Congenital complete absence of pericardium masquerading as pulmonary embolism.
TL;DR: A 23 year-old-male, who presented to the hospital with complaints of pleuritic chest pain and exertional dyspnea, with suspicion of congenital absence of the pericardium is presented, who was misdiagnosed as having pulmonary embolism.
Abstract: 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.
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01 Jan 2020
TL;DR: CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR and most patients remain stable and do not require surgical intervention.
Abstract: 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.
13 citations
TL;DR: The case of a 17-year-old patient with complete pericardial agenesis (diagnosed by chance during a cardiological consultation) is reported and the radiological findings of this entity are reviewed.
Abstract: 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.
4 citations
Cites background from "Congenital complete absence of peri..."
...CAP is usually asymptomatic, especially in complete absence of the pericardium.(2,9) However, non-specific chest pain, dyspnea, recurrent pulmonary infections, fatigue, angina, heart failure, pericarditis, arrhythmias, peripheral embolism, syncope and even sudden death have been reported....
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...Six types of CAP have been described, including total absence, right-sided defects (complete or partial), leftsided defects (complete or partial), and diaphragmatic defects.(2,8) The most common is complete left pericardial defect (70%), while right-sided agenesis is found in 4%, diaphragmatic defects in 17% and total absence in 9%....
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...004% in surgical and pathological series.(2,7) It is three times more common in males and other congenital anomalies may be associated in 30--50% of cases, the most common being patent ductus arteriosus, atrial septal defect, mitral valve stenosis, bronchogenic cyst, tetralogy of Fallot, pulmonary sequestration, diaphragmatic hernia and pectus excavatum....
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...Its pathogenesis is multifactorial, but premature atrophy of the left common cardinal vein (left duct of Cuvier) during the 5th and 6th week of embryonic life compromises the blood supply to the left pleuropericardial fold, preventing it from closing normally.(2,4,6) Its prevalence is approximately 0....
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...also be found as a part of other disorders such as VATER association or Marfan or Pallister-Killian syndromes.(2,7) Six types of CAP have been described, including total absence, right-sided defects (complete or partial), leftsided defects (complete or partial), and diaphragmatic defects....
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TL;DR: In this paper , a 9-year-old boy was diagnosed with Congenital Complete absence of the pericardium (COPD), which is an exceedingly rare condition with a prevalence of 0.002-0.004%.
TL;DR: A case with complete absence of the left pericardium in an asymptomatic young male patient with suspected heart and lung herniation and ventricular entrapment is presented.
Abstract: 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.
Cites background from "Congenital complete absence of peri..."
...304 Journal of Translational Medicine and Research, 21 (4), 2016...
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...306 Journal of Translational Medicine and Research, 21 (4), 2016 consecutive infarction (8), coronal artery compression with myocardial necrosis (2,8), traumatic aortic dissection secondary to cardiac hypermobility (17)....
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...Congenital pericardial absence is a rare condition and usually there is an association with other congenital heart diseases (1-6) or thoracic anomalies (1-3)....
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...Chest X ray shows rotation of the heart to the left side (3,12), without tracheal deviation (4,12), an illdefined right cardiac border (13), prominent left cardiac border (3) and interposition of the lung between the left diaphragm and the inferior cardiac border (12)....
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...Journal of Translational Medicine and Research, 21 (4), 2016 305 role for the heart (3), limits the displacement of the heart in the mediastinum (8)....
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TL;DR: A rare bilateral asymmetric variation in the roots of origin of the phrenic nerve and absence of fibrous pericardium in an old male cadaver is reported and is of practical importance during supraclavicular block during anesthesia.
Abstract: 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. © 2016, Brazilian Society of Anatomy. All rights reserved.
References
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TL;DR: Familiarity with the CT and MR imaging features of these diseases is important, as both modalities provide a larger field of view than does echocardiography, allowing the examination of the entire chest and detection of associated abnormalities in the mediastinum and lungs.
Abstract: In the evaluation of pericardial disease, computed tomography (CT) and magnetic resonance (MR) imaging traditionally have been used as adjuncts to echocardiography. However, CT and MR imaging are particularly useful as sensitive and noninvasive methods for evaluating loculated or hemorrhagic pericardial effusion, constrictive pericarditis, and pericardial masses. Both CT and MR imaging provide excellent delineation of the pericardial anatomy and can aid in the precise localization and characterization of various pericardial lesions, including effusion, constrictive pericarditis and pericardial thickening, pericardial masses, and congenital anomalies such as partial or complete absence of the pericardium. Both modalities provide a larger field of view than does echocardiography, allowing the examination of the entire chest and detection of associated abnormalities in the mediastinum and lungs. Soft-tissue contrast on CT scans and MR images also is superior to that on echocardiograms. Given the many potential applications of these modalities in the evaluation of pericardial diseases, familiarity with the CT and MR imaging features of these diseases is important.
398 citations
TL;DR: It is concluded that the shortest scan time on third-generation units (0.6 second) cannot prevent all artifacts arising from motion in the chest, and even ultrafast scan times are not short enough to eliminate artifacts on these units.
Abstract: Cardiac and ventilatory motions cause artifacts at chest computed tomography (CT). To determine how short the scan times on third-generation units must be to avoid such artifacts, motion was measured with fast and ultrafast CT scans. Minimum detectable motion was then determined. The longest scan time that avoided a barely perceptible artifact was calculated by dividing the minimum detectable motion by the peak physiologic velocity. The posterior left ventricular wall moved at a maximum velocity of 52.5 mm/sec, necessitating a scan time of 19.1 msec or less to avoid artifact. Lung vessels near the heart moved at 40.5 mm/sec for a scan time of 24.7 msec or less. During quiet breathing, pulmonary vessels moved at 10.7 mm/sec for a scan time of 93.5 msec or less. The authors conclude that the shortest scan time on third-generation units (0.6 second) cannot prevent all artifacts arising from motion in the chest. Even ultrafast scan times (50 msec) are not short enough to eliminate artifacts on these units. Th...
208 citations
TL;DR: Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease, and Symptomatic patients with the complete form may benefit from pericardioplasty.
153 citations
TL;DR: Congenital absence of the pericardium is an uncommon finding that may or may not be symptomatic, and may occur with other cardiac or extracardiac abnormalities and a variety of imaging modalities may identify the condition.
96 citations