Case Report An Unusual Case of Report An Unusual Case of Cirrhosis ... LFTs, hepatitis serologies,...
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Case ReportAn Unusual Case of Cirrhosis
Ahmad Alkaddour,1 Kenneth J. Vega,2 and Adil Shujaat3
1 Department of Medicine, University of Florida College of Medicine Jacksonville, 655 West 8th Street, Jacksonville, FL 32209, USA2Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA3Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida College of Medicine-Jacksonville,655 West 8th Street, Jacksonville, FL 32209, USA
Correspondence should be addressed to Ahmad Alkaddour; email@example.com
Received 28 October 2013; Accepted 17 December 2013; Published 4 February 2014
Academic Editors: C. Elsing, G. Kouraklis, and V. Lorenzo-Zuniga
Copyright 2014 Ahmad Alkaddour et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.
49-year-old white female with remote h/o sarcoidosis was referred to GI when her liver was noted to be nodular. Physicalexamination revealed normal vital signs and no icterus, spider nevi, clubbing, ascites, hepatosplenomegaly, or ankle edema.LFTs, hepatitis serologies, ANA, AMA, ASMA, Ferritin, Ceruloplasmin, and 1-AT, level were unremarkable. Liver biopsyshowed cirrhosis. She developed worsening of baseline SOB and was hospitalized. She was eventually diagnosed with constrictivepericarditis. A diagnosis of cardiac cirrhosis was made.
Constrictive pericarditis is a rare but severely disabling conse-quence of the chronic inflammation of the pericardium, lead-ing to an impaired filling of the ventricles and reduced ven-tricular function . The timely diagnosis of a cardiac etio-logy of liver dysfunction is important because such dysfunc-tion is potentially reversible if the underlying cardiac diseaseis treated before the development of frank cirrhosis [2, 3].
Below, we present a case of a 49-year-old female whowas incidentally found to have cirrhosis. Initial workup wasnegative. Thoracic imaging showed pericardial calcificationswhich ultimately led to the diagnosis of constrictive peri-carditis.Wewill briefly discuss the literature on cardiac causesof liver cirrhosis.
2. Case Report
49-year-oold white female with remote h/o sarcoidosis wasreferred to GI when her liver was noted to be nodular duringlaparoscopy for an ovarian cyst. She denied fatigue, vomiting-up blood, abdominal distension and pain, ankle swelling,itching, yellow discoloration of skin and eyes, and episodesof confusion or sleepiness. She denied alcohol abuse. Physicalexamination revealed normal vital signs and no icterus,
spider nevi, clubbing, ascites, hepatosplenomegaly, or ankleedema. LFTs revealed mild elevation in alkaline phosphataseand PT was slightly prolonged. CBC showed mild thrombo-cytopenia. Hepatitis serologies, ANA, AMA, ASMA, Ferritin,Ceruloplasmin, and 1-AT, level were unremarkable. A liverbiopsy was done. It confirmed cirrhosis. Biopsy did notshow any granulomas but showed sinusoidal dilatationwhichprompted a referral to cardiology. ECHO showed enlargedIVC and was otherwise unremarkable. A left and right heartcatheterization was done. LHC showed normal coronariesand RHC showed RAP of 12mmHg, PAP of 32/15 (mean 21)mmHg, PAWP of 18mmHg, LVEDP of 18mmHg, and COof 5.2 L/min. She developed worsening shortness of breathand was referred to pulmonary medicine. PFTs showedmild restriction but CXR was unrevealing. CTPA ruled outPE and showed scattered pericardial calcification. BubbleECHO did not show a right to left shunt. She developedSVT and was hospitalized. She underwent a repeat left andright heart catheterization: RAP was 25mmHg, PAP 52/25(mean 37) mmHg, PAWP 32mmHg, LVEDP 36mmHg,and CO 4.12 L/min. Simultaneous measurement of left- andright-sided pressures confirmed constrictive pericarditis. Inretrospect, the cirrhosis is cardiac cirrhosis and the result oflong-standing elevated right-sided heart pressures. Similarlyher dyspnea on exertion can be explained by constrictive
Hindawi Publishing CorporationCase Reports in Gastrointestinal MedicineVolume 2014, Article ID 670176, 3 pageshttp://dx.doi.org/10.1155/2014/670176
2 Case Reports in Gastrointestinal Medicine
pericarditis. We believe that constrictive pericarditis resultedfrom sarcoidosis. She is now being evaluated for pericardiec-tomy.
Our case illustrates the importance of considering a cardiacetiology in the work-up of cirrhosis especially when the mostcommon causes are not found. Classically, serum albuminis normal unless frank cirrhosis has developed; ascitic fluidanalysis reveals an elevated serum albumin-ascites gradient(>1.1 g/dL) typical of portal hypertension and demonstratesan elevated total protein level (>2.5 g/dL) . In our case,the LFTs were only mildly abnormal with a mildly elevatedPT and mildly elevated alkaline phosphatase. The findingof a nodular liver was incidental and no primary liverdisease was found. Sinusoidal dilation indicating congestivehepatopathy helped steer us towards a cardiac etiology; otherfindings on liver biopsy that may be found include sinusoidaldegeneration, variable degrees of hemorrhagic necrosis inzone 3, fatty change, and variable degrees of cholestasis . Ingeneral, patients with passive hepatic congestion do not havestigmata of portal hypertension such as spider angiomataor evidence of portosystemic shunts such as caput medusa. Patients with constrictive pericarditis, however, maydevelop ascites. Nevertheless, such findings were absent inour patient.
Constrictive pericarditis is a rare but severely disablingconsequence of the chronic inflammation of the pericardium,leading to an impaired filling of the ventricles and reducedventricular function .
Establishing the diagnosis of constrictive pericarditisand secondary congestive hepatopathy/cirrhosis remains achallenge. One study reported a median delay in diagnosisof greater than 10 years . In another case, the diagnosis ofconstrictive pericarditis was made only after liver transplan-tation was performed for presumed cryptogenic cirrhosis .Much of the difficulty in diagnosing constrictive pericarditiscan be attributed to its insidious course and the fact thatsome of the symptoms and signs resulting from it, that is,dyspnea and ascites, can bemistakenly thought to result fromprimary liver dysfunction. Dyspnea in a patient with liverdysfunction usually makes one suspects hepatopulmonarysyndrome, portopulmonary hypertension, cardiomyopathy,hepatic hydrothorax, ascites, or anemia, whereas ascitesitself is a hallmark of liver disease complicated by portalhypertension. Constrictive pericarditis is curable and shouldbe considered in all cases of unexplained cirrhosis, regardlessof atypical hepatic histology .
Pericardial involvement is uncommon in sarcoidosiseven in the presence of extensive myocardial infiltration.It is observed in fewer than 10% of patients with cardiacsarcoidosis . Pericardial calcification should provide a clueto the diagnosis of constrictive pericarditis (see Figure 1).One case study demonstrated the importance of this findingon low-cost imaging to help point towards this diagno-sis . The major differential of constrictive pericarditisis restrictive cardiomyopathy which is more common inpatients with sarcoidosis. Although echocardiography and
Figure 1: CT scan of chest showing pericardial calcifications.
cardiac CT/MR might distinguish one from the other, themost specific finding differentiating constrictive pericarditisfrom restrictive cardiomyopathy is demonstrated on heartcatheterization. Simultaneous measurement of the left andright heart pressures demonstrates respiratory variation inventricular filling and increased ventricular interdependencealong with increased atrial pressures and equalization of end-diastolic pressures .
The timely diagnosis of a cardiac etiology of liver dys-function is important because such dysfunction is potentiallyreversible if the underlying cardiac disease is treated beforethe development of frank cirrhosis [2, 3]. Moreover, earlytreatment of underlying cardiac disease might also preventthe development of hepatocellular carcinoma as suggested byan interesting case study in which a patient with negativehepatitis serologies and cirrhosis secondary to constrictivepericarditis developed hepatocellular carcinoma confirmedby biopsy .
This case study illustrates to gastroenterologists the needto consider a cardiac etiology in the work-up of cirrhosisespecially when the most common causes are not found andif liver biopsy shows evidence of congestive hepatopathy andis inconclusive for primary liver disease. The presence ofpericardial calcification in a patient with cirrhosis shouldsuggest the possibility of constrictive pericarditis and cardiaccirrhosis and prompt cardiac MR/CT imaging and cardiaccatheterization. Simultaneous invasive measurement of thepressures in the right and left heart chambers by an experi-enced cardiologist is the key to making the diagnosis. Con-strictive pericarditis is a rare but well-known manifestationof sarcoidosis. Prompt referral to a thoracic surgeon forpericardiectomy is important once the diagnosis is made toprevent progression to cirrhosis.
Conflict of Interests
The authors declare that there is no conflict of interestsregarding the publication of this paper.
 B. Maisch, P. M. Seferovic, A. D. Ristic et al., Guidelines onthe diagnosis andmanagement of pericardial diseases executive
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summary; The Task force on the diagnosis and managementof pericardial diseases of the Euro