This is a case report of a middle-aged man, who was referred by his general practitioner (GP) to the Gastroenterology outpatients with persistently deranged liver function tests (LFTs).
Detailed clinical assessment and extensive investigations eventually led to the diagnosis of constrictive pericarditis. This is a very unusual presentation of a rare disease in which the diagnosis was greatly complicated.
A 64-year-old man was referred to the Gastroenterology Department by his GP with persistently deranged liver function tests (LFTs) for the last nine months. There was no history of abdominal pain, pruritus, dark urine or clay colored stools. However, on direct questioning, the patient admitted to dyspnoea for nine months, which was present at rest as well as on exertion.
He denied chest pain, orthopnoea, paroxysmal nocturnal dyspnoea or peripheral oedema. The patient had a history of basal cell carcinoma of the right temple.
His medication comprised a diuretic which had been started recently.
He was married with one child and worked as a farmer. He was a non-smoker.
He drank about 20 units of alcohol per week, although he admitted to drinking more heavily in the past. There were no other risk factors for liver disease.
During the previous year, the patient had been extensively investigated for dyspnoea by the respiratory physicians and the cardiologists. Pulmonary function tests, echocardiogram and cardiac angiography, were all reported to be normal.
On examination, the patient was not icteric but had a palmer erythema and facial telangiectasia. He had a large multi-nodular goiter with retrosternal extension but was clinically euthyroid.
His pulse was 70/min and his blood pressure 140/90 mmHg; he was apyrexial.
Cardiovascular examination revealed sinus rhythm; jugular venous pressure (JVP) was markedly raised beyond the angle of the jaw; apex beat was difficult to feel; first and second heart sounds were present with a third sound at the base of the heart; there was no peripheral oedema.
On examination of the respiratory system, the patient was dyspnoeic at rest with peripheral cyanosis; trachea was central; chest expansion was reduced symmetrically; percussion note was dull at both lung bases; air entry was diminished over both lower lobes.
Abdominal examination revealed an enlarged, pulsatile liver and there was flank dullness suggestive of ascites.
A clinical diagnosis of heart failure with bilateral pleural effusions was made. Deranged LFTs were attributed to hepatic congestion, but chronic liver disease related to alcohol was also considered.
Blood tests Initial investigations reported a haemoglobin of 15.7 g/dL, white blood count of 7.1 X 10⁹ and platelets of 253 X 10⁹; urea was 5.8 mmol/L, creatinine 66 μmol/L, sodium 135 mmol/L and potassium 4.5 mmol/L; albumin of 36 g/L, globulin 43 g/L, alanine aminotransferase 28 iu/L, alkaline phosphatase 397 iu/L, gamma-glutamyl transpeptidase 835 iu/L, bilirubin 69 μmol/L; prothrombin time was 13.6 seconds.
Screen for the causes of chronic liver disease – including alpha-1-antitrypsin, alpha-fetoprotein, serum ferritin, total iron binding capacity, iron saturation, serum copper, cerruloplasmin, hepatitis B and C serology, anti-mitochondrial antibodies and anti-nuclear antibodies – was negative.
Chest X Ray reported the presence of bilateral pleural effusions. Abdominal ultrasound scan reported moderate ascites and a coarse texture of the liver. Computed tomography (CT) scan of the chest and abdomen (Fig.1) revealed a heterogeneous liver with lack of enhancement of hepatic veins, raising the suspicion of Budd Chiari Syndrome; but this diagnosis was not confirmed on the doppler ultrasound of the hepatic veins which showed normal flow.
A repeat echo showed a non-dilated left ventricle with good systolic function and no left ventricular hypertrophy; the right ventricle appeared non-dilated with good right ventricular systolic function and a right ventricular systolic pressure of 35mm Hg.
Fig 1. Heterogeneous enlarged liver (A); Hepatic veins not well demonstrated (B).
Liver biopsy was performed which showed the presence of nodular regenerative hyperplasia of the liver.
An upper gastrointestinal endoscopy was carried out which revealed reactive gastropathy but there was no evidence of oesophageal or gastric varices.
In view of the conflicting evidence from the clinical examination and the imaging, the results of the investigations were reviewed.
On this occasion, the radiologist noted subtle hints of pericardial thickening and a tubular configuration of the left ventricle suggestive of a diagnosis of constrictive pericarditis (Fig.2).
The unusual feature however was the lack of calcification of the pericardium.
Fig. 2 CT shows pericardial thickening (A); elongated left ventricle (B). No pericardial effusion or calcification.
Magnetic resonating imaging (MRI) of the heart (Fig.3) was carried out which confirmed the presence of pericardial thickening with apparent anatomical deformation of the cardiac silhouette.
Delayed enhancement images showed gadolinium uptake and delayed washout in parts of the pericardium – these findings were consistent with the diagnosis of constrictive pericarditis.
Fig. 3 MRI heart showing pericardial thickening.
The patient was subsequently transferred to the cardiologists. He underwent left and right heart catherization which confirmed the diagnosis of constrictive pericarditis.
Abnormal LFTs are a very common reason for referral to gastroenterology outpatient clinics.
Common causes include billiary tract obstruction, viral and autoimmune liver disease, malignancy, adverse drug reactions and alcohol excess.
It is very unusual for patients with hepatic congestion to present primarily with deranged LFTs. Furthermore, constrictive pericarditis is a rare cause of hepatic congestion.
The more well-recognized causes of hepatic congestion include Budd-Chiari syndrome (hepatic vein thrombosis), tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, pulmonary hypertension, right ventricular failure and cardiomyopathy.
The primary abnormality in constrictive pericarditis is that the thickened fibrotic pericardium impedes normal diastolic filling. This results in elevated pressures on the right side of the heart which are then transmitted to the venous system.
However, the underlying cause of this particular haemodynamic disturbance is often difficult to diagnose. The various different pathologies that result in elevated right heart pressures share similar symptoms, physical findings and haemodynamic observations (Table 1).
Table 1. Clinical Features of Constrictive Pericarditis
2. Exercise intolerance
3. Rapid, low-volume pulse
4. Elevated jugular venous pulse (JVP) with rapid y descent
5. Kussamaul’s sign (a paradoxical rise in the JVP during inspiration)
6. Loud third heart sound or ‘pericardial knock’
8. Deranged liver function tests
10. Peripheral oedema
11. Pulsus paradoxus (an excessive fall in blood pressure during inspiration) present in some case
While accurate clinical assessment is of considerable importance, advances in technology – particularly, multi-slice CT, MRI and invasive haemodynamic tests – have greatly improved the diagnosis of constrictive pericarditis (Table 2).
Low Voltage QRS and T wave inversion/flattening. Atrial fibrillation is present in one third.
Chest X Ray
Small heart. May show calcification seen on lateral film or by using fluoroscopy.
Thickened pericardium with calcification predominantly over right heart. Small ventricular cavities and dilated atria.
CT scan/ MRI
Pericardial calcification and tubular heart.
Elevation and equalization of the diastolic pressures in all cardiac chambers. Left and right atrial pressures are all equal or differ by less than 5 mmHg. Right and left ventricular tracings show an early diastolic "dip-and-plateau "
Note: Limitations of radiology: Absence of pericardial thickening does not exclude constrictive pericarditis. History of cardiac surgery or post-pericardiotomy syndrome both have significant pericardial thickening in the absence of clinical symptoms.
Reference [2, 3].
The causes of constrictive pericarditis are listed in Table 3.
The search for the aetiology is often inconclusive and most cases diagnosed in developed countries are classified as idiopathic. Of those who have a specific aetiology, the most common causes are tuberculosis, post-bacterial or viral pericarditis and malignancy.
Table 3. Causes of constrictive pericarditis
2. Tuberculosis (usually post-pericardial effusion)
3. Bacterial pericarditis or post-viral
5. Mediastinal radiotherapy
6. Drugs (eg: hydralazine, associated with lupus-like syndrome)
7. Following severe uraemic pericarditis
8. Trauma/post-cardiac surgery
9. Recurrent pericarditis
Reference [2, 3].
Given the rarity of constrictive pericarditis, it is not surprising that there are no randomized controlled trials of treatment in this condition. Indeed, the treatment is based on reversing the haemodynamic disturbance by removing the fibrous tissue from around the heart. The operation of pericardial stripping achieves this objective and allows patients to return to a normal active life.
This case demonstrates an unusual presentation of a rare disease. The patient was referred to gastroenterology outpatients because of abnormal LFTs and was eventually diagnosed to be suffering from constrictive pericarditis.
The importance of an accurate clinical assessment is emphasized as this prompted the suspicion of hepatic congestion which, in turn led to the selection of the appropriate investigations for the diagnosis of constrictive pericarditis.
1. Nicki R. Colledge, et al. (2010). Davidson's Principles and Practice of Medicine (21st edn). Churchill Livingstone.
2. Dan L. Longo, et al. (2011). Harrison's principles of internal medicine (18th edn). McGraw-Hill Medical.
3. Parveen Kumar and Michael L. Clark (2009). Clinical Medicine (7th edn). W. B. Saunders.
4. Bertog, S.C. Thambidoiai, S. K., Parakh, K. et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J. Am Coll Cardiol. 2004; 43; 1445-52.
5. Imazio, M., Brucato, A, Mayosi, B.M. et al. Medical therapy of pericardial diseases: part 1: idiopathic and infectious pericarditis. J Cardiovascular Medicine 2010; 11; 712-22
M Hahd Baqai, James Penston, Sangeetha Thanaraj, Deepak Pai, Usman Ahmad
Department of Gastroenterology,
Scunthorpe General Hospital.
DN15 7 BH
Conflict of interest:
No conflict of interest to report.