– Bubble Echo, ABG & PFT
• Younis I et al Ann Hepatol. 2015 May-Jun;14(3):354-60
• N = 111
• Prevalence – 26%
HPS - PREVALANCE:

5. Pathophysiology

6. Homogenous Lung
Diameter of the Pulmonary capillary ranges between 8 and 15 μm, oxygen diffuses
properly into the vessel, and ventilation– perfusion is well balanced.

7. Hepatopulmonary syndrome
In HPS, capillaries are dilated (15–100 µm),and blood flow is not uniform leading to
ventilation- perfusion mismatch

8. CLINICAL FEATURES:
• CYANOSIS
PLATYPNEA
CLUBBING
CYANOSIS
SPIDER NEVI

9. When to suspect HPS ?
O2 Saturation
Below 96%Above 96 %
Bubble EchoHPS Excluded
HPS Excluded
ABGs Exclude
Lung disease
NegativePositive

10. DIAGNOSTIC EVALUATION:
• ABG ANALYSIS

11. BUBBLE ECHO
The timing of the appearance of microbubbles in the left heart distinguishes
between an intrapulmonary shunt and an intracardiac shunt
Intracardiac shunts - microbubbles appear in the left heart within three
heartbeats of their initial appearance in the right heart

12. CT – PULMONORY ANGIOGRAM
Done mainly to rule out the Pulmonary Shunt

13. MAA SCAN
• Panel A - radioactivity in the anterior lungs
• Panel B - radioactivity in the posterior lungs,
as well as the kidneys.
• Panel C - radioactivity in the right side of
the cerebrum
• Panel D- radioactivity in the left side
of the cerebrum
• (uptake in the brain, 62%; normal uptake <6%)

14. Management:
 No established medical therapy currently available for HPS.
 In patients with PaO2 <60 mmHg at rest or with exertion,
administration of supplemental oxygen is appropriate.
 Administration of garlic has resulted in improvement in the
PaO2 in two uncontrolled trials and a small randomized study.
 No study has explored whether ET-1 receptor antagonist or
angiogenesis inhibitors are clinically useful in HPS patients.

15. LIVER TRANSPLANTATION
• LT is an effective therapy for HPS, resulting in complete resolution or
significant improvement in gas exchange in over 85% of patients with severe
hypoxemia.
• Recent study reported mortality of only 9% in patients with severe HPS, as
defined by PaO2 < 50 mmHg.

16. CASE
PRESENTATION

17. PRE – OP HISTORY:
 Mr. X is a 19 year Man with CLD with portal hypertension and its complications
 Bubble echo study showed intrapulmonary shunt.
 MAA scan -shunt fraction as 80%.
 CT pulmonary angiogram -multiple B/L pulmonary AV shunts.
 Coil embolization of the pulmonary AV fistula was done (twice: 6-July-2017
and 17-Aug-2017).
 Prior to transplant, room air saturation was 84% and PaO2 was 47.8 mmHg.
Hb- 16.7g.
 Undergone Liver Transplantation on 21-08-2017

18. INTROP
•Intra-operatively Inhaled NO kept Standby to
prevent De-saturation.
•Intra –op FiO2 0.6% to 0.7% and SpO2 -94%
•Intra- op PICCO GEDI – 600-700, CI – 5.5 -7.0 , SVRI -
800 – 1100 and ELWI – 9 -10.

19. POST –OPERATIVE CARE:
 The surgical procedure was uneventful. His HD stable and SpO2 > 96%.
But his FiO2 requirement varied in LTICU.
 Tracheostomy done on day 3 in view of persistent ventilatory requirements
with high FiO2.
 Inhaled nitric oxide of 12-20 PPM was supplemented for improving
oxygenation.
 monitored for complications due to NO inhalations such as Methemoglobin
levels.
 Had fever of 38.6 C on day 4 and blood culture grew Acinetobacter baumannii
treated as per sensitivity.

20. Contd….
 Nitric oxide supplementation was stopped on day 21.
 He had thick secretions in the Tracheostomy tube and bronchoscopy and lavage
was done on day 28.
 Had an episode of desaturation on day 29 requiring ventilatory assistance for the
next 3 days and slow wean off.
 He was transferred to the ward on day 39. He walks with support and required
oxygen through tracheal mask.
 Intermittently he was with Speaking Valve , Syle’s Tube and Oxygen through Nasal
Prongs and Tracheal Mask respectively.
 Tracheostomy was decannulated on day 50.
 At discharge on 14-09-2017, He was stable, SpO2 98% on room air, walking without
support and tolerating normal oral diet