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• W2169575861 abstract "A vast spectrum of pulmonary pathologic conditions occurs in association with chronic liver diseases, and clinically important manifestations, such as arterial hypoxemia, can result. Both pulmonary vascular and parenchymal abnormalities can contribute to the dysfunction, as evidenced by results of pulmonary function tests and gas exchange studies. The clinical implications of identifying such pulmonary problems range from alleviation of symptoms, especially dyspnea, to comprehensive assessment of patients before and after liver transplantation. Physicians should be aware of these potential pulmonary disorders that can complicate liver disease and liver transplantation so that management of affected patients can be improved. A vast spectrum of pulmonary pathologic conditions occurs in association with chronic liver diseases, and clinically important manifestations, such as arterial hypoxemia, can result. Both pulmonary vascular and parenchymal abnormalities can contribute to the dysfunction, as evidenced by results of pulmonary function tests and gas exchange studies. The clinical implications of identifying such pulmonary problems range from alleviation of symptoms, especially dyspnea, to comprehensive assessment of patients before and after liver transplantation. Physicians should be aware of these potential pulmonary disorders that can complicate liver disease and liver transplantation so that management of affected patients can be improved. The consequences of chronic liver disease include not only cirrhosis, portal hypertension, and ascites but pulmonary, hematologic, renal, and neurologic complications as well.1Williams R Maddrey WC Liver. Butterworths, Boston1984Google Scholar, 2Sherlock S Diseases of the Liver and Biliary System. Sixth edition. Blackwell Scientific Publications, Oxford1981Google Scholar Major noninfectious hepatic diseases and their associated pulmonary abnormalities are listed in Table 1. In this review, we had the following four goals: to summarize the reported pulmonary pathophysiologic features of these liver diseases, to discuss the interpretation of pulmonary function tests in patients with liver disease, to describe hepatopulmonary drug interactions, and to address the preoperative and postoperative pulmonary implications for liver transplantation candidates.Table 1Pulmonary Abnormalities Associated With Selected Chronic Liver DiseasesLiver disorderPathologic or pathophysiologic pulmonary findingsCirrhosis*Alcoholic, cryptogenic, and postnecrotic cirrhosis.Intrapulmonary shuntingPortopulmonary shuntingPleural shuntingPulmonary hypertensionPleural effusionsImpaired hypoxic vasoconstrictionVentilation-perfusion mismatchPrimary biliary cirrhosisThoracic deformitiesIntrapulmonary granulomasLymphocytic interstitial pneumonitisInterstitial pneumonitis (usual and desquamative)Obstructive airways disease (Sjögren's syndrome)Chronic active hepatitisInterstitial pneumonitis (nonspecific)Pleuritis and pleural effusionsα1-Antitrypsin deficiencyObstructive airways diseaseIntrapulmonary shuntingPrimary sclerosing cholangitisSuppurative bronchitis (?)Bronchiectasis (?)* Alcoholic, cryptogenic, and postnecrotic cirrhosis. Open table in a new tab Cirrhosis is the most prevalent type of chronic liver disease in the United States; it is essentially an irreversible process of fibrosis and nodular regeneration of the liver parenchyma.1Williams R Maddrey WC Liver. Butterworths, Boston1984Google Scholar The literature reviewed in this section refers mainly to patients with alcoholic cirrhosis and less commonly to those with cryptogenic or postnecrotic cirrhosis. Approximately 15 to 45% of patients with cirrhosis have one or more of the following abnormalities: arterial hypoxemia (Pao2 less than 80 mm Hg) in conjunction with hemoglobin desaturation (related to hypoxemia or increased methemoglobin [Krowka MJ, Cortese DA: Unpublished data]),3Flückiger M Vorkommen von trommelschlägelförmigen Fingerendphalangen ohne chronische Veränderungen an den Lungen oder am Herzen.Wien Med Wochenschr. 1884; 34: 1457Google Scholar, 4Snell AM The effects of chronic disease of the liver on the composition and physicochemical properties of blood: changes in the serum proteins; reduction in the oxygen saturation of the arterial blood.Ann Intern Med. 1935; 9: 690-711Crossref Google Scholar, 5Keys A Snell AM Respiratory properties of the arterial blood in normal man and in patients with disease of the liver: position of the oxygen dissociation curve.J Clin Invest. 1938; 17: 59-67Crossref PubMed Google Scholar, 6Rodman T Sobel M Close HP Arterial oxygen unsaturation and the ventilation-perfusion defect of Laënnec's cirrhosis.N Engl J Med. 1960; 263: 73-77Crossref PubMed Google Scholar, 7Heinemann HO Emirgil C Mijnssen JP Hyperventilation and arterial hypoxemia in cirrhosis of the liver.Am J Med. 1960; 28: 239-246Abstract PubMed Google Scholar, 8Abelmann WH Kramer GE Verstraeten JM Gravallese Jr, MA McNeely WF Cirrhosis of the liver and decreased arterial oxygen saturation.Arch Intern Med. 1961; 108: 34-40Crossref PubMed Google Scholar, 9Williams Jr, MH Hypoxemia due to venous admixture in cirrhosis of the liver.J Appl Physiol. 1960; 15: 253-254PubMed Google Scholar, 10Mellemgaard K Winkler K Tygstrup N Georg J Sources of venoarterial admixture in portal hypertension.J Clin Invest. 1963; 42: 1399-1405Crossref PubMed Google Scholar, 11Bashour FA Cochran P Alveolar-arterial oxygen tension gradients in cirrhosis of the liver: further evidence of existing pulmonary arteriovenous shunting.Am Heart J. 1966; 71: 734-740Abstract PubMed Google Scholar, 12Martini GA Baltzer G Arndt H Some aspects of circulatory disturbances in cirrhosis of the liver.Prog Liver Dis. 1972; 4: 231-250PubMed Google Scholar, 13Georg J Mellemgaard K Tygstrup N Winkler K Venoarterial shunts in cirrhosis of the liver.Lancet. 1960; 1: 852-854Abstract PubMed Google Scholar, 14Silverman A Cooper MD Moller JH Good RA Syndrome of cyanosis, digital clubbing, and hepatic disease in siblings.J Pediatr. 1968; 72: 70-80Abstract PubMed Google Scholar, 15Furukawa T Hara N Yasumoto K Inokuchi K Arterial hypoxemia in patients with hepatic cirrhosis.Am J Med Sci. 1984; 287: 10-13Crossref PubMed Google Scholar clubbing,3Flückiger M Vorkommen von trommelschlägelförmigen Fingerendphalangen ohne chronische Veränderungen an den Lungen oder am Herzen.Wien Med Wochenschr. 1884; 34: 1457Google Scholar, 12Martini GA Baltzer G Arndt H Some aspects of circulatory disturbances in cirrhosis of the liver.Prog Liver Dis. 1972; 4: 231-250PubMed Google Scholar, 16Stein H Stein S Digital clubbing in cirrhosis of the liver.Lancet. 1961; 2: 999-1000Abstract PubMed Google Scholar and hyperventilation.7Heinemann HO Emirgil C Mijnssen JP Hyperventilation and arterial hypoxemia in cirrhosis of the liver.Am J Med. 1960; 28: 239-246Abstract PubMed Google Scholar, 17Vanamee P Poppell JW Glicksman AS Randall HT Roberts KE Respiratory alkalosis in hepatic coma.Arch Intern Med. 1956; 97: 762-767Crossref Google Scholar, 18Karetzky MS Mithoefer JC The cause of hyperventilation and arterial hypoxia in patients with cirrhosis of the liver.Am J Med Sci. 1967; 254: 797-804Crossref PubMed Google Scholar, 19Rodman T Hurwitz JK Pastor BH Close HP Cyanosis, clubbing and arterial oxygen unsaturation associated with Laennec's cirrhosis.Am J Med Sci. 1959; 238: 534-541Crossref PubMed Google Scholar, 20Tyor MP Sieker HO Biochemical, blood gas and peripheral circulatory alterations in hepatic coma.Am J Med. 1959; 27: 50-59Abstract PubMed Google Scholar Clinically, the hypoxemia and subsequent hemoglobin desaturation predominantly reflect a spectrum of pulmonary vascular disorders rather than a parenchymal process or a hemoglobin dissociation curve shift (to the right), as was initially hypothesized.5Keys A Snell AM Respiratory properties of the arterial blood in normal man and in patients with disease of the liver: position of the oxygen dissociation curve.J Clin Invest. 1938; 17: 59-67Crossref PubMed Google Scholar, 7Heinemann HO Emirgil C Mijnssen JP Hyperventilation and arterial hypoxemia in cirrhosis of the liver.Am J Med. 1960; 28: 239-246Abstract PubMed Google Scholar, 21Caldwell PRB Fritts Jr, HW Cournand A Oxyhemoglobin dissociation curve in liver disease.J Appl Physiol. 1965; 20: 316-320Google Scholar The pulmonary vascular abnormalities include intrapulmonary shunting, portopulmonary shunting, pleural shunting, pulmonary hypertension, impaired hypoxic pulmonary vasoconstriction, and ventilation-perfusion mismatching (Table 1). Pulmonary parenchymal diseases that reduce gas transport are unusual in patients with alcoholic cirrhosis and are more commonly associated with the other hepatic diseases that are listed in Table 1. Intrapulmonary shunting (Table 2), first described in a case report by Rydell and Hoffbauer22Rydell R Hoffbauer FW Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis.Am J Med. 1956; 21: 450-460Abstract PubMed Google Scholar in 1956, refers to the entry of systemic venous blood into the arterial circulation without exposure to alveolar oxygen.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar It can occur by means of three mechanisms: (1) venous blood flowing through nonventilated alveoli, (2) anatomic communications between pulmonary arteries and veins and thus bypassing the capillary-alveoli interfaces, and (3) dilated capillary and precapillary beds in which diffused oxygen ineffectively reaches the midstream deoxygenated hemoglobin molecules.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar, 24Davis II, HH Schwartz DJ Lefrak SS Susman N Schainker BA Alveolar-capillary oxygen disequilibrium in hepatic cirrhosis.Chest. 1978; 73: 507-511Crossref PubMed Google Scholar, 25Robin ED Laman D Horn BR Theodore J Platypnea related to orthodeoxia caused by true vascular lung shunts.N Engl J Med. 1976; 294: 941-943Crossref PubMed Google Scholar Shunts in patients with liver disease, which have been quantified by using alveolar-arterial oxygen tension gradients, can reach a considerable proportion (20 to 70%) of the cardiac output.10Mellemgaard K Winkler K Tygstrup N Georg J Sources of venoarterial admixture in portal hypertension.J Clin Invest. 1963; 42: 1399-1405Crossref PubMed Google Scholar, 11Bashour FA Cochran P Alveolar-arterial oxygen tension gradients in cirrhosis of the liver: further evidence of existing pulmonary arteriovenous shunting.Am Heart J. 1966; 71: 734-740Abstract PubMed Google Scholar, 13Georg J Mellemgaard K Tygstrup N Winkler K Venoarterial shunts in cirrhosis of the liver.Lancet. 1960; 1: 852-854Abstract PubMed Google Scholar, 24Davis II, HH Schwartz DJ Lefrak SS Susman N Schainker BA Alveolar-capillary oxygen disequilibrium in hepatic cirrhosis.Chest. 1978; 73: 507-511Crossref PubMed Google Scholar, 26Wolfe JD Tashkin DP Holly FE Brachman MB Genovesi MG Hypoxemia of cirrhosis: detection of abnormal small pulmonary vascular channels by a quantitative radionuclide method.Am J Med. 1977; 63: 746-754Abstract PubMed Google Scholar, 27Chiesa A Ciappi G Balbi L Chiandussi L Role of various causes of arterial desaturation in liver cirrhosis.Clin Sci. 1969; 37: 803-814PubMed Google Scholar Shunts may also become worse with exercise28Kennedy TC Knudson RJ Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis.Chest. 1977; 72: 305-309Crossref PubMed Google Scholar and, by definition, should not respond substantially to 100% oxygen therapy.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar Supplemental oxygen, however, may be beneficial in the case of dilated capillary beds; increased driving pressures of alveolar oxygen may increase oxygen diffusion and improve hemoglobin saturation.Table 2Intrapulmonary Shunting Associated With Chronic Liver Disease: Clinical ConsiderationsProbably is the most important mechanism of severe hypoxemia in patients with cirrhosisCan exist in the absence of portal hypertensionRadiographically, resembles bibasilar interstitial infiltrates on chest roentgenogramVascular pathologic changes are predominantly in lung basesPremortem diagnosis is confirmed by 99mTcMAA whole-body scanning or indocyanine green dye echocardiography*99mTcMAA = technetium-labeled macroaggregates of albumin.Is not reversed by liver transplantationMay be associated with orthodeoxia and platypnea* 99mTcMAA = technetium-labeled macroaggregates of albumin. Open table in a new tab Recently, additional quantification of intrapulmonary shunting has been provided by whole-body radionuclide scanning with technetium-labeled macroaggregates of albumin.26Wolfe JD Tashkin DP Holly FE Brachman MB Genovesi MG Hypoxemia of cirrhosis: detection of abnormal small pulmonary vascular channels by a quantitative radionuclide method.Am J Med. 1977; 63: 746-754Abstract PubMed Google Scholar, 29Bank ER Thrall JH Dantzker DR Radionuclide demonstration of intrapulmonary shunting in cirrhosis.AJR. 1983; 140: 967-969Crossref PubMed Google Scholar, 30Keren G Boichis H Zwas TS Frand M Pulmonary arteriovenous fistulae in hepatic cirrhosis.Arch Dis Child. 1983; 58: 302-304Crossref PubMed Google Scholar, 31Genovesi MG Tierney DF Taplin GV Eisenberg H An intravenous radionuclide method to evaluate hypoxemia caused by abnormal alveolar vessels: limitation of conventional techniques.Am Rev Respir Dis. 1976; 114: 59-65PubMed Google Scholar, 32Sang Oh K Bender TM Bowen A Ledesma-Medina J Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia.Pediatr Radiol. 1983; 13: 111-115Crossref PubMed Google Scholar, 33Seward JB Hayes DL Smith HC Williams DE Rosenow III, EC Reeder GS Piehler JM Tajik AJ Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases.Mayo Clin Proc. 1984; 59: 221-231Abstract PubMed Google Scholar These particles, which range from 20 to 50 μm in diameter, are trapped in normal capillaries, which maintain diameters of 8 to 15 μm.26Wolfe JD Tashkin DP Holly FE Brachman MB Genovesi MG Hypoxemia of cirrhosis: detection of abnormal small pulmonary vascular channels by a quantitative radionuclide method.Am J Med. 1977; 63: 746-754Abstract PubMed Google Scholar By demonstrating the accumulation of technetium in extrapulmonary circulations with whole-body scanning, right-to-left shunts can be quantified.29Bank ER Thrall JH Dantzker DR Radionuclide demonstration of intrapulmonary shunting in cirrhosis.AJR. 1983; 140: 967-969Crossref PubMed Google Scholar Contrast two-dimensional echocardiography with intravenous injection of isotonic saline or indocyanine green dye can also be used to detect intrapulmonary shunting that may not be demonstrated by conventional angiography.34Shub C Tajik AJ Seward JB Dines DE Detecting intrapulmonary right-to-left shunt with contrast echocardiography: observations in a patient with diffuse pulmonary arteriovenous fistulas.Mayo Clin Proc. 1976; 51: 81-84PubMed Google Scholar, 35Schomerus H Buchta I Arndt H Pulmonary function studies and oxygen transfer in patients with liver cirrhosis and different degree of portasystemic encephalopathy.Respiration. 1975; 32: 1-20Crossref PubMed Google Scholar Qualitative evidence of these shunts has been shown by infusions of micropaque gelatin36Berthelot P Walker JG Sherlock S Reid L Arterial changes in the lungs in cirrhosis of the liver: lung spider nevi.N Engl J Med. 1966; 274: 291-298Crossref PubMed Google Scholar and vinyl acetate22Rydell R Hoffbauer FW Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis.Am J Med. 1956; 21: 450-460Abstract PubMed Google Scholar into the pulmonary vascular tree at autopsy. In addition, numerous angiographic reports have described a characteristic “spongy” appearance of the basilar pulmonary vessels, corresponding to the basilar infiltrates seen on chest roentgenograms in patients with chronic liver disease.29Bank ER Thrall JH Dantzker DR Radionuclide demonstration of intrapulmonary shunting in cirrhosis.AJR. 1983; 140: 967-969Crossref PubMed Google Scholar, 32Sang Oh K Bender TM Bowen A Ledesma-Medina J Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia.Pediatr Radiol. 1983; 13: 111-115Crossref PubMed Google Scholar, 37El Gamal M Stoker JB Spiers EM Whitaker W Cyanosis complicating hepatic cirrhosis: report of a case due to multiple pulmonary arteriovenous fistulas.Am J Cardiol. 1970; 25: 490-494Abstract PubMed Google Scholar, 38Stanley NN Ackrill P Wood J Lung perfusion scanning in hepatic cirrhosis.Br Med J. 1972; 4: 639-643Crossref PubMed Google Scholar, 39Stanley NN Woodgate DJ Mottled chest radiograph and gas transfer defect in chronic liver disease.Thorax. 1972; 27: 315-323Crossref PubMed Google Scholar Clinically, intrapulmonary shunts have been associated with a debilitating condition characterized by orthodeoxia (arterial deoxygenation accentuated in the upright position and reversed in recumbency) and platypnea (dyspnea induced in the upright position and relieved by recumbency) in up to 5% of patients with cirrhosis.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar, 25Robin ED Laman D Horn BR Theodore J Platypnea related to orthodeoxia caused by true vascular lung shunts.N Engl J Med. 1976; 294: 941-943Crossref PubMed Google Scholar, 28Kennedy TC Knudson RJ Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis.Chest. 1977; 72: 305-309Crossref PubMed Google Scholar Orthodeoxia and platypnea have usually been associated with classic right-to-left shunts (Table 3). Robin and associates25Robin ED Laman D Horn BR Theodore J Platypnea related to orthodeoxia caused by true vascular lung shunts.N Engl J Med. 1976; 294: 941-943Crossref PubMed Google Scholar first described this entity in two patients with chronic liver disease (alcoholic cirrhosis in one and unspecified in the other) in whom the Pao2 decreased 19 and 14 mm Hg, respectively, from the supine to the seated position and the Paco2 changed only minimally. In the supine position, both patients had significant hypoxemia (Pao2 less than 60 mm Hg). Calculated shunts were 51 and 33% of the cardiac output, respectively, measured with the patients in the seated position. Because the vascular shunts predominate in the lung bases, redistribution of blood to the lung bases in the upright positions may explain the increased shunting in these positions. In addition to hypoxemia and oxygenation change with position, up to 20% of patients with cirrhosis may have an appreciable abnormality in diffusing capacity, which can be erroneously attributed to interstitial lung disease.39Stanley NN Woodgate DJ Mottled chest radiograph and gas transfer defect in chronic liver disease.Thorax. 1972; 27: 315-323Crossref PubMed Google Scholar, 42Golding PL Smith M Williams R Multisystem involvement in chronic liver disease: studies on the incidence and pathogenesis.Am J Med. 1973; 55: 772-782Abstract PubMed Google Scholar Even after correction for anemias, low diffusing capacities have been found in patients with liver disease. Thus, in the absence of interstitial disease, some investigators have speculated that some type of abnormality of pulmonary blood flow or gas exchange exists.39Stanley NN Woodgate DJ Mottled chest radiograph and gas transfer defect in chronic liver disease.Thorax. 1972; 27: 315-323Crossref PubMed Google ScholarTable 3Differential Diagnosis of Orthodeoxia and PlatypneaDisorderMechanismChronic liver disease (predominantly cirrhosis)Increased blood flow to the lung bases (attributable to gravitational change with upright posture), resulting in greater perfusion of shunt vesselsIntracardiac shunts (patent foramen ovale or atrial septal defect)33Seward JB Hayes DL Smith HC Williams DE Rosenow III, EC Reeder GS Piehler JM Tajik AJ Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases.Mayo Clin Proc. 1984; 59: 221-231Abstract PubMed Google ScholarSpeculative: a combination of (1) increased right-sided cardiac pressures causing a gradient of blood flow between the right and left atria which is dependent on posture and (2) a “streaming” effect of blood from the inferior vena cava into the left atrium. Latter factor is induced by upright posture, which causes septal shifts, enlarges the foramen ovale, and results in increased blood flow without pressure gradients After pneumonectomy40Franco DP Kinasewitz GT Markham RV Tucker WY George RB Postural hypoxemia in the postpneumonectomy patient.Am Rev Respir Dis. 1984; 129: 1021-1022PubMed Google Scholar After pulmonary embolusChronic obstructive pulmonary disease41Miller WC Heard JG Unger KM Enlarged pulmonary arteriovenous vessels in COPD: another possible mechanism of hypoxemia.Chest. 1984; 86: 704-706Crossref PubMed Google ScholarSame as for chronic liver diseasePulmonary arteriovenous malformations33Seward JB Hayes DL Smith HC Williams DE Rosenow III, EC Reeder GS Piehler JM Tajik AJ Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases.Mayo Clin Proc. 1984; 59: 221-231Abstract PubMed Google ScholarSame as for chronic liver disease Open table in a new tab Intrapulmonary shunting does not seem to be associated with any of the following factors: abnormal results of liver function tests, ascites, splenomegaly, portal or pulmonary hypertension, specific types of liver disease, or digital clubbing.7Heinemann HO Emirgil C Mijnssen JP Hyperventilation and arterial hypoxemia in cirrhosis of the liver.Am J Med. 1960; 28: 239-246Abstract PubMed Google Scholar, 10Mellemgaard K Winkler K Tygstrup N Georg J Sources of venoarterial admixture in portal hypertension.J Clin Invest. 1963; 42: 1399-1405Crossref PubMed Google Scholar, 12Martini GA Baltzer G Arndt H Some aspects of circulatory disturbances in cirrhosis of the liver.Prog Liver Dis. 1972; 4: 231-250PubMed Google Scholar, 13Georg J Mellemgaard K Tygstrup N Winkler K Venoarterial shunts in cirrhosis of the liver.Lancet. 1960; 1: 852-854Abstract PubMed Google Scholar, 36Berthelot P Walker JG Sherlock S Reid L Arterial changes in the lungs in cirrhosis of the liver: lung spider nevi.N Engl J Med. 1966; 274: 291-298Crossref PubMed Google Scholar, 38Stanley NN Ackrill P Wood J Lung perfusion scanning in hepatic cirrhosis.Br Med J. 1972; 4: 639-643Crossref PubMed Google Scholar Clearly, many investigators believe that this abnormality is primarily responsible for the severe hypoxemia (Pao2 less than 50 mm Hg) occasionally found in patients with chronic liver disease.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar, 24Davis II, HH Schwartz DJ Lefrak SS Susman N Schainker BA Alveolar-capillary oxygen disequilibrium in hepatic cirrhosis.Chest. 1978; 73: 507-511Crossref PubMed Google Scholar, 26Wolfe JD Tashkin DP Holly FE Brachman MB Genovesi MG Hypoxemia of cirrhosis: detection of abnormal small pulmonary vascular channels by a quantitative radionuclide method.Am J Med. 1977; 63: 746-754Abstract PubMed Google Scholar Severe intrapulmonary shunting may result in increased cardiac output to meet the demands of peripheral oxygen delivery and in altered hepatocytic metabolism in a previously damaged liver.12Martini GA Baltzer G Arndt H Some aspects of circulatory disturbances in cirrhosis of the liver.Prog Liver Dis. 1972; 4: 231-250PubMed Google Scholar, 23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar The mechanism of intrapulmonary shunting, although speculative, may relate to two processes: (1) an imbalance between vasoconstrictive and vasodilatory substances that are abnormally metabolized by an impaired liver and (2) unusual regulation of certain pulmonary vessels which results in vasodilatation during hypoxia and thus impaired hypoxic vasoconstriction.43Robin ED Some basic and clinical challenges in the pulmonary circulation.Chest. 1982; 81: 357-363Crossref PubMed Google Scholar In a small percentage of patients with hepatic disease, the portal venous system anastomoses with both the superior vena caval system and the pulmonary venous system, through mediastinal, paraesophageal, and azygous vascular channels.40Franco DP Kinasewitz GT Markham RV Tucker WY George RB Postural hypoxemia in the postpneumonectomy patient.Am Rev Respir Dis. 1984; 129: 1021-1022PubMed Google Scholar, 41Miller WC Heard JG Unger KM Enlarged pulmonary arteriovenous vessels in COPD: another possible mechanism of hypoxemia.Chest. 1984; 86: 704-706Crossref PubMed Google Scholar, 44Schoenmackers J Vieten H Porto-cavale und porto-pulmonale Anastomosen im postmortalen Portogramm.Fortsch Rontgenstrahl. 1953; 79: 488-498Crossref PubMed Google Scholar, 45Calabresi P Abelmann WH Porto-caval and porto-pulmonary anastomoses in Laennec's cirrhosis and in heart failure.J Clin Invest. 1957; 36: 1257-1265Crossref PubMed Google Scholar, 46Shaldon S Caesar J Chiandussi L Williams HS Sheville E Sherlock S The demonstration of porta-pulmonary anastomoses in portal cirrhosis with the use of radioactive krypton (Kr85).N Engl J Med. 1961; 265: 410-414Crossref PubMed Google Scholar, 47Nunez Jr, D Russell E Yrizarry J Pereiras R Viamonte Jr, M Portosystemic communications studied by transhepatic portography.Radiology. 1978; 127: 75-79PubMed Google Scholar, 48Sano A Kuroda Y Moriyasu F Takahashi Y Koizumi S Kimura S Okuda K Porto-pulmonary venous anastomosis in portal hypertension demonstrated by percutaneous transhepatic cineportography.Radiology. 1982; 144: 479-484PubMed Google Scholar Unlike patients in whom intrapulmonary shunts may develop in the absence of portal hypertension, those with portopulmonary shunts usually do have portal hypertension as a result of cirrhotic liver disease or rare congenital malformations.45Calabresi P Abelmann WH Porto-caval and porto-pulmonary anastomoses in Laennec's cirrhosis and in heart failure.J Clin Invest. 1957; 36: 1257-1265Crossref PubMed Google Scholar These shunts were first detected by the use of postmortem angiography.44Schoenmackers J Vieten H Porto-cavale und porto-pulmonale Anastomosen im postmortalen Portogramm.Fortsch Rontgenstrahl. 1953; 79: 488-498Crossref PubMed Google Scholar, 45Calabresi P Abelmann WH Porto-caval and porto-pulmonary anastomoses in Laennec's cirrhosis and in heart failure.J Clin Invest. 1957; 36: 1257-1265Crossref PubMed Google Scholar Calabresi and Abelmann,45Calabresi P Abelmann WH Porto-caval and porto-pulmonary anastomoses in Laennec's cirrhosis and in heart failure.J Clin Invest. 1957; 36: 1257-1265Crossref PubMed Google Scholar for example, demonstrated that 4 of 10 patients who had had cirrhosis had mediastinal venous plexus-bronchial vein anastomoses at autopsy. In two cases, a gelatin mass that had been injected into the portal vein was found in the pulmonary veins and left atrium. These authors postulated that if the pressure gradient between the portal and pulmonary veins exceeded 22 mm Hg, portopulmonary shunting could occur. Premortem radioisotopic studies with use of krypton-8546Shaldon S Caesar J Chiandussi L Williams HS Sheville E Sherlock S The demonstration of porta-pulmonary anastomoses in portal cirrhosis with the use of radioactive krypton (Kr85).N Engl J Med. 1961; 265: 410-414Crossref PubMed Google Scholar and percutaneous transhepatic portography47Nunez Jr, D Russell E Yrizarry J Pereiras R Viamonte Jr, M Portosystemic communications studied by transhepatic portography.Radiology. 1978; 127: 75-79PubMed Google Scholar, 48Sano A Kuroda Y Moriyasu F Takahashi Y Koizumi S Kimura S Okuda K Porto-pulmonary venous anastomosis in portal hypertension demonstrated by percutaneous transhepatic cineportography.Radiology. 1982; 144: 479-484PubMed Google Scholar have also recently been used to substantiate the presence of such shunts. Importantly, such portopulmonary shunts are now thought to be too small to produce the degree of hypoxemia and hemoglobin desaturation described in some patients with portal hypertension46Shaldon S Caesar J Chiandussi L Williams HS Sheville E Sherlock S The demonstration of porta-pulmonary anastomoses in portal cirrhosis with the use of radioactive krypton (Kr85).N Engl J Med. 1961; 265: 410-414Crossref PubMed Google Scholar, 49Nakamura T Nakamura S Tazawa T Abe S Aikawa T Tokita K Measurement of blood flow through portopulmonary anastomosis in portal hypertension.J Lab Clin Med. 1965; 65: 114-121PubMed Google Scholar because, in portal blood, Pao2 is approximately 50 mm Hg and hemoglobin saturation is 70%.23Robin ED Horn B Goris ML Theodore J Van Kessel A Mazoub J Tilkian A Detection, quantitation and pathophysiology of lung “spiders.”.Trans Assoc Am Physicians. 1975; 88: 202-215PubMed Google Scholar, 50Schiff L Schiff ER Diseases of the Liver. Fifth edition. JB Lippincott Company, Philadelphia1982: 30Google Scholar Pleural vascular abnormal" @default.
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• W2169575861 title "Pulmonary Aspects of Chronic Liver Disease and Liver Transplantation" @default.
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