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Current concepts
This document discusses hepatopulmonary syndrome (HPS), a condition characterized by liver disease, hypoxemia, and pulmonary vascular dilatations. HPS causes hypoxemia ranging from 5-20% and is most commonly associated with cirrhosis. Orthotopic liver transplantation is currently the only effective treatment for HPS as it resolves the hypoxemia. Screening for HPS with arterial blood gases is recommended for patients with chronic liver disease reporting dyspnea.
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Current concepts
- 2. Hepatopulmonary Syndrome — ALiver-Induced Lung Vascular Disorder Dr Hafeez Yaqoob PGR Gastroenterology BMCH Quetta
- 4. Hepatopulmonary Syndrome • Triadof liver disease, hypoxemia, and pulmonary vascular dilatations • Hypoxemia caused by hepatopulmonary syndrome ranges from 5 to 20% • Most commonly associated with cirrhosis but also reported in noncirrhotic portal hypertension • No consistent relationship between hepatic dysfunction and Child-Pugh classification with severity of hypoxemia or shunt
- 5. Case of Ms.AM: 54 y/o female with worsening dyspnea on exertion, platypnea, and fatigue • Past medical history includes Hepatitis C cirrhosis diagnosed 4 years ago • Physical exam was significant for tachypnea, clubbing, a spider nevi on the thorax, and splenomegaly • Arterial blood gas revealed hypoxemia with a PaO2 of 43 mmHg and patient was placed on 100% oxygen • CXR was normal and CT Angiogram revealed possible dilated peripheral pulmonary vessels but no pulmonary embolus
- 6. • Liver-Related Causesof Dyspnea in a Patient with Chronic Liver Disease?
- 7. Liver-Related Causes ofDyspnea in a Patient with Chronic Liver Disease Pulmonary - Parenchymal • Alveolar – Aspiration pneumonia – Basal atelectasis • Interstitial lung disease – Lymphocytic interstitial pneumonia – Fibrosing alveolitis – Noncardiogenic pulmonary edema • Vascular – Pulmonary hemorrhage – HPS – PPHTN • Extraparenchymal – Pleural effusions – Restriction from tense ascites • Extrapulmonary – Cirrhotic cardiomyopathy – Cirrhotic myopathy – Chronotropic dysfunction – Muscle wasting – Deconditioning from impaired mobility
- 9. Liver Lung Interaction LiverFailure Acute Liver Failure Chronic Liver Failure ARDS Vasodilatation Vasoconstriction HPS Portopulm HTN
- 11. Clinical Manifestations • Dyspnea •Platypnea • Orthodeoxia • Clubbing • Liver dysfunction • Spider nevi • Elevated Cardiac Output • Decreased SVR and PVR • Narrowed A-V O2 difference Pathogenesis • V/Q mismatch • Intrapulmonary shunting • Limitation of oxygen diffusion • Failure to clear and production of circulating vasodilators by damaged liver • Inhibition of vasoconstrictive substance by damaged liver
- 20. Hypothesis of PulmonaryVessel Dilatation in Hepatopulmonary Syndrome
- 21. Pathophysiology of Hypoxemiain HPS Ramsay MA. Int Anesthesiol Clin. 2006 Summer;44(3):69-82
- 23. Pathophysiology of Hypoxemiain HPS Hoeper MM et al. Lancet 2004 May 1;363(9419):1461-8
- 31. Diagnostic Criteria for HepatopulmonarySyndrome • Portal hypertension with or without cirrhotic liver disease • Arterial hypoxemia PaO2 < 70 mmHg or PA-a, O2 15 mmHg • Pulmonary vascular dilatation demonstrated by Delayed “positive” contrast enhanced transthoracic echocardiography or Abnormal brain uptake (>6%) after 99mTcMAA lung perfusion scanning
- 32. Diagnostic criteria forthe HPS
- 33. Contrast-enhanced Echocardiography • IVadministration of hand-agitated normal saline (using 3-way stop cock) • Microbubbles average 10 to 20 microns (normal capillary is 8 microns) • Diffuse dilatations allows passage of microbubbles within 3 to 6 cardiac cycles • Right-to-left intracardiac shunt if within 3 cardiac cycles • Transesophageal echocardiography further distinguishes intracardiac and intrapulmonary shunting
- 39. 99mTcMAA Lung PerfusionScanning • Peripheral injection of 99mTcMAA • Aggregates are 20 to 90 microns • Demonstration of abnormal uptake over the brain (>6%) • Does not distinguish between intracardiac and intrapulmonary shunts • May offer complementary information for stratification of HPS patients at greater risk of OLT mortality
- 40.
- 46. Management of Hepatopulmonary Syndrome •Currently, no effective medical therapies for the hepatopulmonary syndrome exist, and liver transplantation is the only successful treatment.
- 47. Management of Hepatopulmonary Syndrome Pharmacological Treatment •Somatostatin analogue • B-blockers • Cyclooxygenase inhibitor • Glucocorticoids • NO inhibitors • Immunosuppressors • Vasoconstrictors • Antimicrobials • Garlic preparation Nonpharmacological Treatment • Long term oxygen therapy • Transjugular intrahepatic portosystemic shunts • Embolization • Orthotopic Liver Transplantation
- 48. The MELD Score: Modelfor End-Stage Liver Disease • Determines priority for Orthotopic Liver Transplant • Uses the following formula: 3.8 x log (e) (bilirubin mg/dL) + 11.2 x log (e) (INR) + 9.6 log (e) (creatinine mg/dL) • Scores range from 6 to 40 • Score can be increased if PaO2 < 60 in patient with Hepatopulmonary Syndrome
- 49. No HPS Negative CEE Follow-up PaO2>60 - <80 mmHg (and/or) PA-aO2 > 15mmHg OLT PaO2 >50 - <60 mmHg OLT High risk for post-op mortality PaO2 <50 mmHg MAA >20% PositiveCEE + PFTs + HRCT CEE PaO2 < 80 mmHg (and/or) PA-aO2 > 15 mmHg No HPS PaO2 > 80 Arterial Blood Gases OLT candidates Hepatic disease patients with dyspnea
- 51. Orthotopic transplant • Anorthotopic transplant is the most common type of liver transplant. The whole liver is taken from a recently deceased donor. This is usually from a donor who has pledged his or her organs for donation prior to death and has not transmissible illness or cancers that may be transmitted to the recipient.
- 52. Transjugular intrahepatic portosystemic shunt •TIPS • reduce portal pressure in patients with the hepatopulmonary syndrome. However, the limited available data, along with the risk of exacerbating the hyperkinetic circulatory state, thereby enhancing pulmonary vasodilatation and increasing the severity of the hepatopulmonary syndrome, do not provide support for its use as a palliative strategy.
- 53. Prognosis • patients withthe hepatopulmonary syndrome had a 5-year survival rate of 76% after liver transplantation, a rate not significantly different from that among patients without the hepatopulmonary syndrome who underwent transplantation.
- 54. summary, • In summary,screening for the hepatopulmonary syndrome with the use of arterial blood gases is recommended in patients with chronic liver disease who report dyspnea or who are candidates for liver transplantation. • Future research should address the genetic polymorphisms associated with the hepatopulmonary syndrome, circulating factors emanating from the hepatic veins that may affect the pulmonary vascular tone, and angiogenic factors (including, among the most relevant factors, endothelin-1, vascular endothelial growth factor, and platelet-derived growth factor)
- 55. summary • patients withthe hepatopulmonary syndrome who undergo liver transplantation should be examined for biomarker sentinel clinical correlates that could lead to effective medical interventions.
- 56. summary, Finally, the questionof which patients with the hepatopulmonary syndrome should receive a high priority for liver transplantation should be answered on the basis of long-term outcomes of transplantation in patients with various degrees of severity of the syndrome and various causes of liver disease.
- 57. Take Home Massage •Diagnose HPS early, transplant early • Pulse oximetry and Arterial blood gas to screen for HPS • Annual screening echocardiography for patients on the OLT waiting list to evaluate for HPS . • Close monitoring of patients after OLT because recurrence of HPS and conversion to PPHTN have been reported.
- 58. Take home Massage •Contrast-enhanced transthoracic echocardiography with saline (shaken to produce microbubbles >10 μm in diameter) is the most practical method to detect pulmonary vascular dilatation. • LT is the treatment of choice in HPS .
- 60. I can breathbetter nowthat I got a new liver!