Left-side pericardial agenesis: Putting the pieces together

A 56-year-old woman complained of atypical chest pain. At initial diagnostic workup, chest radiograph and transthoracic echocardiogram depict specific findings that raised the suspicion of pericardial agenesis. Cardiac computed tomography showed extreme levoposition of the heart and interposition of lung parenchyma between the aorta and the pulmonary artery. Those findings were consistent with the diagnosis of left-side pericardial agenesis, which was subsequently confirmed in cardiac magnetic resonance study. Left-side pericardial agenesis is rare and can result in nonspecific symptoms. An integrated multimodality imaging approach may provide incremental value on diagnosis approach. Its benign prognosis allows a conservative approach.