Pericardial Agenesis Research Articles

The Heart Has its Reasons Which Reason Knows Not: A Curious Case of Chest Pain

BackgroundElectrocardiographic (ECG) findings of T-wave inversions in V1–V3, with or without accompanying epsilon waves, often raise concerns for the rare, but potentially lethal, arrhythmogenic right ventricular cardiomyopathy (ARVC). However, this pattern may be found in pericardial agenesis, an even rarer pathology. Concomitant myocarditis can confuse this presentation further. Case ReportWe report a case of a previously healthy man who presented with left-sided chest pain, ECG findings suggestive of ARVC, and a final diagnosis of myocarditis with underlying partial pericardial agenesis. Why Should an Emergency Physician Be Aware of This?A growing number of cases have reported pericardial agenesis demonstrating ECG changes similar to ARVC. We discuss an approach to a diagnostically challenging patient. This case emphasizes the importance of a broad differential and the danger of premature closure.

Pericardial agenesis - the wandering heart

BackgroundCongenital pericardial absence is an uncommon cardiac anomaly that is typically asymptomatic and commonly misdiagnosed due to a lack of symptoms or atypical symptoms. Pericardial agenesis (PA) should be considered one of the differential diagnoses when the patient presents with chest pain. This case shows how the diagnosis of pericardial agenesis is made exclusively using multi-modality imaging, starting from findings in a basic chest radiograph to cardiac MRI, while also demonstrating the classic signs seen in this condition. Magnetic resonance imaging of the heart is the gold standard for determining the absence of pericardium in the prognosis.Case presentationA 32-year-old male who presented with chest discomfort and radiating pain to his back and left shoulder mimicking myocardial infarction with normal ECG and enzyme markers. A chest radiograph (taken 24 h apart) demonstrates the left lateral position of the heart and the bulging contour of the left heart border, a lucent area between the aorta and pulmonary artery. Subsequently, cardiac MRI reveals left pericardial agenesis.ConclusionsThis article provides insight into a rare differential to consider in a young patient presenting with chest discomfort. This case shows how the diagnosis of pericardial agenesis is made exclusively using multi-modality imaging, starting from findings in a basic chest radiograph to cardiac MRI, while also demonstrating the classic signs seen in this condition.

Left mediastinal pleural and left pericardial agenesis, diagnosis, complications and management

Left mediastinal pleural and left pericardial agenesis, diagnosis, complications and management

Congenital Complete Left-Sided Absence of the Pericardium Incidentally Identified in the Autopsy: A Case Report

Congenital absence of the pericardium, also known as pericardial agenesis, is an extremely rare anomaly. Although most cases are asymptomatic, some experience chest pain, dyspnea, dizziness, and syncope. A few sudden death cases have been reported. We report a case of congenital complete left-sided absence of the pericardium that is not related to the cause of death. In the autopsy of a skinny 45-year-old deceased man, there was no left pericardium, and the heart had direct contact with the left lung. Inflammation and adhesion around the heart, torsion of great vessels, structure abnormality of the heart, and histologic lesion of the myocardium were absent. Due to the chemical analysis result and morphology, we suspected that the cause of death was related to starvation. The congenital absence of the pericardium is usually accompanied by myocardial infarction, aortic dissection, and variable congenital anomalies in the heart and other organs. Additionally, both complete and partial defects can cause myocardial infarction. Therefore, we suggest that precise gross examination should be performed to determine the ischemic lesions in the heart and other anomalies if congenital absence of the pericardium is noted in the autopsy.

C37 CONCOMITANT PARTIAL PERICARDIAL AGENESIS, PERICARDIAL CYST AND POSTERIOR MITRAL VALVE LEAFLET HYPOPLASIA: A CASE REPORT

Abstract Background Pericardial agenesis (1) is a congenital developmental disorder of the pleuro–pericardial membranes that is usually asymptomatic. A pericardial cyst (2) is a congenital cyst composed of mesothelial cells originating most often from the right pericardium. Hypoplasia of the posterior mitral leaflet (3) is congenital and it is very rarely diagnosed in adulthood. These alterations are usually sporadic and not associated. Case presentation A 53–year–old woman with obesity and bronchial asthma was referred to the emergency department for left hemiparesis and concomitant dyspnoea in new–onset atrial fibrillation. A right frontal ischemic stroke was diagnosed. During the hospitalization transthoracic echocardiography was performed showing a large prolapsed anterior mitral leaflet (AML) with an eccentric and significant regurgitation. Transesophageal echocardiography was performed. It confirmed the severity of mitral regurgitation, due to a large AML prolapse, but also showed a severely hypoplastic posterior leaflet (PML). Swinging motion was also reported in the absence of pericardial effusion. In the past the patient was surgically treated for an anterior pericardial cyst that was excised. Old CT images were reviewed and showed the absence of pericardial sheets along posterior and lateral left ventricle walls, which were not contiguous to the cyst (that was anterior). The patient was finally candidated to surgery for mitral valve replacement. Discussion The case report shows a unique case of association between partial pericardial agenesis, pericardial cyst, severe PML hypoplasia with AML prolapse and severe mitral regurgitation. The association of these three anomalies has never been described before. This could indicate a common pathogenetic denominator and therefore the need to look for this combination of structural abnormalities in carriers of even just one of them. Bibliography 1. Lopez D. et al, doi: 10.1016/j.pcad.2016.12.002 2. Khayata M. et al, doi: 10.1007/s11886–019–1153–5 3. Parato VM. et al, doi: 10.4103/jcecho.jcecho_73_17

Partial pericardial agenesis in a dog

Pericardial defects are considered rare in animals and humans. Pericardial agenesis is characterized by partial or total pericardial sac malformation. Here is reported a case of apical partial pericardial agenesis in an adult mixed-breed male dog referred for necropsy. The pericardial sac was absent from the apical region up to the middle area of the ventricles resulting in a bilateral ventricular compression by the remaining pericardium changed the organ conformation. Microscopically, there was evidence of neutrophilic and hemorrhagic myocarditis with cardiomyocyte atrophy and interstitial fibrosis. Agenesis is often a subclinical disorder, and it is an incidental postmortem finding in most cases; however, it can cause complications in cases of cardiac structure herniation.

Freedom in the chest

Pericardial agenesis is a rarely seen congenital defect characterised by the partial or, more rarely complete, absence of the pericardium. Most often asymptomatic, it is usually incidentally discovered following the demonstration of heart’s laevorotation on imaging, in the operating room or at autopsy. In this article, we report the case of an 80-year-old patient with asymptomatic complete pericardial agenesis fortuitous discovered. Pericardial agenesis observations are extremely uncommon reported in the literature, which substantiate its original epidemiological character. In addition, this observation brings some clinical, electrical as well as iconographic elements to better understand the pathology and raises clinical suspicions. Finally, this case report confirms the exceptionally symptomatic nature of the pathology, illustrating the irrelevance of treatment or specific follow-up.

Multimodality imaging and clinical significance of isolated congenital absence of the pericardium

Background. Congenital absence of the pericardium is a rare anomaly characterized by variable and non-specific clinical manifestations. The prevalence of this pathology in the population is approximately 0.0001 – 0.044% and it is almost three times more common in men. However, the real prevalence, including asymptomatic and undiagnosed cases, may be higher. Purpose – highlighting the echocardiographic and tomographic signs of isolated congenital absence of the pericardium. Materials and methods. A 42-year-old patient M. Echocardiography did not permit to obtain standard echo images via typical echocardiographic approaches due to poor acoustic window. Magnetic resonance imaging and computed tomography of the heart revealed marked left heart rotation and confirmed the most common variant of congenital absence of the pericardium, that is complete left-sided absence pericardial agenesis. Results. The presented case illustrates the non-specificity of clinical symptoms in patients with congenital absence of the pericardium. The electrocardiogram of the patient showed right axis deviation, right bundle branch block, poor R wave progression in the precordial leads. Chest X-ray in the anterior-posterior projection typically shows a pronounced displacement of cardiac silhouette to the left without deviation of the trachea, elongated left border of the heart and inability to delineate the right border, indicating left heart rotation. Echocardiography revealed an unusual orientation of the heart in the chest, i.e. the displacement of the heart (particularly, of the apical segments) to the left and backwards. In cases when the pericardium is absent on the right and on both sides, the right ventricle can indeed be enlarged, sometimes there is a paradoxical motion of the interventricular septum, severe tricuspid regurgitation as well as the swinging motion of the cardiac apex. Magnetic resonance imaging and computed tomography of the heart enable detection of the displacement of the heart to the left half of the chest with cardiac apex directed backwards, the interposition of lung tissue between the ascending aorta and the pulmonary artery trunk. Conclusions. Congenital absence of the pericardium is a rare pathology and thus it can be challenging in terms of diagnosis, while sometimes it remains undiagnosed. The combination of echocardiography with magnetic resonance imaging and/or computed tomography allows to accurately diagnose congenital absence of the pericardium and assess the risks associated with it. Most patients with complete unilateral or bilateral congenital absence of the pericardium have a favorable prognosis and do not require treatment.

Complete pericardial agenesis

A case report of pericardial agenesis identified in the course of a thoracoscopic resection of pulmonary sequestration in a 9-month-old boy.

Complete Pericardial Agenesis in a Pregnant Patient: A Clinical Dilemma

Pericardial agenesis is a rare congenital anomaly found predominantly in men, and its complete form is extremely rare and difficult to diagnose. This report describes the case of a pregnant patient with complete pericardial agenesis in which mode of delivery and sterilization raised debate among specialists. Complete Pericardial Agenesis in a Pregnant Patient: A Clinical Dilemma

Marked levoposition of the heart – a clue to diagnosis of complete pericardial agenesis

Marked levoposition of the heart – a clue to diagnosis of complete pericardial agenesis

498 A missed and misdiagnosed case of pericardial agenesis

Abstract Introduction Pericardium is a fibroserous sac surrounding the heart and the roots of the great vessels. Pericardial agenesis which is a rare condition can be complete or partial and the most common form is absence of left hemipericardium. Pericardial agenesis is often an incidental finding, as it is usually asymptomatic. Moreover patients presenting with symptoms can be missed diagnosed, because it can be difficult to visualise the pericardium clearly with imaging modalities. Herein we present a misdiagnosed pericardial agenesis case. Case Report A 49 years old male was admit to our cardiology clinic for confirmation, and follow-up of a previous diagnosis of right ventricular cardiomyopathy (CMP). He had been previously diagnosed based on transthoracic and transesophageal echocardiography findings 13 years ago. Since then he had been attending routine follow-ups, he had also underwent several imagining modalities even cardiac magnetic resonance (CMR) to confirm the diagnosis of right ventricular CMP. No arrhythmia had been detected in Holter monitoring. He had no dyspnea, or other specific signs and symptoms. His medical and family histories were otherwise unremarkable. A 12-lead electrocardiogram (ECG) showed normal sinus rhythm without having specific abnormalities. The chest X-ray revealed laevorotation of the heart (Fig A). The transthoracic echocardiography showed a laterally displaced left ventricle (LV) apex, and an enlarged right ventricle (RV) with normal systolic function (Fig B). Coronary computed tomography angiography (CCTA) revealed normal coronary arteries, leftward displacement of the heart with mild RV dilatation. The pericardium was only visible around right atrium (Fig C). CMR demonstrated mild RV dilatation (end-diastolic volume 167ml) with reasonable systolic function (EF 50%), normal LV dimensions with normal systolic function (Fig D). The absence of pericardium at both left and right sides, except around right atrium, was confirmed, whereas there was no additional finding such as wall motion abnormality. Therefore the patient was diagnosed with partial pericardial agenesis and scheduled for follow-ups. Conclusion The pericardial agenesis which is a rare disorder is generally benign. Although MRI is believed to be the gold standard technique, it might not be able to delineate entire pericardium, because of various artefacts or paucity of surrounding fat. Therefore physicians should be aware of the indirect signs on various imaging modalities like excessive laevorotation of the heart, as direct recognition can be tricky, and the patient can be easily missed and/or misdiagnosed. Our patient was asymptomatic. His pericardial defect was almost total and this abnormality hadn’t been recognized in his previous CMR or echocardiograpy, leading to a misdiagnosis of CMP. Complete or unilateral absence of the pericardium is considered to be benign, surgical pericardioplasty may only be considered for highly symptomatic patients. Abstract 498 Figure.

A rare case about pericardium: Left deviated heart and pericardial agenesis

Congenital absence of the pericardium is not a common condition in daily practice. There are no obvious and clear symptoms. This condition, which is diagnosed incidentally, may cause some complications when not diagnosed. Therefore, imaging techniques, such as echocardiography, are essential. In this article, we present a rare case of pericardial agenesis.

Complete Left Pericardial Agenesis associated with Ventricular Septal Defect

9 years old male patient admitted to our department with complaints of recurrent respiratory tract infection since 2 years of age. No History of cyanosis His left eye is absent and it was the abnormality at birth.

A floating heart: A case of congenital pericardial agenesis associated with atrial septal defect

Congenital absence of the pericardium is a rare condition. Diagnosis and management of this condition remain a dilemma for clinicians. Here, we report a case of 15-year-old boy who had complaint of palpitation. Considering the echocardiographic features, pericardial agenesis associated with atrial septal defect (ASD) was suspected and multimodality imaging was performed for subsequent confirmation. Mal-aligned septum leads to failure in ASD device closure; therefore, the defect was repaired surgically by using a Dacron patch. Physicians need to be aware of this disorder and its associated congenital abnormalities, to enable them to make a correct diagnosis and treatment plan.

Right partial pericardial agenesis: An unusual case discovered during a heart surgery

Abstract The pericardial agenesis occurs from a premature atrophy of the left Cuvier vein during embryonic development (Larsen, 2003). It is a non-frequent disease, most of the time accidentally discovered and mainly found at the left side of the pericardium (Centola et al., 2009). We report a case of right partial pericardial agenesis discovered during a sternotomy.

Surgical Treatment of Partial Agenesis of the Left Pericardium

Introduction: The pericardial agenesis is a rare congenital anomaly. Classified as partial or total being asymptomatic in most cases, but some patients are at risk for serious complications and death, and these have indication for surgical treatment. Case Presentation: This paper describes the case of a 21 year old patient, with atypical precordial pain, diagnosed with agenesis of the left pericardium confirmed by cardiac magnetic resonance imaging (MRI), in which a surgical correction was performed, by means of a neopericardium with bovine pericardial patch. Discussion: Follow-up in immediate post-operative period showed that the patient had no complications and clinical improvement, and after 15 months, a new MRI showed a normal cardiac image.

Pericardial Agenesis

We report the case of a 37-year-old woman with completel pericardial agenesis. A young female patient presented with a history of atypical chest pain. She was otherwise fit and well and medication free. Transthoracic echocardiography revealed unusual echocardiographic window, abnormal ventricular septal motion and an abnormal anteroposterior cardiac movement within the thoracic cavity. Computed tomography examination showed prominence of the main pulmonary artery and the interposition of the lung tissue between the aorta and the main pulmonary artery thus confirming the diagnosis. Pericardial agenesis is a rare anomaly, difficult to diagnose. Its evolution is usually benign. However, on rare occasions, partial defects have been the cause of sudden death.

Extralobar Sequestration in Anterior Mediastinum With Pericardial Agenesis

We report a very rare case of extralobar sequestration and pericardial agenesis in a 22-year-old man. A computed tomographic (CT) scan demonstrated an anterior mediastinal mass. No aberrant artery was preoperatively identified. The patient underwent surgery with an impression of thymoma. An extralobar sequestration receiving its blood supply from the left pulmonary artery, accompanied with pericardial agenesis, was noted at the time of operation. The anterior mediastinum is an unusual site for extralobar sequestions. It is recommended to include extralobar sequestration in the differential diagnosis of anterior mediastinal masses, even if the aberrant artery is not recognized on the computed tomographic scan.

Diagnóstico de imagen de la agenesia de pericardio

Pericardial agenesis is a rare anomaly, difficult to diagnose. Its evolution is usually benign. However, on rare occasions, partial defects have been the cause of sudden death. Therefore, surgical treatment has sometimes been indicated, even though in the cases were asymptomatic. We report the case of a 50 year-old woman with partial pericardial agenesis and herniation of left atrial appendage trough. The defect was discovered by a routine chest x-ray and treated in a conservative way. Current diagnostic and therapeutic techniques are reviewed.