Objective Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%–0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition. Methods We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed. Results Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5–144.9 months) and all remained asymptomatic. Conclusion CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.

https://openheart.bmj.com/content/openhrt/7/1/e001103.full.pdf

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects

Congenital absence of the pericardium is a very rare entity that is usually asymptomatic and hence difficult to diagnose. However, cases of sudden death have been reported in patients with partial pericardial defects (even asymptomatic ones), and such patients require surgical treatment. We report the case of a 17-year-old patient with complete pericardial agenesis (diagnosed by chance during a cardiological consultation) and briefly review the radiological findings of this entity.

Congenital complete absence of pericardium in a young woman with non‐specific symptoms

https://doi.org/10.1016/j.radcr.2022.08.075

Congenital Pericardial Agenesis presenting as non-specific chest pain: A Case Report

Congenital absence of the pericardium is a rare condition, which can be isolated, but most frequently associated with other cardiac or extracardiac congenital anomalies. Generally, most the patients with pericardial absence are asymptomatic. In some cases, partial pericardial defects can be life threatening, because of heart and lung herniation and ventricular entrapment. The pericardium has a supporting and protecting role for the heart and the proximal great vessels (ascending aorta and pulmonary arterial trunk), so absence of the pericardial layers allows important thoracic displacement of the heart. Diagnosis is established by the relevant radio-imaging findings suggestive of complete absence of the pericardium, with characteristic cardiac silhouette abnormalities. We present a case with complete absence of the left pericardium in an asymptomatic young male patient. A review of literature focusing on congenital pericardial defects and the radiological findings of this condition, focused on cardiacMR imaging, is also presented.

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Congenital Complete Absence of the Left Pericardium

Phrenic nerve provides the major motor supply to diaphragm. Various anatomical variations in the course and distribution of the phrenic nerve have reported before. Here we report a rare bilateral asymmetric variation in the roots of origin of the phrenic nerve and absence of fibrous pericardium in an old male cadaver. Specifically, the right phrenic nerve was arising from the upper trunk of the brachial plexus (C5) and the left side nerve originated from the supraclavicular nerve and a tiny branch from C5. In the same cadaver both sides phrenic nerve were buried in the mediastinal pleura. Another interesting finding was absence of the fibrous pericardium. To the best of our knowledge the presented case showed a very rare variation in the roots of origin of the phrenic nerve accompanied with pericardial anomaly which has been less reported. We think such case is of practical importance during supraclavicular block during anesthesia. Â© 2016, Brazilian Society of Anatomy. All rights reserved.

/pdf/bilateral-variations-in-the-roots-of-origin-of-the-phrenic-4b8o85v3wr.pdf

Bilateral variations in the roots of origin of the phrenic nerve and absence of fibrous pericardium in an old male cadaver

Congenital absence of the pericardium is a rare cardiac condition, which can be either isolated or associated with other cardiac and extracardiac anomalies. There are six different types, depending on the severity of the involvement. Most of the patients with this defect are asymptomatic, especially the ones with complete absence of the pericardium. However, some patients are symptomatic, reporting symptoms that include chest pain, palpitations, dyspnea, and syncope. Diagnosis is established by the characteristic features on chest X-ray, echocardiogram, chest computed tomography (CT), and/or cardiac magnetic resonance imging (MRI). We present here a case of a 23 year-old-male, who presented to our hospital with complaints of pleuritic chest pain and exertional dyspnea, of a two-week duration. He was physically active and his past history was otherwise insignificant. His chest CT with contrast was interpreted as showing evidence of multiple emboli, predominantly in the left lung, and he was started on a heparin and warfarin therapy. A repeat chest CT with contrast three weeks later showed no significant change from the previous CT scan. Both scans showed that the heart was abnormally rotated to the left side of the chest. An echocardiogram raised the suspicion of congenital absence of the pericardium, with a posteriorly displaced heart. In retrospect, motion artifact on the left lung, attributed to cardiac pulsations and the lack of pericardium, resulted in a CT chest appearance, mimicking findings of pulmonary embolism. The misdiagnosis of pulmonary embolism was attributed to the artifact caused by excessive cardiac motion artifact on the chest CT scan. In non-gated CT angiograms, excessive motion causes an artifact that blurs the pulmonary vessels, reminiscent of a 'seagull' or a 'boomerang'. Physicians need to be aware of this phenomenon, as well as the characteristic radiological features of this congenital anomaly, to enable them to make a correct diagnosis.

Samuel Madeira

Papers

Congenital complete absence of pericardium masquerading as pulmonary embolism.