P162 AN UNUSUAL CASE OF RIGHT VENTRICULAR DILATATION

Abstract

Abstract We describe the case of a 58 years old man presenting to our Cardiology Clinic because of palpitations, after a complete evaluation performed elsewhere. His symptoms showed a progressive worsening althoug present since at least 15 years. No other significant information came from his clinical history. Both his physical examination and EKG did not show abnormalities. An isolated mild enlargement of the right ventricle was seen by ecocardiography. 24–hour ECG recording revealed few ventricular ectopic beats and some couplets. An ergometric stress test did not suggest an impaired coronary reserve. Cardiac MRI was performed and the report described again a mild right ventricular enlargement without any other abnormalities. Both wall motion and late enhancement were analyzed and no pathologic behavior was detected. The patient was admitted to our Clinic for a second opinion. The analysis of the patients‘ chest x–ray showed a “tongue–like” presence of pulmonary tisse between the aortic knob and the pulmonary trunk (arrow), and a less pronounced right mediastinal border (Fig 1). A careful analysis of the Cardiac MRI showed a leftward rotation of the heart, a pronounced fatty area between the heart and the anterior thoracic wall. Finally it can be appreciated the partial absence of the pericardium limited to its anterior portion. The partial absence of pericardium is a rare congenital condition often diffucult to detect. Its prevalence ranges from 0.007–0.015% in autoptic and 1–0.044% in surgical series. Usually isolated it can accompany other malformations. It may be complete or partial, and sometimes so limited in extension to determine a sort of foramen that may strangle the heart itself. While this condition is usually benign when the heart is constricted through the above mentioned pericardial orifice a sudden death may occurr. Symptoms may be absent but sometimes the absence of pericardium is associated with an atypical chest pain, often piercing, sometimes very intense. In our patient no condition at risk was detected. We excluded other potential causes for his ventricular arrhythmyas (particular attention was posed to excluding ARVC as this suspicion was the one that prompted all the exams that the patient had previously performed) . He will be followed regularly without any specific measure.