Effusive Constrictive Pericarditis

Constrictive physiology combined with a pericardial effusion: after the effusion is drained so that intrapericardial pressure falls to about zero, the right atrial pressure fails to fall by at least 50% or to less than 10 mmHg. It reflects visceral (epicardial) and parietal pericardial thickening, so the constricting layer remains after drainage.

• Causes: tuberculosis (commonest in endemic areas), idiopathic or viral pericarditis, radiation, malignancy, post-cardiac surgery, purulent pericarditis, and connective tissue disease.
• It occurs in roughly 5 to 10% of patients drained for tamponade (estimates vary) and is easily missed when the patient seems to be recovering after pericardiocentesis.
• Many cases are inflammatory and reversible with anti-inflammatory treatment (about half within 2 to 3 months), but a persistent or fibrotic form needs surgery, and the operation is more complex than for classic constriction because the epicardium must be removed.

• Persistent signs of right heart failure after drainage of an effusion: raised jugular venous pressure with a prominent y descent, Kussmaul sign, peripheral oedema, ascites, hepatomegaly, fatigue and dyspnoea.
• Before drainage: the picture of tamponade or large effusion with raised filling pressures.
• Differential diagnosis: tamponade with incomplete drainage, loculated effusion or clot, restrictive cardiomyopathy, tricuspid regurgitation, right ventricular failure, pulmonary hypertension, cirrhosis, and nephrotic syndrome.
• Complications: refractory heart failure, hepatic congestion, protein losing enteropathy, atrial fibrillation, renal impairment, and cachexia.

• Invasive haemodynamic measurement is definitive: right atrial and intrapericardial pressures measured during pericardiocentesis. Constriction is confirmed when right atrial pressure stays 10 mmHg or more (or falls by less than 50%) after intrapericardial pressure has been lowered to zero or negative values, with early diastolic dip-and-plateau.
• Echocardiography after drainage: persistent plethoric inferior vena cava, septal bounce, respiratory shift of the septum, thickened pericardium, mitral inflow variation greater than 25%, hepatic vein diastolic flow reversal, and preserved or raised medial annular e' velocity.
• Cardiac MRI or CT: pericardial thickening, calcification, and late gadolinium enhancement indicating inflammation.
• Tests for the cause: tuberculosis tests on blood, sputum and pericardial fluid (Xpert MTB/RIF, culture, adenosine deaminase greater than 40 U/L), HIV test, CRP, antinuclear antibody, cytology, and renal and thyroid function.

• Drain the effusion (pericardiocentesis with a pigtail catheter) and measure pressures to recognise the condition early.
• Anti-inflammatory therapy for the reversible form:
  • Ibuprofen 600 mg 8-hourly (maximum 2400 mg/day) or aspirin 750 to 1000 mg 8-hourly for 2 to 4 weeks then taper; omeprazole 20 mg daily.
  • Colchicine 0.5 mg twice daily (once daily if weight less than 70 kg) for 3 to 6 months.
  • Prednisolone 0.25 to 0.5 mg/kg/day for 2 to 4 weeks then slow taper, when NSAIDs are inadequate or contraindicated (evidence is limited).
  • Reassess at 2 to 3 months with symptoms, CRP, echocardiography and, if available, MRI.
• Tuberculous disease: rifampicin 10 mg/kg (maximum 600 mg), isoniazid 5 mg/kg (maximum 300 mg), pyrazinamide 25 mg/kg (maximum 2 g), ethambutol 15 mg/kg (maximum 1.2 g) daily for 2 months, then rifampicin and isoniazid for 4 months, with pyridoxine 25 to 50 mg daily. Prednisolone may reduce constriction, but it has been linked to excess HIV-related cancers, so avoid it in untreated HIV; evidence is limited.
• Symptom control: furosemide 40 to 80 mg daily with spironolactone 25 to 100 mg daily, using minimal doses to avoid hypotension; salt restriction; avoid high dose beta blockers.
• Surgery: pericardiectomy when constriction persists after 2 to 3 months of medical treatment, or earlier when there is progressive failure. Complete removal of both parietal and visceral (epicardial) layers is needed. Operative mortality is about 10% or more, higher than for classic constriction, and function improves in most survivors.
• Special groups:
  • HIV: antiretroviral therapy; steroids only with specialist advice.
  • Children: tuberculosis is the leading cause in endemic areas.
  • Renal failure: dialysis with minimal heparin; the effusion may be uraemic.
  • Radiation: worse outcome, with associated valve and myocardial disease.
• Red flags: failure of jugular venous pressure to fall after drainage, recurrent effusion, refractory oedema or ascites, rising creatinine, cachexia.
• Follow up: review at 2 to 4 weeks, 3 months, 6 months, then yearly; repeat echocardiography at each visit.