Congenital Absence of the Pericardium
A 19-year-old woman was referred to our clinic for further evaluation of cardiomegaly on chest radiography. She had no chest pain, shortness of breath, or other specific signs and symptoms. Her past medical history and family history were unremarkable. She had not taken any medications and denied use of illicit drugs and tobacco or alcohol abuse. On cardiac examination, a regular heart rhythm was heard without heart murmur. A 12-lead electrocardiogram (ECG) showed normal sinus rhythm, with a heart rate of 62 bpm and right deviation of the heart axis (). The chest radiograph revealed that the heart had bulging contour of the left superior cardiac border and leftward shift (). The echocardiography showed a globe-shaped heart and bulbous ventricle due to suspension of the heart from its basal pedicle (). It also revealed mitral valve prolapse. The estimated left ventricular ejection fraction was 65%. Chest computed tomography (CT) revealed outpouching of the right ventricle (RV), which contacted the left chest wall, and interposition of the lung between the aortic arch and prominent pulmonary trunk (). The patient was diagnosed with congenital absence of the left pericardium.\nA 56-year-old man was referred to our clinic for ECG abnormality. He had no chest pain, dyspnea, or other specific signs and symptoms. His past medical history and family history were unremarkable. He neither took medications nor used tobacco or alcohol. On examination, his blood pressure was 126/76 mmHg. A systolic ejection-type murmur was present with regular heart rhythm. A 12-lead ECG showed sinus rhythm with a heart rate of 59 bpm, an incomplete right bundle branch block, and a slight increase in left ventricular voltage (). Chest radiography revealed flattening of the left heart border and a lucent area between the aorta and pulmonary artery due to lung interposition (). The patient underwent an echocardiogram and chest CT because of the abnormal chest radiography, murmur, and ECG. The echocardiography showed a left laterally displaced left ventricle (LV) apex with a swinging motion that was highly suggestive of congenital absence of the pericardium (). The RV cavity was enlarged as 38 mm and demonstrated paradoxical septal motion. LV systolic function was normal. The chest CT revealed nonvisualization of the pericardium and leftward displacement of the entire heart with mild RV dilatation (). The patient was diagnosed with congenital absence of the pericardium.\nA 71-year-old male patient presented with a history of dyspnea on exertion. He reported atypical chest pain and shortness of breath. He suffered from asthma and was taking medication. Medical and family histories were otherwise unremarkable. He was a current, 100 pack-year smoker. On examination, his blood pressure was 120/70 mmHg. Regular heart rhythm without murmur and clear breath sounds were heard on auscultation. A 12-lead ECG showed normal sinus rhythm, with a heart rate of 73 bpm and left deviation of the heart axis (). Q-waves were seen in leads II, aVF, V1, V2, and V3. Chest radiography showed bulging contour of the left superior cardiac border (). Due to his symptoms, abnormal chest radiography, and ECG, the patient underwent an echocardiogram and heart CT. The echocardiography revealed a laterally displaced LV apex that was highly suggestive of congenital absence of the pericardium (). It also showed an enlarged LV cavity with mild global hypokinesia without regional wall motion abnormality. Valvular morphology and function were normal, and the estimated LV ejection fraction was 47%. The cardiac CT revealed absence of the pericardium at the left side of the heart without evidence of associated anomaly such as atrial septal defect or ventricular septal defect (). The patient was diagnosed with congenital absence of the left pericardium.