Authors

• Blanka Glavas Konja — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-1134-4856
• Vlatka Reskovic Luksic — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-4721-3236
• Josko Bulum — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
• Martina Lovric Bencic — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0001-8446-6120
• Zvonimir Ostojic — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-1762-9270
• Aleksander Ernst — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0003-0742-3359
• Jadranka Separovic Hanzevacki — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-3437-6407

Keywords

constrictive pericarditis, thrombosis, embolism, thrombophilia, atrial fibrillation

DOI

Full Text

## Background Constrictive pericarditis is a rare disease but can cause diagnostic problems. The diagnosis can be even more difficult if combined by pulmonary emobolism (1, 2). We present the case of constrictive pericarditis complicated with several thromboembolic incidents. ## Case Presentation A 58-year-old was admitted to Clinical Hospital with symptoms of dyspnoea, ortopnoe, fatigue and signs of dominantly right heart congestion. As a 6-year old child he had left sided pleuritis. Later, he was working at shipyard in the region close to asbestos factory. Four years before admission he was accidentally diagnosed atrial fibrillation. Anticoagulant therapy with dabigatran was started. Electrocardioversion was done successfully for three times and finally radiofrequency ablation had been planned when patient felt breathless suddenly. He was admitted to local hospital where thoracic MSCT showed bilateral incapsulated pleural effusion, bronchial deformities and a small pericardial effusion that measured 0.7 cm. Right sided pneumonia was suspected so patient was treated with antibiotics but with no benefit. MSCT pulmonary angiography did not show pulmonary embolism, but verified worsening of pericardial effusion, hepatic congestion and ascites. PET CT showed no pathology. Fiber optic bronchoscopy found only nonspecific mucopurulent substrate. Quantiferon test was negative. Dyspnoea was worsening, as well as pericardial effusion. Anticoagulant therapy had been stopped for a while. Echocardiography examination found dilatation of both atria with an indirect signs of high right atrial pressure and constrictive hemodynamic and thrombus in right atrium (Figure 1). Control CT showed multiple thrombotic masses in right atrium, segmental pulmonary arteries, left atrial auricular. Doppler did not show peripheral vein thrombosis. Anticoagulant therapy was started again. Searching for thrombophilia found normal FV, FII, MTHFR (CC gene type), polymorphism (4G/5G) for PAI-I, and insertion ACE genotype. Polyserositis was suspected so corticosteroid wad added to therapy. Heart MR confirmed constrictive pericarditis and made suspicious of thrombotic masses in both atria. Meanwhile patient had another thromboembolic episode; inferior and superior caval vein thrombosis, hepatic and renal veins thrombosis, and right iliac artery embolism. Iliac artery trombendarterektomia was successfully done. Patient was transferred to the tertiary hospital for the diagnostic confirmation of constrictive pericarditis. Figure 1. Echocardiography shows thrombus in right atrium at the entrance of superior vena cava. At the admission, patient was afebrile, cachectic, tachypneic, orthopnoic and hypotensive with signs of hepatomegaly, ascites, peripheral oedema and jugular distension. Decubital ulcer stared to form at sacrum. Fatigue end exhaustion was in progression. As PET CT showed intensively metabolic active pericardial region, antituberculotic therapy was started despite of negative Quantiferone test with partial benefit (Figure 2). Heart catheterization was performed, but typically four chamber equalization of pressure was not confirmed (Figure 3). Pulmonary artery pressure was normal, and right atrial pressure was high. Patient was re-evaluated and transferred to cardiac surgery department for pericardiectomy. Procedure risk was classified as very high. Figure 2. PET CT. Figure 3. Ventricular pressures with left ventricular dip-and-plateau pattern. Pericardiectomy has been done partially because of heavily adherent thick pericardium. Rehabilitation procedure was started, and clinically status of the patient was improving gradually. Samples of pericardium were sent to pathology department, but the histological examination did not show specific inflammation. Antituberculotic therapy was stopped. Patient was discharged in improved condition, without dyspnoea or oedema. ## Conclusion The diagnosis of constrictive pericarditis is difficult. The cardiac catheterization with intracavitary pressure curves analysis is considered as a gold standard. Typically equalization within 5 mmHg range difference of end-diastolic pressure in all chambers has low sensitivity and specificity. The diastolic curve profile in both ventricles that represents dip-and-plateau pattern is not present always (3, 4). Right atrial thrombus associated with pericarditis is very rare. (5) Thrombosis of other vascular structures associated with pericarditis is even more rare. Cases of chronic inflammatory diseases as Behcet's disease or hematogic malignant diseases have been described. (6) We have presented the case of idiopathic constrictive pericarditis combined with thrombophilia and atrial fibrillation. Every of this conditions predispose to thrombi formation. Pericardiectomy followed by anticoagulant therapy is optimal treatment option. (7)

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