Answers

Short answers

1. The jugular venous pressure will be raised—sometimes so much so that the ears waggle—and it will fail to fall—or paradoxically rise—on inspiration (Kussmaul’s sign).

2. Constrictive pericarditis. Cardiac magnetic resonance imaging showed a thickened fibrotic pericardium as a black rim around the cardiac silhouette surrounded by white pericardial fat (fig 2). The normal pericardium is usually barely visible on magnetic resonance imaging.

View larger version (121K): [in this window] [in a new window] [PowerPoint Slide for Teaching]   Fig 2 Cardiac magnetic resonance imaging scan showing a thickened fibrotic pericardium as a black rim around the cardiac silhouette (arrow) surrounded by normal pericardial fat, which is white

3. The condition can be idiopathic or the result of rheumatoid arthritis, cardiac surgery, malignant infiltration (lymphoma, carcinoma of the lung or breast), radiotherapy, tuberculosis, and other chronic infections.

4. It is treated by surgical removal of the pericardium.

Long answers
1. Clinical signs
The clinical sign (box) most likely to be seen on clinical examination is a raised jugular venous pressure—sometimes so much so that the ears waggle—that will fail to fall—or paradoxically may even rise—on inspiration (Kussmaul’s sign).

Facial flushing and hepatic venous congestion on liver biopsy suggest a cardiac cause, and should lead to a careful assessment of the jugular venous pressure. In constrictive pericarditis, both ventricular and atrial filling are restricted, and this results in systemic venous congestion that is indicated by a raised jugular venous pressure. The jugular venous pulse will have a prominent rapid collapsing y descent with a normal or exaggerated x descent, resulting in an M or W shaped jugular venous waveform.¹

Kussmaul’s sign is the paradoxical filling of the neck veins on inspiration, which causes the jugular venous pressure to rise or fail to fall; this was first recognised in patients with constrictive pericarditis.¹ Kussmaul’s sign is a reflection of reduced right ventricular filling compliance. The main causes to consider here are constrictive pericarditis and restrictive cardiomyopathy. The facial flushing in this case is an unusual symptom in constrictive pericarditis and is presumably explained by impaired venous return to a non-compliant right ventricle.

2. Diagnosis
The diagnosis is constrictive pericarditis. The differential diagnosis in this case is:

• Constrictive pericarditis
  
• Budd-Chiari syndrome
  
• Metastatic carcinoid syndrome
  
• Restrictive cardiomyopathy.
  

The results of the liver biopsy suggested hepatic venous engorgement of the liver. This is caused by chronic Budd-Chiari syndrome⁶ or cardiac disease. The raised jugular venous pressure and facial flushing both suggest a cardiac cause and argue against the diagnosis of Budd-Chiari syndrome.

The facial flushing could also be caused by carcinoid syndrome with right heart involvement. This diagnosis is not consistent with the liver ultrasound findings or biopsy result, however, because metastatic deposits would be seen in the liver.

Another important diagnosis to consider is restrictive cardiomyopathy. Constrictive pericarditis and restrictive cardiomyopathy both restrict ventricular filling and have similar presentations, and it can be difficult to differentiate between the two diagnoses. However, constrictive pericarditis causes a distinctive dynamic variation in cardiac chamber pressures during respiration.⁴ The rigidity of the pericardium results in underfilling of the left ventricle during inspiration, and a pressure communication causes increased right ventricular filling. Constrictive pericarditis can therefore be identified by the reciprocal pressure changes between the ventricles seen on echocardiography or cardiac catheterisation.³ In addition, increases in serum brain natriuretic peptide concentrations are often greater in restrictive cardiomyopathy than in constrictive pericarditis.⁷ If the diagnosis is still in doubt then endomyocardial biopsy can be performed.

The patient’s symptoms of fatigue, abdominal distension, and ankle swelling are commonly seen in constrictive pericarditis. Most patients with this condition also have dyspnoea,⁸ but this is not a prominent feature in this case. The lack of dyspnoea makes restrictive cardiomyopathy a much less likely diagnosis.

Constrictive pericarditis is a rare condition where there is scarring and thickening of the pericardium. It sometimes presents to gastroenterologists because of hepatomegaly, ascites, or abnormal liver blood tests. In addition, symptoms are often non-specific, so that diagnosis can be delayed for 12-18 months after the onset of symptoms. It is not until the jugular venous pressure is carefully examined that the diagnosis is considered. Diarrhoea and associated protein losing enteropathy are sometimes seen as a result of gut wall venous engorgement.

3. Causes
The causes of constrictive pericarditis include^{9 10 11 12 13 14}:

• Idiopathic
  
• Previous cardiac surgery
  
• Previous mediastinal radiotherapy
  
• Previous pericarditis
  
• Infection—bacterial (including tuberculosis), viral, fungal, or parasitic
  
• Rheumatoid arthritis (and other connective tissue disorders)
  
• Other—myeloproliferative disorders, malignant infiltration (lymphoma, carcinoma of the lung or breast), chronic uraemia, sarcoidosis, asbestosis, drug induced disorder.
  

Almost any pericardial reaction with chronic inflammation can cause constrictive pericarditis. The current leading identifiable causes of constrictive pericarditis in economically developed countries are previous cardiac surgery (incidence about 0.2% in the 1990s¹⁴), previous radiotherapy, and previous pericarditis. In developing countries the most common cause is tuberculosis.

4. Treatment
Pericardiectomy is the only cure for constriction and is recommended by the European Society of Cardiology.¹⁵ Functional results are excellent in most patients who are suitable for the operation. However, even with selected cases surgical mortality is 6-12%.¹⁵ This proportion may be reduced by excluding patients with extensive myocardial fibrosis or atrophy. The risks and benefits of surgery should be carefully considered, and a conservative approach may be more appropriate, particularly in elderly patients.⁹

Cite this as: BMJ 2008;337:a2042