A 68-yr-old male smoker with a new 1.4-cm peripheral left upper lobe (LUL) lesion suspicious for lung cancer on positron emission tomography imaging presented for video-assisted thoracoscopic resection. The patient gave written consent for publication of the images and video recordings. The preoperative plan was wedge resection with completion lobectomy if the frozen section confirmed malignancy. A baseline electrocardiogram (ECG) showed sinus bradycardia with a right axis deviation and concave ST elevations in V4–V5. This was consistent with a previous ECG and there was no history of angina. A preoperative echocardiogram showed normal biventricular function without valvular or pericardial abnormalities. A retrospective review of a chest X-ray (Figure 1, Panel A) showed signs of left complete pericardial agenesis (CPA) including a straightened and elongated left heart border, radiolucency separating the left ventricle and hemidiaphragm, and loss of the right heart border.1

Figure 1
figure 1

Radiography and direct visualization of CPA. Panel A: Chest radiograph showing leftward and posterior shift of the cardiac silhouette with straightening and elongation of the left heart border (blue arrow), loss of the right heart border (yellow arrow), and radiolucency between the base of the heart and left hemidiaphragm (red arrow). Panel B: Video-assisted thoracoscopy showing the heart in systole. In this still-capture, the left ventricle (black asterisk) and left atrial appendage (yellow asterisk) are visualized anteromedial to the left lung (blue asterisk). CPA = complete pericardial agenesis

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During thoracoscopy, a complete absence of pericardium was observed (Figure 1, Panel B). The cardiac apex moved freely with each contraction (Electronic Supplementary Material, eVideo). Trocars were placed with caution to avoid cardiac injury. Wedge resection of the LUL nodule was performed. Frozen section and subsequent completion lobectomy were not pursued because of the abnormal anatomic appearance of the hilar vessels, non-sustained ventricular arrhythmias precipitated by gentle manipulation of the lung parenchyma, and the possibility of cardiac torsion with additional resection. The postoperative course was uneventful, and final pathology determined a necrotizing granuloma with no malignancy.

Complete pericardial agenesis is usually asymptomatic, with no risk of cardiac herniation or extrinsic compression of the coronary arteries. Defects are found incidentally on imaging, in the operating room, or at autopsy. Diagnosis is challenging because the condition is rare and specific clinical exam findings are lacking. Indirect signs may be present on echocardiography and cardiac magnetic resonance imaging is the preferred imaging modality.1 Left CPA presents a unique challenge in left-sided pulmonary resection. A tendency toward cardiac levorotation might increase risk of cardiac torsion for left-sided lobectomies or pneumonectomy.