h epatopulmonary syndrome and cirrhotic cardiomyopathy perceptor: dr shalimar
TRANSCRIPT
![Page 1: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/1.jpg)
HEPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY
Perceptor: Dr Shalimar
![Page 2: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/2.jpg)
PULMONARY COMPLICATIONS IN LIVER DISEASE
Parenchyma • Pneumonia• Lymphocytic/
organising pneumonia - PBC
• Panacinar emphysema – alpha1 anti trypsin deficiency
• Aspiration pneumonia – Hepatic encephalopathy
Pleura / Diaphragm • Hepatic hydrothorax• Chylothorax• Effect of massive
ascites
Pulmonary vasculature• HPS• PPH
![Page 3: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/3.jpg)
HPS
1884 Fluckiger, described a patient with
cirrhosis, marked cyanosis and clubbing
1966 Berthelot- dilatation of pulmonary vessels
in an autopsy series
‘Hepatopulmonary syndrome’ coined in 1977
Kennedy et al. Exercise aggravated hypoxemia and orthodeoxia in cirrhosis. Chest 1977;72:305-9
![Page 4: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/4.jpg)
HPS
Triad
Arterial oxygenation defect
Intrapulmonary vasodilation
Presence of liver disease
Prevalence among liver transplant patients 4% to 47%
Variability in prevalence- Nonspecificity of clinical criteria & lack of
a confirmatory test
For eg: 91% of healthy subjects: varying degrees of
intrapulmonary shunting during submaximal aerobic exercise!
Can occur in Chronic hepatitis and in NCPF
Mortality rate of 41% ( 9 of 22 adult patients ) at a mean of 2.5
years ( range, 1 to 5 years ) after the diagnosis
Grace et al, journal of gastroenterology and hepatology 28 (2013) 213-219
![Page 5: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/5.jpg)
HPS
![Page 6: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/6.jpg)
PATHOGENESIS OF HPS
Grace et al, journal of gastroenterology and hepatology 28 (2013) 213-219
Liver injury TGF/VEGF Angiogenesis
![Page 7: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/7.jpg)
![Page 8: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/8.jpg)
HEPATOPULMONARY SYNDROME
Roberto et al. N Engl J Med 2008;358:2378-87
![Page 9: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/9.jpg)
CLINICAL PRESENTATION
Dyspnea, platypnea and orthodeoxiaClubbing
• CLD + PHTN (82% of patients).• Dyspnea (18%); may be accompanied by
platypnea and orthodeoxia. Khan et al : Pulmonary vascular complications of CLD , Annals of thoracic medicine – vol 6,issue 2, April –
June 2011
Spider angioma - may represent cutaneous markers of intrapulmonary vascular dilatations
Lima et al , Frequency , clinical characteristics resp parameters of HPS. Mayo Clin Proc 2004;79:42-8
![Page 10: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/10.jpg)
ORTHODEOXIA 3 - definitions for orthodeoxia : a decline in
PaO2 of > 4% , of > 5% , or of > 10% 4 & 5% decline - derived from studies that
correlated a PaO2 with a measurable increase in shunt fraction
A decrease of > 10 mmHg in PaO2 commonly considered
20% to 80% in patients with HPS
Gomez FP, Martinez-Pali G, Barbera JA, et al. Gas exchange mechanism of orthodeoxia in hepatopulmonary syndrome. Hepatology 2004;40(3):660–6
Edell ES, Cortese DA, Krowka MJ, et al. Severe hypoxemia and liver disease. Am Rev Respir Dis 1989;140(6):1631–5.
![Page 11: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/11.jpg)
INVESTIGATIONS
• Determination of hypoxemia
• Pulse oximetry useful screening tool
cut off ≤ 97% has high sensitivity
• Specificity - PaO2 ≤ 70 mm Hg
less sensitive in mild HPS
• Arterial blood gas analysis reveal high alveolar-arterial
differences, more sensitive
• Abrams GA, Jaffe CC, Hoffer PB, Binder HJ, Fallon MB. Diagnostic utility of
contrast echocardiography and lung perfusion scan in patients with
hepatopulmonary syndrome. Gastroenterology 1995;109:1283-1288
![Page 12: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/12.jpg)
INVESTIGATIONS
• Determination of IPVD
• Contrast ECHO
• Lung perfusion scan using
macroaggregated albumin
![Page 13: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/13.jpg)
Contrast echocardiography
Agitated normal saline injected into peripheral vein
and cardiac chambers visualised through thoracic
echocardiography
Bubbles 25 mcm, vessels 5-8 mcm
Normally trapped in alveolar capillary bed
In presence of intracardiac right to left shunt bubbles
seen in left heart within 3 cycles
In case of intrapulmonary shunting seen after 3 cycles
![Page 14: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/14.jpg)
TRANSTHORACIC ECHOCARDIOGRAPHY Opacification of the RA and
RV with microbubbles and delayed opacification of the LA and LV approximately five cardiac cycles later.
Roberto et al. N Engl J Med 2008;358:2378-87.
![Page 15: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/15.jpg)
Lung perfusion scan using 99m Tc MAA
Peripheral venous injection of
MAA labelled with Tc 99m
Diameter of 10-90µm, removed
in normal pulmonary circulation
Detection of radioactivity in
fraction >6% in brain
![Page 16: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/16.jpg)
Lung perfusion scan using 99m Tc MAA
Measures shunt fraction
Highly specific but less sensitive -ve in most
patients with positive bubble contrast echo
Cannot differentiate between intracardiac shunts
![Page 17: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/17.jpg)
Other investigations
• CXR /HRCT- usually normal/ increased vascular
markings in lower zone
• PFT - reduced DLCO
• Pulmonary angiography Type 1 or minimal pattern
Finely diffuse, spidery abnormalities Severe hypoxemia and a response to 100% O2
The type 2 or discrete pattern Localized arteriovenous communications Poor response to supplemental oxygen
![Page 18: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/18.jpg)
DIAGNOSTIC CRITERIA
Rodríguez-Roisin et al. Eur Respir J 2004; 24: 861-880
![Page 19: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/19.jpg)
SCREENING ALGORITHM
Abrams GA, Sanders MK, Fallon MB: Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver
Transpl 2002; 8:391-6.
![Page 20: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/20.jpg)
TREATMENT OF HPS
Treatment
MedicalInterventiona
lradiology
Liver transplant
![Page 21: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/21.jpg)
TREATMENT PaO2 response to 100% O2 (> 550
mmHg) ventilation-perfusion mismatch or diffusion-
perfusion defect benefit clinically with this treatment
Poor response (PaO2 < 150 mmHg direct AV communications or extensive and
extremely vascular channels pulmonary angiography type 2 pattern
therapeutic embolization.
Liver Transplantation, Vol 6, No 4, Suppl 1 (July), 2000:pp S31-35
![Page 22: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/22.jpg)
MEDICAL - POTENTIAL TARGETS OF THERAPY
PTX: pentoxifylline, MB: methylene blue, MMF: mycophenolate mofetil, and CAPE: caffeic acid phenethyl ester Eshraghian et al. Biomed Res Int. 2013;2013:670139
![Page 23: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/23.jpg)
MEDICAL MANAGEMENT- HUMAN TRIALS
Small human trials of medical therapies- disappointing results
Pentoxifylline - small number of patients: failed to improve arterial oxygenation
Norfloxacin- failed to produce any improvement in gas exchange
Tried medications- aspirin, IV Methylene blue
Sani MN, Kianifar HR, Kianee A, Khatami G. Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. World J. Gastroenterol.2006; 12: 2427–31.
![Page 24: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/24.jpg)
INTERVENTIONAL RADIOLOGYTIPS- Few case reports, some showed benefit But majority- no benefitTIPS may worsen HPS by increasing the
hyperkinetic state more pulmonary vasodilatation, shunting, and hypoxemia
Intra-arterial coil embolization of pulmonary AV communications in patients with large shunts- Moderate improvement in hypoxemia
Krowka MJ. Hepatopulmonary syndrome: what are we learning from interventional radiology, liver transplantation, and other disorders? Gastroenterology1995; 109: 1009–13
![Page 25: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/25.jpg)
ROLE OF LIVER TRANSPLANTATION
Only effective treatment, complete resolution in
gas exchange abnormalities in 80% of patients
Exception of MELD points
HPS with PaO2 < 60 mm Hg liver Tx indication
Preoperative PaO2 ≤ 50 mm Hg & 99m Tc MAA
fraction > 20% - increased mortality
immediate post OLT (OR 2.21)
UNOS, United Network for Organ Sharing; Liver Transplantation, Vol 6, No 4, Suppl 1 (July), 2000:pp S31-35. Arguedas et al. Hepatology 2003;37:192-7
![Page 26: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/26.jpg)
‘NATURAL HISTORY OF HEPATOPULMONARY
SYNDROME: IMPACT OF LIVER TRANSPLANTATION. ‘
Observational study N= 57
29/37 (78 % ) with HPS who did not undergo OLT
& 5/24 patients (21 %) with HPS who underwent
OLT died over a period of 2 years
After OLT HPS had a five-year survival rate of 76 %
Not significantly different to those without HPS
Swanson KL et al. Natural history of hepatopulmonary syndrome: Impact of liver
transplantation. Hepatology 2005; 41:1122.
![Page 27: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/27.jpg)
RECOVERY AFTER LT
Recovery from HPS after Tx varies from days to 14 months
Post-OLT nonresolution of HPS uncommon (2%) Higher baseline macroaggregated albumin
shunt fraction - lower rate of postoperative improvement in oxygenation
Patients whose hypoxemia fails to improve- PPH
Aucejo, F, Miller, C, Vogt, D, et al. Pulmonary hypertension after liver transplantation in patients with antecedent hepatopulmonary syndrome: a report of 2 cases and review of the literature. Liver Transpl 2006; 12:1278
![Page 28: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/28.jpg)
PPH
PPH is defined as the development of PAH with m PAP > 25 mm Hg at rest or 30 mm Hg with exercise, in presence of PHTN
Moderate PPH (mPAP > 35 Hg) is associated with an increased operative risk for liver transplantation
![Page 29: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/29.jpg)
HPS PPHTNClinical Exam Cyanosis, clubbing No cyanosis
Clubbing less commonECG findings None RBBB, RAD, RV
hypertrophyABG Mod/severe hypoxemia Mild hypoxemia
Chest x-ray Normal Hilar enlargement
Contrast ECHO
Always + 3-6 cardiac cycles
-ve
99mTcMAA index
6% <6%
Pulmonary angiography
Normal/”spongy” (type I)Discrete AV Commns (II)
Large main PA, distal arterial pruning
OLT Always indicated in severe
Only indicated in mild stages
Medical Mx Ineffective Prostacyclin I2- Epoprostenol
![Page 30: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/30.jpg)
CIRRHOTIC CARDIOMYOPATHY(CC)
‘A sound heart is the life of the flesh…’
Proverbs 14:30
![Page 31: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/31.jpg)
DEFINITION
Clinical syndrome in cirrhosis Abnormal and blunted CV response
Physiological stress Pathological sress Pharmacologic stress
Normal / increased cardiac output and contractility at rest
Zardi et al JACC 2010
![Page 32: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/32.jpg)
INTRODUCTION
Gould - 1969 - cardiac contractile response to stimuli was depressed in alcoholic cirrhosis
Lee Et al- 1990- down Beta-adrenergic receptor density in cardiac cells in BDL rats
Multiple HD changes in cirrhosisSystemic Increase in plasma volume, non-central blood volume
and heart rate Decrease in central arterial blood volume and
systemic vascular resistance
![Page 33: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/33.jpg)
INTRODUCTION
Heart Increase in LAV, LVV and pulmonary blood flow
30-50% advanced cirrhosis show CC Up to 21% deaths post transplant
attributable to cardiac failure
Ripoll et al Transplantation 2008
Tiukinhoy- Laing et al AmJCardiol 2006
![Page 34: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/34.jpg)
PATHOGENESIS
![Page 35: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/35.jpg)
![Page 36: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/36.jpg)
MANIFESTATIONS
Diastolic dysfunction
Increased collagen contentIncreased ventricular stiffnessInadequate ventricular
relaxation
Pozzi et al Hepatology 1997Coutu et al Circ Res 2004Torregosa et al J Hepatol 2005
![Page 37: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/37.jpg)
MANIFESTATIONS
Systolic dysfunction Normal or increased function at rest Deteriorates on stress Prolonged total electromechanical systole Inotropic and chronotropic incompetence
On maximal exercise, cardiac output increases by 97% in cirrhosis: 300% increase in healthy controls
Limas et al Circulation 1974Zambruni et al J Hepatol 2006Pozzi et al Hepatol 1997
![Page 38: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/38.jpg)
EVIDENCE OF FUNCTIONAL AND STRUCTURAL CARDIAC ABNORMALITIES IN CIRRHOTIC PATIENTS WITH AND WITHOUT ASCITES
Pozzi et al. Hepatology1997;26:1131–7.
![Page 39: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/39.jpg)
PAPILLARY MUSCLE CONTRACTILITY IN CIRRHOTIC AND NON CIRRHOTIC RATS
N= 29
Gastroenterology 1996
![Page 40: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/40.jpg)
MANIFESTATIONS
Electrophysiological changes QT prolongation (>0.44 sec) Multiple extra-systoles BBB ST depression Electromechanical dyssynergia
Bernardi et al hepatology 1998Henriksen et al J hepatol 2002
![Page 41: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/41.jpg)
SERUM MARKERS
Cardiac troponin I and ANP/BNP elevated Troponin I level elevated in about 1/3 of cirrhotic
patients BNP levels correlate with QT interval
prolongation, interventricular septal thickness, and impairment of diastolic function
Pateron D et al. Elevated circulating cardiac troponin I in patients with cirrhosis. Hepatology1999; 29: 640-3.
Wong F, Siu S, Liu P, Blendis LM. BNP : is it a predictor of cardiomyopathy in cirrhosis? Clin Sci2001; 101: 621-628.
![Page 42: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/42.jpg)
‘CIRRHOTIC CARDIOMYOPATHY: AN OVERALL ASSESSMENT AND ROLE OF NT-PROBNP’
Aim: To evaluate levels of NTproBNP and its relationship with CC
N= 100 cirrhotic patients & 25 controls Cirrhotics: LV mass, E wave velocity- increased LV diastolic function- decreased NT-proBNP higher (1551 pg/ml vs. 856 pg/ml; p < 0.05)
o 26% of cirrhotic had NT-proBNP levels > 2000 pg/ml- consistent with CHF
o Regression analysis, NT-proBNP significantly related to CTP score, LV mass and cardiac index (β= 0.299, 0.232, 0.243 respectively,p < 0.05)
AASLD Abstracts 2013
![Page 43: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/43.jpg)
DIAGNOSTIC CRITERIA
Systolic dysfunction: Blunted increase in CO with
exercise, volume challenge OR pharmacological stimuli;
resting LVEF <55%
Diastolic dysfunction: prolonged deceleration time
(>200 ms), E:A ratio <1
Supportive criteria
EPS abnormalities- abnormal chronotropic response; prolonged QTc
Enlarged LA ; increased myocardial mass; increased BNP and proBNP,
troponin I levels
2005 WGO cirrhotic cardiomyopathy criteriaCardiovascular complications of cirrhosis. Gut 57, 268–278. 2008
![Page 44: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/44.jpg)
CLINICAL IMPLICATIONS
![Page 45: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/45.jpg)
HRS AND CC
Impaired cardiac function may predispose patients to HRS
Especially in stressful conditions In one study
23 patients with SBP, all cleared infection – 8 developed HRS
Lower CO at admission and decreased with resolution of infection
MAP was low in those who developed renal failure
Inadequate ventricular contractility in the face of the CV-Renal stresses imposed by sepsis may contribute to HRSRuiz del Arbol et al Hepatology 2003
![Page 46: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/46.jpg)
HRS AND CC
In another study 24 patients with cirrhosis and ascites 8 with low CI <1.5 GFR was low 39 Vs 63 Creatinine higher 1.3 vs 0.78 HRS increased 3/7 Vs 1/16 Worse survival at 3, 6, 12 months
Krag et al Gut 2010
![Page 47: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/47.jpg)
TIPS AND CARDIOMYOPATHY
CCF is an absolute contraindication for TIPS Worsening of the hyperdynamic circulation,
manifested by an acute increase in CO and a decrease in the SVR
In one study 32 patients undergoing TIPS Day 28 E/A ratio independent predictor of death
at one year 6/10 with E/A <1 died 0/22 with E/A >1
Cazzaniga et al Gut 2007Huonker et al Gut 1999
![Page 48: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/48.jpg)
‘TIPS VERSUS PARACENTESIS PLUS ALBUMIN FOR REFRACTORY ASCITES IN CIRRHOSIS...’
Gines et alRCT N= 70CHF was reported in 12% of the TIPS group
Not seen in the paracentesis group
Gines P et al.(2002) TIPS versus paracentesis plus albumin for refractory ascites in cirrhosis. Gastroenterology 123:1839–184
![Page 49: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/49.jpg)
LIVER TRANSPLANT AND CC
OLT-severe stress on CVSIntra & Post OP CO compromised due to reduced preload or to impaired myocardial contractility
Cardiac failure cause of 7-21% deaths after OLT
Ripoll et al Transplantation 2008
Tiukinhoy- Laing et al AmJCardiol 2006Torregosa et al J Hepatol 2005Moller et al Post Grad Med 2009
![Page 50: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/50.jpg)
LIVER TRANSPLANT AND CC
Prospective study N=190 patients with ESLD 71 - OLT During the hospitalization period after
transplantationChest radiographic evidence of pulmonary
edema in 39 patients (56%) Overt LVF in 4 patients (6%)All the patients had no prior evidence of cardiac
illness
Donovan CL et al 1996 Transplantation 61:1180–1188
![Page 51: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/51.jpg)
‘CARDIAC ALTERATIONS IN CIRRHOSIS: REVERSIBILITY AFTER LIVER TRANSPLANTATION’
N=40 Echocardiography and radionuclide angiography Complete reversal of all abnormal
cardiovascular parameters CV function reverted to normal when studied an
average of 9 months after OLT Improved cardiac workload and exercise
capacity
Torregrosa , et al. Cardiac alterations in cirrhosis: reversibility after liver transplantation. J
Hepatol 2005; 42: 68–74.
![Page 52: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/52.jpg)
MANAGEMENT
![Page 53: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/53.jpg)
TREATMENT - GENERAL
Overt CHF is an uncommon – low afterload
Basic principles in Mx of CCFCorrect dyselectrolytemiaMaintain volume statusAvoid toxins like alcohol and cardio-depressant
drugsCareful use of diuretics
![Page 54: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/54.jpg)
TREATMENT - SPECIFIC
Beta agonist- Dopamine: Probably ineffective Amrinone which inhibits cAMP degradation might be
useful Ouabain, a short acting cardiac glycoside- ineffective Propranolol improved the prolonged
electrocardiographic QT interval in cirrhotic patients
24 weeks of treatment using an aldosterone receptor antagonist: Impvt in left ventricular wall thickness, peripheral sympathetic activation, and showed a nonsignificant tendency to improve the diastolic dysfunction
Ma et al Hepatol 1997Wong et al Hepatol 2002
![Page 55: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/55.jpg)
TREATMENT - SPECIFIC
Liver transplantNormalisation of QTcImprovement in cardiac
functionsDisappearance of LVHNormalisation of exercise
capacityRipoll et al Transplantation 2008
![Page 56: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/56.jpg)
CONCLUSIONS
HPS and CCM both are under recognised conditions
HPS: progressive hypoxemia even in the absence of deteriorating hepatic function Tx
CCM & HPS: Affect outcome after TIPS & liver transplant
Both are reversible after OLT Knowledge in pathogenesis- hope of effective
medical treatment
![Page 57: H EPATOPULMONARY SYNDROME AND CIRRHOTIC CARDIOMYOPATHY Perceptor: Dr Shalimar](https://reader035.vdocuments.net/reader035/viewer/2022062321/56649e295503460f94b16be7/html5/thumbnails/57.jpg)
THANK YOU