J Cardiol 2005 Apr; 45（4）: 161 – 164

Esophageal Varices Without PortoSystemic Venous Pressure Gradientin a Patient With Post-Pericardiotomy Constrictive Pericarditis:
A Case Report
Kazuyuki

OZAKI, MD

Makoto

KODAMA, MD

Fumio

YAMASHITA, MD

Tsuyoshi

YOSHIDA, MD

SatoruHIRONO, MD

Kiminori

KATO, MD

Yoshifusa

AIZAWA, MD,FJCC

Abstract ─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────
A 51-year-old woman was admitted with intractable congestive heart failure and progressive anemia.
She had undergone mitral valve replacement for mitral regurgitation at age 23 years. Subsequently, hermitral prosthesis was replaced twice due to thrombotic stack and valve insufficiency. Signs of congestive
heart failure became evident at age 46 years. Gastrointestinal endoscopy revealed esophageal varices,which were treated by endoscopic variceal ligation. Cardiac catheterization disclosed elevated pulmonary
capillary wedge pressure（mean 16 mmHg）, right atrial pressure（mean 15 mmHg）, and hepatic vein wedge
pressure（mean 15 mmHg）. She died at age 53 years. Autopsy showed severe congestive liver but not livercirrhosis. Esophageal varices may progress in spite of the absence of porto-systemic pressure gradient inpatients with severely high venous pressure.
─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────J Cardiol 2005 Apr ; 45（4）: 161−164

Key Words
Heart failure
Hypertension portal
Pericarditis post-pericardiotomy constrictive

INTRODUCTION

CASE REPORT

Development of esophageal varices depends on
the portal hypertension whereas heart failure tends
to cause liver cirrhosis and occasionally esophagealvarices. However, detailed observations of thepathogenesis are scarce. We investigated the pressure gradient between the portal circulation andsystemic central vein in a patient with chronic
intractable heart failure complicated withesophageal varices.

A 51-year-old woman was admitted to our hospital with intractable congestive heart failure and progressive anemia. She was treated under a diagnosisof mitral regurgitation at age 11 years. She underwent mitral valve replacement surgery at the 23years. Subsequently, her mitral prosthesis wasreplaced twice due to thrombotic stack episode andvalve insufficiency. She received an implanted permanent pacemaker because of complete atrioventricular block at age 45 years. Symptoms and signs──────────────────────────────────────────────
新潟大学大学院医歯学総合研究科 循環器学分野 : 〒 951−8510 新潟県新潟市旭町通 1−757
Division of Cardiology, Niigata University Graduate School of Medical & Dental Sciences, Niigata
Address for correspondence : OZAKI K, MD, Division of Cardiology, Niigata University Graduate School of Medical & DentalSciences, Asahimachi-dori 1−757, Niigata, Niigata 951−8510 ; E-mail : k-ozaki@med.niigata-u.ac.jp
Manuscript received February 6, 2004 ; revised August 27 and October 12, 2004 ; accepted October 12, 2004

161

162

Ozaki, Kodama, Yamashita et al

Fig. 1 Abdominal computed tomography scans showing findings of congestive liver, but no findingsof liver cirrhosisof congestive heart failure became evident from age
46 years. Intractable heart failure, progressive anemia and tarry stool appeared 2 years before admission. On admission, she also complained of epigastric discomfort.
Her height was 156 cm and weight was 44 kg.
Blood pressure was 104/60 mmHg and heart rate
was 70 beats/min. Chest examination revealed norales. There was a grade 3/6 holo-systolic murmur
at the apex. Liver was palpable 3FB under the rightcostal border and the hepato-jugular reflux wasobserved. Her abdomen was soft and flat, but fluctuation was not evident. Pretibial edema was notnoted. Chest roentgenogram revealed cardiothoracic ratio of 68%. Laboratory examinations did notsuggest liver cirrhosis or hepatitis of viral orautoimmune origin. Abdominal computed tomography scanning showed findings of congestive liver,
but splenomegaly and ascites were not shown, andthere were no findings of liver cirrhosis（Fig. 1）.
Gastrointestinal endoscopy was performed because
she complained of epigastric discomfort andrevealed esophageal varices（ F 2 , C b , L S , RCsign ＋）（Fig. 2）.
Transthoracic echocardiography showed remarkably dilated left atrium（7.6 cm）, moderate mitral
regurgitation of perivalvular leakage and severe tricuspid regurgitation. Cardiac catheterization disclosed elevation of pulmonary capillary wedge
pressure（ mean 16 mmHg）, right atrial pressure（mean 15 mmHg）
and mild left ventricular dysfunction（Table 1）. Left ventriculography demonstrated
second degree mitral regurgitation. Coronary
angiography revealed no significant stenosis.
Hepatic vein wedge pressure was measured in theFig. 2 Gastrointestinal fiberscope image showing
esophageal varices

Table 1

Hemodynamic data from cardiac catheterization

Pulmonary capillary wedge pressure a14v25（mean 16）mmHg
Pulmonary artery pressure

39/15（mean 24）mmHg

Right ventricular pressure

36/EDP 13 mmHg
a12v25（mean 15）mmHg

Right atrial pressure
Left ventricular pressure

98/EDP 14 mmHg

Aortic pressure

99/47（mean 65）mmHg

Cardiac index

2.9 l/min/m2

Left ventricular end-diastolic volume index

126 ml/m2

Left ventricular end-systolic volume index

71 ml/m2
44%

Ejection fraction
EDP＝end-diastolic pressure.

J Cardiol 2005 Apr; 45（4）: 161 – 164

Constrictive Pericarditis With Esophageal Varices

right hepatic vein. Mean hepatic vein wedge pressure was 15 mmHg, which implied portal hypertension. Cardiac catheterization was performed when
her condition of heart failure was stable. Endoscopic
ligation of the esophageal varices was performed.
Her esophageal varices disappeared and she
returned to the outpatient clinic.
Her congestive heart failure was intractable. Oneand a half years after discharge, ascites and emaciation became evident. Protein and albumin levels ofascites fluid were 5.2 and 3.5 g/dl, respectively, andserum protein and albumin levels were 7.1 and4.4 g/dl. She died of congestive heart failure at age
53 years. At the autopsy, thickened adhesive pericardium which firmly surrounded the heart wasobserved. The autopsy showed high degree of atrophy, degeneration, and necrosis of the liver cells,and fibrosis of the central zone. The degree of central fibrosis varied significantly from region toregion. Thrombosis of hepatic veins and portal
veins were not observed. Reconstruction of thelobes was also not observed. Severe congestive
liver was found but no evidence of liver cirrhosis.
Other esophageal varices were not present. The
final diagnosis was post-pericardiotomy constrictive pericarditis with congestive liver.
DISCUSSION
Congestive heart failure affects the liver throughtwo ways : forward failure through decreased
hepatic blood flow and backward failure throughcongestive liver. Both lead to hepatocellular hypoxia. Ischemic damage and passive congestion resultin liver fibrosis around the perivenular area and in
the space of Disse. The process impairs the bloodto-hepatocyte diffusion of oxygen and nutrients. As
a result, cardiogenic liver cirrhosis develops1−4）.
Generally, liver dysfunction occurs in patients withheart failure persisting for 60 days. Fibrosis of thecentral zone progresses and liver cirrhosis develops
after 3 years5）. Currently cardiac liver cirrhosis isquite rare. The chief causes of cardiac cirrhosis areischemic heart disease（ 31%）, cardiomyopathy（23%）, valvular heart disease（23%）, restrictive1）
lung disease（15%）, and pericardial disease（8%）.
Patients with cardiogenic liver cirrhosis rarely
show systemic signs of porto-systemic shunts, suchas caput medusae2）. None of 28 autopsied cases disclosed esophageal varices 6）. These observations
may imply that cardiogenic liver cirrhosis is not
involved in the porto-systemic pressure gradient.
J Cardiol 2005 Apr; 45（4）: 161 – 164

163

However, chronic and severe right heart failure isbelieved to involve liver cirrhosis with esophageal
varices occasionally1）. Five cases of esophageal
varices related to congestive heart failure without
liver cirrhosis were reported among consecutive
adult autopsies4）.
Development of esophageal varices depends on
the portal hypertension and on the pressure gradient
between the portal circulation and systemic central
veins. Once liver cirrhosis has developed, portal
hypertension may subsequently progress to exceed
the congestive central vein pressure. If the pressure
gradient between the portal circulation and central
vein becomes significant, porto-systemic shunt may
develop. Pressure in the esophageal varices is15.5 mmHg in cirrhotic patients, significantly lower
than the main portal pressure of about 18.8mmHg7）. Right heart failure actually leads to portal
hypertension through congestive liver, but does it
produce porto-systemic pressure gradient? There is
no low or normal pressure region in the systemic
central veins in patients with severe congestive
right heart failure. Detailed observation of thepathogenesis of esophageal varices in congestive
heart failure is rare.
In this case, we investigated the pressure gradient between the portal circulation and systemic central veins in a patient with chronic intractable heart
failure complicated by esophageal varices. Portal
hypertension was evident, but the hepatic veinwedge pressure was equal to the right atrial pressure and pulmonary capillary wedge pressure.
There was no pressure gradient between the portal
circulation and systemic central veins.
The esophageal varices in this case were thought
to have appeared with the progression of congestive
heart failure. Gastrointestinal endoscopy showed nofindings of esophageal varices at age 46 years.
From that time, signs of congestive heart failure
became evident. Esophageal varices had developed
at age 51 years in spite of the absence of porto-systemic venous pressure gradient. Why did the portosystemic shunt vessels develop in this patient without a pressure gradient? The intermittent pressure
gradient may have occurred only after daily postprandial periods. Another hypothesis is that a period of porto-systemic pressure gradient occurred
during progression of congestive heart failure. If
congestive liver leads to significant portal hypertension in the early phase of congestive heart failure
with not so high venous pressure, a period of sig-164

Ozaki, Kodama, Yamashita et alnificant pressure gradient would occur. Anotherpossibility is that the venous hypertension and portal hypertension caused dilation of the submucosalveins even in the absence of a pressure gradient.
To clarify the pathogenesis of esophageal varices
要in patients with severe chronic right heart failure
and without porto-systemic pressure gradient,examination of hemodynamics at several time
points will be necessary during the progression ofcongestive liver.
約

門脈系静脈系圧較差が認められずに食道静脈瘤が発現した
心膜切開後収縮性心膜炎の 1 例
尾±和幸

小 玉誠山下文男

吉 田広 野暁

加藤公則

相澤義房

剛

症例は 51 歳，女性．難治性のうっ血性心不全および進行性の貧血により入院した．23 歳時に僧帽弁閉鎖不全症により僧帽弁置換術を受けた．その後，人工弁血栓症および人工弁不全により 2 回の再僧帽弁置換術を受けた．46 歳より心不全症状が出現した．入院後，上部消化管内視鏡により食道静脈瘤が認められ，内視鏡的結紮術を施行した．心臓カテーテル検査では，肺動脈楔入圧（平均 16 mmHg），右房圧（平均 15 mmHg），肝静脈楔入圧（平均 15 mmHg）の上昇が認められた．53 歳で死亡した．剖検により高度のうっ血肝が認められたが，肝硬変はなかった．静脈圧が高度に上昇した心不全症例では，食道静脈瘤は門脈系と静脈系の圧較差が存在しなくても増悪する可能性が示された．

J Cardiol 2005 Apr; 45（4）: 161−164

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J Cardiol 2005 Apr; 45（4）: 161 – 164