Associazione Nazionale Medici Cardiologi Ospedalieri



Agenesis of the left pericardium: report of a case

Placci Angelo Parma(Parma) – Cardiologia AOU PARMA | Palumbo Alessandro Parma(Parma) – Radiologia AOU Parma | Suma Sergio Parma(Parma) – Cardiologia AOU Parma

We report a case of left pericardial agenesis in a 61 year old man presenting palpitations, without syncope. Clinical picture was not specific on clinical field: atypical chest pain, systolic murmur 2/6 Levine on the second left interspace were found.

ECG showed sinus rhythm, clockwise deviation of axis in the precordial leads and incomplete right bundle branch block. Frequent isolated polymorphic premature extrabeats.

Holter ecg was perfomed: frequent polymorphic extrabeats (9834/24 ore) with some couplets and triplets, without sustained ventricular tachycardia episodes.

Starting metoprololo 100 mg per day the arrhythmic burden decreases significantly.

Chest X-ray showed marked levocardia and dilatation of pulmonary trunk. Levocardia increased with the patient on the left side. Levoposition of the heart, lung interposition between the diaphragm and the base of the heart and between the aorta and pulmonary artery

Echocardiography showed a classic abnormal swinging motion of the heart in the chest, a paradoxical interventricular septal movement, apparent right-sided heart enlargement, with unusual bulging of the apex of the right ventricle.

Magnetic resonance imaging (currently the gold standard for diagnosing) confirmed the absence of the left pericardium, and a left pericardial agenesia diagnosis was performed

Patients have a similar life expectancy to those without pericardial defects; however in certain cases, herniation and strangulation of cardiac chambers can be life threatening and lead to sudden cardiac death.

We analyze the diagnostic problems and the prognostic doubts of this rare congenital anomaly.