Sudden cardiac death in a case of undiagnosed pericardial agenesis.

Rev Esp Cardiol. 2010;63(9):1100-9 1103 chest pain to his parents before going to sleep. An EKG performed two years before the sudden death showed normal SR, LV hypertrophy, negative T wave in leads V5 and V6, positive/negative T wave in lead V4, no lead I available, real QT of 0.40 and a correct QT 0.3. After 6 months an EKG showed: normal SR, artifact in lead V6, negative T wave in lead I, VL, V5, positive/negative T wave in lead V4, real QT 0.40 and corrected QT 0.37 (Figure 1). After another 6 months, an EKG showed normal SR, LV hypertrophy, flattening of T wave in leads I, V5 and V6, real QT 0.36 and correct QT 0.34. A 2-dimensional echocardiography was performed showing normal 4-chambers dimensions and septum. Six months before the sudden death, an EKG showed: normal SR, positive T waves in all the leads, high voltage of QRS in the precordial derivation, real QT 0.32, corrected QT 0.32. Autopsy showed a CAP with a “tongue” of pericardium on great vessels. The heart was completely caved into the left hemi-thorax and interposition of lung between the left hemidiaphragm and inferior border of the heart was observed (Figure 2 A-C). The heart was slightly dilated, 460 gr in weight; the LV wall thickness was normal without septal asymmetry. Coronary arteries showed no stenosis or thrombotic occlusion. The valvular apparatus was normal. Patchy fibrosis and foci of contraction band necrosis in sub-endocardial layers (Figure 2 D-E) were microscopically detected. In some fields, the myofibers were stretched and an interstitial margination of polymorphonuclear leukocytes was observed (Figure 2 F). The parietal pericardial appendage showed an increased thickness and neovascularisation. The toxicological analyses were negative. In patients with absence of the pericardium, EKG often shows incomplete right bundle-branch block with poor R-wave progression as a result of the leftward displacement of the heart. In this case the patient was in sinus rhythm; presence of right axis deviation, right bundle-branch block pattern, and leftward displacement of the transition zone in the precordial leads were not recorded.2 T-wave abnormalities at standard EKGs were considered aspecific and not worthy of further diagnostic tests. Pericardial defects are associated with other congenital abnormalities in up to 50% of patients, mostly cardiac and pulmonary in nature. Chest wall abnormalities have also been described.3 Genetic factors may play a greater, as yet undiscovered, aetiological role in CAP. CAP usually has an excellent prognosis, but sometimes the edge of the defect may compress the great vessels, which may affect filling and systolic function. Involvement of coronary arteries may lead to myocardial ischemia and sudden death, while involvement of the phrenic nerve may contribute to the symptoms. Ramón López-Palop, Pilar Carrillo, Araceli Frutos, and Alberto Cordero Servicio de Cardiología, Hospital Universitario San Juan de Alicante, San Juan de Alicante, Alicante, Spain