Congenital pericardial defects (CPDs) are rare congenital abnormalities characterized by the complete or partial absence of the pericardium. They are often asymptomatic and discovered incidentally through imaging. Some individuals can experience non-specific symptoms, whilst others can have serious complications. The gold standard for diagnosing pericardial defects is cardiac MRI. Management is case-dependent and usually reserved for partial defects. Here, we present a case of a 57-year-old male who presented with recurrent chest pain and was found to have partial pericardial defect, a diagnosis missed on prior imaging, and discuss the diagnosis and management.

The principal roles of the pericardium include protection from microorganisms, prevention of cardiac friction, and restriction of unlimited dilation of the heart. In the case of a congenital pericardial defect in which structural, electrophysiological, and haemodynamic abnormalities manifested during postural changes, we propose another indispensable pericardial function of cardiac central anchorage. A 29-year-old man with atypical chest pain was referred to our hospital. Electrocardiography revealed fluctuations in the QRS transitional zone, electrical axis, and atrial polarity with body posture. Echocardiography revealed a far dorsally displaced heart, paradoxical motion of the interventricular septum (IVS), and hyperdynamic motion of the posterior wall in the left lateral decubitus position, which normalized to the right lateral decubitus position, along with significant haemodynamic alterations. Multidetector-row computed tomography revealed a complete absence of the left pericardium. When pericardial fixation is impaired, the heart falls dorsally owing to gravity in the left lateral decubitus position, resulting in right ventricular overstretch and enlargement, which, in turn, results in compression of the left ventricle via a leftward shift of the IVS and a consequent reduction in cardiac output. Moreover, the energy generated by the myocardium, which should normally be concentrated only on blood ejection, would be distributed between the energy used for ejection and that used for the leap-up movement of the heart, reducing its energy efficiency. Through detailed observation of the congenital pericardial defect, haemodynamic insights into the important functions of the pericardium, which were not visible through static observation, were obtained.

Congenital absence of the pericardium is a rare condition, usually detected incidentally and often asymptomatic. Symptomatic cases are uncommon, and they pose diagnostic and therapeutic challenges. A 65-year-old man presented with exertional chest discomfort that varied with body position. Transthoracic echocardiography suggested a pericardial defect. Chest computed tomography and cardiac magnetic resonance imaging confirmed complete congenital absence of the pericardium. Thoracoscopic minimally invasive repair without cardiopulmonary bypass or blood transfusion relieved his symptoms. This case highlights the diagnostic value of multimodal imaging and the feasibility of minimally invasive repair in older patients with symptomatic pericardial agenesis. Although complete pericardial agenesis is often asymptomatic, surgery may be warranted in symptomatic cases, even in older patients. Thoracoscopic minimally invasive repair without cardiopulmonary bypass or blood transfusion is a feasible approach.

Congenital absence of the pericardium (CAP) is a rare anomaly, often silent and easily mistaken for other cardiac conditions. In this case report, a 72-year-old man without cardiovascular risk factors presented with intermittent, non-exertional left-sided chest discomfort over two weeks. His ECG showed absent R-wave progression, raising suspicion of coronary artery disease. Transthoracic echocardiography revealed a poorly defined cardiac silhouette with a leftward shift. The stress echo was negative for inducible ischemia. A chest X-ray was done to look into the cause of the displaced apex and showed loss of the right heart border, an elongated left cardiac contour, and normal lungs with a central trachea. The CT confirmed the complete absence of the left pericardium. No high-risk features of cardiac herniation were observed. Given the benign nature of complete unilateral pericardial absence and absence of complications, the patient was managed conservatively and reassured for his complaints. This case report highlights that recognition of characteristic imaging signs, especially on chest X-ray and CT, is crucial for distinguishing pericardial agenesis from other cardiothoracic conditions. Awareness of this rare entity aids in preventing unnecessary interventions and ensuring optimal patient care.

We report a case of complete pericardial agenesis (PA). PA is a rare congenital anomaly characterized by complete or partial defects in the pericardium [1]. Cardiac magnetic resonance imaging (MRI) is key in diagnosing this condition.