Question
A 41-year-old woman develops gradually worsening abdominal distension and bilateral leg oedema over several months. She previously underwent surgical removal of a thymic tumour associated with myasthenia gravis, followed by mediastinal radiotherapy because the resection margins were involved. On examination, the JVP is elevated, the liver is enlarged and visibly pulsatile, and there is peripheral oedema. The lungs are clear and heart sounds are soft. Chest radiography shows a small amount of calcification around the pericardium.
Which treatment offers the best definitive management?
A. Oral spironolactone
B. Therapeutic pericardiocentesis
C. High-dose oral prednisolone
D. Repeated therapeutic paracentesis
E. Pericardiectomy
Answer
Pericardiectomy
Explanation
This patient has constrictive pericarditis, most likely secondary to previous mediastinal radiotherapy.
The important clues are elevated JVP, peripheral oedema, ascites/abdominal swelling, hepatomegaly with a pulsatile liver, clear lungs despite marked systemic venous congestion, and pericardial calcification. In constrictive pericarditis, a rigid, non-compliant pericardium prevents normal diastolic expansion of the ventricles. This causes elevated systemic venous pressure and predominantly right-sided heart failure.
Previous chest or mediastinal radiotherapy is an important cause of chronic constrictive pericarditis. Calcification may support the diagnosis, but extensive calcification is not required; patients can have significant constriction despite minimal calcification on chest X-ray.
The definitive treatment for chronic symptomatic constrictive pericarditis is surgical pericardiectomy, in which the constricting pericardium is removed. Diuretics can temporarily reduce oedema and ascites, particularly while awaiting surgery or when surgery is unsuitable, but they do not correct the underlying mechanical problem.
Pericardiocentesis is not the treatment because the problem is not simply accumulation of pericardial fluid, as occurs in cardiac tamponade. Similarly, steroids are useful only in selected inflammatory or transient forms of pericardial disease and are not definitive therapy for established chronic fibrotic constriction.
A useful distinction from cardiac tamponade is the JVP waveform. In constrictive pericarditis, the x and prominent y descents are preserved, because early ventricular filling occurs rapidly before suddenly stopping when the rigid pericardium limits further expansion. In cardiac tamponade, the y descent is absent or blunted because ventricular filling remains impaired throughout diastole.
Other classic findings of constrictive pericarditis include Kussmaul’s sign, where the JVP rises or fails to fall during inspiration, and a pericardial knock, an early diastolic sound produced by abrupt cessation of ventricular filling.
Summary for quick exam revision
Constrictive pericarditis occurs when a thickened, fibrotic or calcified pericardium restricts ventricular filling during diastole. Important causes include previous pericarditis, tuberculosis, cardiac surgery and mediastinal radiotherapy. Patients typically develop features of predominantly right-sided heart failure such as raised JVP, peripheral oedema, ascites and hepatomegaly. The lungs may remain relatively clear despite severe systemic venous congestion. A pulsatile enlarged liver may be present because of elevated right-sided filling pressures. The JVP classically shows a prominent y descent due to rapid early diastolic filling followed by abrupt cessation of filling. The x descent is also preserved or prominent. Kussmaul’s sign is typically positive, meaning the JVP rises or fails to fall during inspiration. A pericardial knock may be heard shortly after S2. Chest X-ray may demonstrate pericardial calcification, although its absence does not exclude the diagnosis. Echocardiography and cardiac imaging help demonstrate ventricular interdependence and abnormal diastolic filling. Cardiac tamponade differs because its y descent is absent, whereas constrictive pericarditis has prominent x and y descents. Pulsus paradoxus is much more typical of tamponade than chronic constriction. Diuretics can provide temporary symptomatic relief from oedema and ascites. However, they do not remove the mechanical restriction produced by the diseased pericardium. In a patient with persistent symptomatic chronic constrictive pericarditis, pericardiectomy is the definitive treatment.