Teaching Case

Female 69-year old patient with a systolic heart rumor over the pulmonary valve.

The patient was admitted for a change of the tip of her pacemaker, which was implanted because of bradycardia after ablation of an accessory conduction pathway in 1992. Family history revealed that the patient´s mother had died in the age of 43 because of non-ischemic heart disease. Clinical examination showed a systolic heart rumor over the pulmonary heart valve. Pre-interventional chest x-ray shows calcifications following the contour of the heart on the bottom of the right ventricle in the postero-anterior view and retrosternal in the lateral view. Echocardiography showed an enlarged left atrium and a thickened, partially calcified pericardium.

These imaging findings are typical for a calcified pericarditis, a clinical entity today mainly of idiopathic origin. Other causes are tuberculosis, viral infection, collagenosis, uremia, neoplasms and secondary to previous closed chest trauma, heart surgery and radiation therapy of the mediastinum. Scary adhesions of the pericardium and additional calcifications disturb diastolic relaxation as well as systolic contraction of the right heart and therefore cause elevated mean right-atrial and pulmonary wedge pressures. Possible symptoms include dyspnea, chest pain, fatigue and peripheral edema. Diagnosis is usually based on the finding of calcifications of the pericardium in chest x-ray or computed tomography (approximately one third to one-half of cases demonstrate calcifications), hemodynamical relevance is then evaluated by echocardiography. The characteristic site of pericardial calcifications is over the right-sided cardiac chambers and in the atrioventricular grooves. A calcified former left ventricular aneurysm is the only radiological differential diagnosis, mainly presenting with calcifications in the apex of the left ventricle. Oral steroids have been reported to improve the clinical situation but the definitive form of therapy is pericardial stripping.