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considering both common and rare developmental vascular abnormalities in young adults who present with unusual spontaneous and recurrent lung hemorrhage. ACKNOWLEDGMENT: We thank Peter Byers, MD, for performing the biochemical studies and for providing the data to include in this report. High-Output Congestive Heart Failure Following Transjugular Intrahepatic Portal-Systemic Shunting* Alan C. Braverman, MD; Mark A. Steiner, MD; Daniel Picus, MD; and Heather White, MD REFERENCES 1 Steinmann B. The Ehlers-Danlos syndrome. In: Steinmann B, et al, eds. Connective tissue and its hereditary disorders. New York: Wiley-Liss, 1993; 351-407 2 Ainsworth SP, Aulicino PL. A survey of patients with Ehlers- Danlos syndrome. Clin Orthop 1993; 296:250-56 3 McKusick VA, ed. Heritable disorders of connective tissue, 4th ed. St. Louis, MO: CV Mosby, 1972; 292-371 4 Lloyd J, Narcisi P, Richards A, et al. A T+6 to C+6 mutation in the donor splice site of Col 3A1 IVS7 causes exon skipping and A hyperdynamic circulatory state with elevated cardiac output, decreased peripheral vascular resistance, and sodium retention occurs in patients with portal cirrhosis. Surgical portal-systemic shunts and transjugular intrahepatic portal-systemic shunts (TIPS) have been shown to worsen the high-output state in these patients. However, clinical evidence of high-output congestive heart failure has been reported only rarely to complicate cirrhosis. We describe a patient who developed highoutput congestive heart failure with markedly elevated filling pressures after TIPS and had complete resolution of heart failure after liver transplantation. (Chest 1995; 107:1467-69) results in Ehlers-Danlos syndrome type IV. J Med Genet 1993; 30:376-80 TIPS=transjugular intrahepatic portal-systemic shunting 5 Milewicz DM, Witz AM, Smith ACM, et al. Parental somatic and germ-line mosaicism for a multiexon deletion with unusual endpoints in a type III collagen (Col 3A1) allele produces Ehlers-Danlos syndrome type IV in the heterozygous offspring. Am J Hum Genet 1993; 53:62-70 6 Grenko RT, Burns SL, Golden EA, et al. Type IV Ehlers-Dan- los syndrome with aspirin sensitivity: a family study. Arch Pathol Lab Med 1993; 117:989-92 7 Byers BH. Disorders of collagen biosynthesis and structure. In: Scriver CR, Beaudet AL, Sly Ws, et al, eds. The metabolic basis of inherited disease. 6th ed. New York: McGraw-Hill, 1989; 2824-2833 8 Jones KL. Smith's recognizable patterns of human malformation. 4th ed. Philadelphia: WB Saunders Co, 1988; 430-31 9 Bonadio J, Holbrook KA, Gelinas RE, et al. Altered triple he- lical structure of type I procollagen is associated with prolonged survival in lethal perinatal osteogenesis imperfecta. J Biol Chem 1985; 260:1734-42 10 Muller NL, Miller RR. Diffuse pulmonary hemorrhage. In: Muller NL, ed. Radiologic clinics of North America. Philadel- phia: WB Saunders, 1991; 965 Key words: high-output state; hyperdynamic circulation; liver transplantation; portal hypertension; transjugular intrahepatic portal-systemic shunts A hyperdynamic circulatory state with elevated cardiac output and decreased peripheral vascular resistance has.been described in patients with portal cirrhosis.' 3 Sodium and water retention may accompany and contribute to the high-output state.4'5 Surgical portal-systemic anastomoses created to treat complications of portal hypertension may worsen the hyperdynamic circulation in cirrhosis.6-8 Despite this hyperkinetic state, high-output congestive heart failure is rarely reported to complicate cirrhosis.2 Transjugular intrahepatic portal-systemic shunts (TIPS), by creating an artificial fistula between branches of the hepatic vein and intrahepatic portal veins, has been developed as a less invasive method of portal-systemic shunting.9-11 Patients previously considered at highest risk for surgical shunts may now be considered candidates for TIPS in treating complications of esophageal varices and ascites.9 Complications from TIPS include worsening of encephalopathy, shunt closure, precipitation of liver failure, infection, and bleeding. *From the Cardiovascular Division (Dr. Braverman), Department of Medicine (Drs. Braverman and Steiner), Department of Radiology (Dr. Picus), and the Gastroenterology Division, Department of Medicine (Dr. White), Washington University School of Medicine, St. Louis. Reprint requests: Dr. Braverman, Cardiology, Washington University School of Medicine, 4940 Parkview Place, St. Louis, MO 63110 CHEST / 107 / 5 / MAY, 1995 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21713/ on 05/02/2017 1467 Table 1-Hemodynamic Data Before Transplantation Right atrial pressure, mm Hg Pulmonary artery pressure, After Transplantation 28 10 55/32 30/13 mm Hg VuXmonsary capiAXary wedge VXankavion 3A 6.2 12.8 <300 andx an ec5ocaXdoam S mo'ftiMs aiXei discharge reveals normal left ventricular function. pressure, mm Hg Cardiac index, L/min/m2 Cardiac output, L/min Systemic vascular resistance, dyne s cm-5 reduction in cardiac output from 13.5 L/min to 10.4 L/min. The patient developed worsening encephalopathy and hepatic dysfunction and underwent successful orthotopic liver transplantation a few days later. Pathology reports confirmed cirrhosis. The hemodynamic measurements changed dramatically after transplantation. The hemodynamics 48 h post transplantation are shown in Table 1. The patient continues to do well posttrans- 3.3 6.9 850 Hemodynamic measurements after TIPS have documented a significant worsening of the hyperdynamic state in cirrhotic patients with increased cardiac output and decreased peripheral vascular resistance.12 Recently, cardiovascular complications have been reported after TIPS'0'12 raising concerns about the safety of this procedure in patients with limited cardiac reserve. 12 Herein, we report a case of high-output congestive heart failure that occurred following TIPS in a cirrhotic patient with no prior cardiac disease and completely resolved after liver transplantation. CASE REPORT A 50-year-old man with alcoholic cirrhosis was admitted to Barnes Hospital for management of congestive heart failure and consideration of liver transplantation for end-stage liver disease. Besides a history of pedal edema treated with diuretics, he had no functional limitations and no cardiac history. Three months before hospital admission, the patient suffered an esophageal variceal bleed treated with sclerotherapy and subsequent TIPS. He did well until 4 to 6 weeks before admission when he noted increasing edema, abdominal swelling, dyspnea, and a 9-kg weight gain. Chest radiograph revealed cardiomegaly, bilateral pleural effusions, and interstitial pulmonary edema. Echocardiogram showed a small pericardial effusion and normal ventricular function. The left ventricular ejection fraction was 64% by radionuclide ventriculography. The patient was treated with diuretics, digoxin, multivitamins, and thiamine and transferred to Barnes Hospital. The patient denied alcohol intake for 2 years. There was no recent change in his diet and no prior cardiac disease. Examination revealed the following: blood pressure, 118 /50; mm Hg; heart rate, 92 beats/min; and respiratory rate, 28/min. Chest examination revealed bilateral rales. The jugular venous pressure was markedly elevated. Carotid upstrokes were hyperdynamic. There was a systolic ejection murmur and a third heart sound present. The abdomen was distended with ascites. There was 2+ peripheral edema and asterixis present. There was no digital clubbing present. Laboratory findings included the following: hematocrit, 30.3%; platelet count, 124,000/mm3; prothrombin time, 15.7 s; total bilirubin, 2.3 mg/dL; and albumin, 3.0 g/dL. Arterial blood gas values while breathing 80% oxygen by face mask were pH of 7.44, Po2 of 71 mm Hg, and Pco2 of 40 mm Hg. Results of thyroid function tests were normal and there was no evidence for infection. Hemodynamic measurements obtained using a pulmonary artery catheter are shown in Table 1. Because of the high-output state with markedly elevated filling pressures, a hepatic arteriogram was performed revealing a patent TIPS and no angiographically visible arteriovenous malformations. Transient balloon occlusion of the TIPS led to a \iospWax DISCUSSION A hyperkinetic circulatory state has been documented in chronic liver disease and is unrelated to anemia or nutri- tional deficiencies. 13 Murray et a12 performed hemodynamic measurements in 24 patients with chronic liver disease and found an elevated cardiac index in patients with portal cirrhosis (mean cardiac index, 5.36 + 1.98 L/min/ m2) and even higher cardiac index in patients who had undergone surgical portocaval anastomoses (6.31 ± 1.70 L/min/m2). Despite this high-output state, only a single patient with the clinical syndrome of high-output congestive heart failure complicating chronic liver disease was described.2 Liver dysfunction and the degree of extrahepatic portal-systemic collateral shunting were considered important factors contributing to the hyperkinetic state. The increased cardiac output and increased total plasma volume were likened to high-output states such as arteriovenous fistula or generalized vasodilatation.2 Surgical portal-systemic anastomoses reduce portal pressure and blood flow decreasing the risk of variceal bleeding in cirrhotic patients. The hemodynamic consequences of surgical portal-systemic shunts have been variable, in part due to the type of shunt and timing of the measurements.6-8,13 A worsening of the hyperdynamic state with increased cardiac output and decreased systemic vascular resistance has been reported following portocaval shunts7,8 and distal splenorenal shunts,6 while others have found no significant hemodynamic change after shunting.'3 Transjugular intrahepatic portal-systemic shunts (TIPS) are a less invasive, less expensive, and less morbid procedure than surgical portal-systemic anastomosis for treating selected patients with complications of portal hypertension.9'11 Major complications of TIPS include worsening hepatic encephalopathy and stenosis or occlusion of the stent.9 Cardiovascular complications following TIPS have been rarely reported and include myocardial infarction and pulmonary edema.'0 A patient with underlying heart disease who died of heart failure 16 days after TIPS was recently described.'2 Azoulay et al'2 examined the cardiovascular consequences of TIPS making hemodynamic measurements before and after TIPS in 12 patients. Compared with before TIPS, at 1 month after TIPS, the cardiac index was significantly increased from 4.5 ± 1.3 L/min/m2 to 7.4 ± 1.4 L/min/m2 and the systemic vascular resistance was significantly decreased from 808 ± 323 dyne s cm-5 to 467 ± 101 dyne s cm-5. The pulmonary artery and pulmonary capillary wedge pressure were not significantly changed after TIPS. The authors cautioned that patients with poor cardiac reserve may not tolerate the hemodynamic abnormalities associated with this procedure.'2 1468 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21713/ on 05/02/2017 Selected Reports The factors underlying the hyperdynamic circulation in cirrhotic portal hypertension are incompletely understood. Hepatic dysfunction and portal-systemic collaterals may allow certain vasodilator substances (glucagon, prostaglandins, nitric oxide) to escape hepatic inactivation leading to local or systemic vasodilatation.i5"4 A reduced vascular sensitivity to endogenous vasoconstrictors may also contribute.'4 The opening of preformed but functionally inactive arteriovenous anastomoses by a vasodilator substance was postulated over 30 years ago by Murray et al.2 In fact, arteriovenous malformations in the skin, splanchnic vascular bed, and pulmonary circulation are known sequelae of cirrhosis.2-4 Sodium retention accompanies systemic vasodilation and may contribute to the hyperkinetic circulation in cirrhosis.45 The hepatopulmonary syndrome describes the clinical relationship between chronic liver disease and intrapulmonary vascular dilatations that results in abnormal pulmonary gas exchange and an increased alveolar-arterial oxygen gradient.'5"16 Clinical manifestations include dyspnea, digital clubbing, platypnea, and orthodeoxia.1516 The hyperdynamic circulatory state of liver disease may accompany the hepatopulmonary syndrome and influence the level of arterial hypoxemia present.16 As with highoutput state in portal cirrhosis, the cause of the abnormal pulmonary vascular physiology in this condition remains unclear. The worsening hyperdynamic state after surgical portalsystemic anastomosis and TIPS is poorly understood and of uncertain long-term importance. These procedures reduce hepatic blood flow and may exacerbate encephalopathy from failure to metabolize offending substances. Likewise, the increased cardiac output after shunting may result from the failure to clear a putative vasodilator substance. The patient described in our report developed clinically significant high-output congestive heart failure after TIPS and returned to baseline immediately after liver transplantation. While the hyperkinetic circulation of cirrhosis has rarely been reported to be associated with congestive heart failure,2 our report documents that some patients without underlying cardiac disease may develop this syndrome after TIPS. REFERENCES 1 Kowalski HJ, Abelmann WH. The cardiac output at rest in Laennec's cirrhosis. J Clin Invest. 1953; 32:1025-33 2 Murray JF, Dawson AM, Sherlock S. Circulatory changes in chronic liver disease. Am J Med 1958; 24:358-67 3 Lee SS. Cardiac abnormalities in liver cirrhosis. West J Med 1989; 151:530-35 4 Schrier RW. Pathogenesis of sodium and water retention in high-output and low-output cardiac failure, nephrotic syndrome, cirrhosis, and pregnancy. N Engl J Med 1988; 319:106572, 1127-34 5 Colombato LA, Albillos A, Groszmann RJ. Temporal relationship of peripheral vasodilatation, plasma volume expansion and the hyperdynamic circulatory state in portal-hypertensive rats. Hepatology 1992; 15:323-28 6 Levi JU, Zeppa R, Hutson DG, et al. Early hemodynamic effects of the distal splenorenal shunt. Surg Forum 1976; 27:370 7 Reichle FA, Owen OE. Hemodynamic patterns in human be- patic cirrhosis: a prospective randomized study of the hemodynamic sequelae of distal splenorenal (Warren) and mesocaval shunts. Ann Surg 1979; 190:523-34 8 Gelman S, Aldrete JS, Halpern N. Hemodynamics during portacaval shunt surgery in humans. Anesth Analg 1982; 61:185-86 9 Conn HO. Transjugular intrahepatic portal-systemic shunts: the state of the art. Hepatology 1993; 17:148-58 10 Helton WS, Belshaw A, Althaus S, et al. Critical appraisal of the angiographic portacaval shunt (TIPS). Am J Surg 1993; 165: 566-71 11 Ring EJ, Lake JR, Roberts JP, et al. Using transjugular intrahepatic portosystemic shunts to control variceal bleeding before liver transplantation. Ann Intern Med 1992; 116:304-09 12 Azoulay D, Castaing D, Dennison A, et al. Transjugular intrahepatic portosystemic shunt worsens the hyperdynamic circulatory state of the cirrhotic patient: preliminary report of a prospective study. Hepatology 1994; 19:129-32 13 Del Guercio LRM, Commaraswamy RP, Feins NR, et al. Pulmonary arteriovenous admixture and the hyperdynamic cardiovascular state in surgery for portal hypertension. Surgery 1964; 56:57-74 14 Pizcueta MP, Pique JM, Bosch J, et al. Effects of inhibiting nitric oxide biosynthesis on the systemic and splanchnic circulation of rats with portal hypertension. Br J Pharmacol 1992; 105:184-90 15 Krowka MJ, Cortese DA. Hepatopulmonary syndrome: current concepts in diagnostic and therapeutic considerations. Chest 1994; 105:1528-37 16 Rodriguez-Roisin R, Krowka MJ. Is severe arterial hypoxaemia due to hepatic disease an indication for liver transplantation? a new therapeutic approach. Eur Respir J 1994; 7:839-42 Acute, Reversible Left Ventricular Dysfunction in Status Asthmaticus* Glenn N. Levine, MD; Charles Powell, MD; Sheilah A. Bernard, MD; Debra Sherman, MD; L. Jack Faling, MD, FCCP; and Ravin Davidoff, MB, BCh Three cases of rapidly reversible severe myocardial depression are described in patients with status asthmaticus. Initial echocardiograms obtained within 1 day of hospital admission revealed global left ventricular hypokinesis with ejection fractions of 11 to 34%. Follow-up echocardiograms obtained only 3 to 8 days later revealed marked improvement of left ventricular function. Possible mechanisms responsible for the observed rapidly reversible myocardial depression and the clinical implications of this finding are discussed. (Chest 1995; 107:1469-73) *From the Department of Medicine, Evans Memorial Department of Clinical Research, Boston University Medical Center Hospital, Boston City Hospital, and Boston Veterans Affairs, Boston. Reprint requests, Dr. Davidoff, Boston University Medical Center, 88 E. Newton Street, Boston, MA 02118 CHEST / 107/5 / MAY, 1995 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21713/ on 05/02/2017 1469