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Idiopathic acute pericarditis rapidly evolving to constrictive pericarditis

A 36 years old male patient was admitted to hospital for acute pericarditis and successfully treated with ibuprofen, colchicine and prednisone. The etiology search was inconclusive and the final diagnosis was idiopathic acute pericarditis. In a few months the patient developed dyspnoea, peripheral oedema and jugular distension. Echocardiography showed thickened pericardium, with signs of constriction and no pericardial effusion. Cardiac magnetic resonance showed diffuse pericardial thickening and confirmed the hypothesis of constriction. Cardiac catheterization showed square root sign and right and left ventricles telediastolic pressure equalization. Pericardiectomy performed eleven months after the acute attack induced complete symptomatic relief. Histology showed non-specific chronic diffuse inflammation and fibrosis. In conclusion we describe here for the first time a patient who clearly evolved from acute idiopathic pericarditis to constriction over a few months.
Constrictive pericarditis can occur after virtually any pericardial disease process (1); in the past it has been traditionally considered a possible feared complication of idiopathic recurrent pericarditis, but large studies and systematic reviews have been reassuring regarding the incidence of constrictive pericarditis in this clinical setting (2,3).
Pericardial Disease


A 36 years old male patient, 70 kg weight, was admitted to hospital with acute pericarditis (typical chest pain, fever, diffuse ST elevation, slight PR depression, small pericardial effusion); C-reactive protein (CRP) was elevated (89.4 mg/L). Therapy with ibuprofen 1800 mg daily was started and continued for the following weeks but despite resolution of chest pain, CRP remained elevated and pericardial effusion modestly increased.

Colchicine 1 mg daily was added, but was then withdrawn for diarrhoea, and prednisone 37.5 mg was started; diuretics were added within one month from presentation, due to hydrosaline retention with weight increase, and beta-blockers were prescribed for persistent tachycardia. Extensive infective and immunologic assessment was carried out but gave negative results. After 6 months the patient was complaining of dyspnoea, peripheral oedema and jugular distension. Echocardiography showed a thickened pericardium with Doppler findings suggestive for constriction (transmitral flow variation >25% during respiration).

   Anatomy: morphologic T1-weighted imaging showing circumferential pericardium thickening without effusion (arrows)

(Figure 1. Anatomy: morphologic T1-weighted imaging showing circumferential pericardium thickening without effusion (arrows)











Cardiac magnetic resonance (CMR) showed diffuse pericardial thickening (Figure 1), pathologic ventricular interdependence with inspiratory septal flattening on cine real-time imaging, characteristic of constrictive pericarditis (4,5) (Figure 2) and enhanced visceral pericardium after gadolinium (Magnevist, 0.15 mmol/kg) injection, suggestive of active inflammation. Cardiac catheterization confirmed pericardial constriction (square root sign and right and left ventricle telediastolic pressure equalization), and coronary angiography revealed normal coronary arteries. Eleven months after the first hospital admission for acute pericarditis, extensive pericardiectomy was performed, with complete symptomatic relief and regression of signs of constriction. Histology showed non-specific chronic diffuse inflammation and fibrosis.

Physiology: real-time cardiovascular magnetic resonance images

(Figure 2. Physiology: real-time cardiovascular magnetic resonance images during expiration (left panel) and inspiration (right panel). Ventricular interdependence is evident, with inspiratory septal flattening (right panel, arrow))

Constrictive pericarditis more commonly presents directly with the typical picture of congestive heart failure, eventually years after chest radiotherapy or cardiac surgery; it has been proposed that more rarely it may present as the progressive subacute evolution of an acute idiopathic pericarditis (3). This second presentation is sometimes erroneously labelled as an idiopathic “recurrent” pericarditis, but should more properly be considered as a single, first episode of acute idiopathic pericarditis directly evolving into constriction. Constriction may sometimes be transient (10-20% of cases) (6), with resolution within few months.

CMR can be a very useful tool in the diagnostic work up of patients with suspected constrictive pericarditis, thanks to its capability to thoroughly depict both the morphologic aspects of the pericardium and the dynamic physiologic consequences of pericardial constriction; moreover, the administration of gadolinium can identify persisting inflammation causing pericardial enhancement (4).

Timing of surgery is important, and pericardiectomy should be performed by surgeons with experience with this procedure. Long-term survival after pericardiectomy is inferior to that of an age-matched and sex-matched population. In the Mayo Clinic series, the 5-year and 10-year survival was 78 and 57%, respectively (1). Idiopathic constrictive pericarditis had the best prognosis (7-year survival 88%) followed by postsurgical (66%) and postradiation constriction (27%).

Conclusion:

In conclusion constrictive pericarditis may rarely complicate acute pericarditis. We describe here for the first time a patient who clearly evolved from pain and fever to constriction over a few months; integrated diagnostic cardiac imaging (echocardiography, CMR, cardiac catheterization) was very useful in assessing the pathophysiology and damage entity, and pericardiectomy was curative.

References


  1. Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB, et al. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999; 100:1380–1386.
  2. Brucato A., Brambilla G., Moreo A. et al. Long-term outcomes in difficult-to-treat patients with recurrent pericarditis Am J Cardiol 2006; 98; 267-271
  3. Imazio M., Brucato A., Adler Y.et al. Prognosis of idiopathic recurrent pericarditis as determined from previously published reports Am J Cardiol 2007; 100: 1026-1028
  4. Francone M., Dymarkowski S., Kalantzi M. et al. Assessment of ventriculat coupling with real-time cine MRI and its value to differentiate constrictive pericarditis from restrictive cardiomypathy Eur J Radiol 2006 16: 944-951
  5. Ariyarajah V, Jassal DS, Kirkpatrick I, Kwong RY. The utility of cardiovascular magnetic resonance in constrictive pericardial disease. Cardiol Rev 2009; 17:77–82.
  6. Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004; 43:271–275.

Notes to editor


Presented by: Patrizia Pedrotti(a) MD, Antonio Brucato (b) MD, Massimo Imazio(c) MD, Silvia Maestroni(b) MD, Caterina Simon(d) MD, Paolo Ferrazzi(d)  MD, Alberto Roghi MD(a)Cardiovascular Magnetic Resonance Imaging Unit (a) , Ospedale Niguarda Ca’ Granda Milano Italy;Internal Medicine (b), Cardiac Surgery (d), Ospedali Riuniti, Bergamo; Cardiology Department (c), Maria Vittoria Hospital, Torino, Italy
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.

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