Congenital absence of pericardium revisited (original) (raw)

Partial Congenital Absence of The Pericardium: A Case Report

Brazilian Journal of Cardiovascular Surgery

The complete or the partial absence of pericardium is a rare congenital malformation for which the patients are commonly asymptomatic and the diagnosis is incidental. The absence of the left side of the pericardium is the most common anomaly that is reported in the literature while the complete absence of pericardium or the absence of the right side of the pericardium are uncommon and their criteria are still unrecognized given their rare occurrence in clinical practice. This paper aims to report a case of 19-year-old male with the congenital partial absence of both sides of the pericardium and to highlight the symptoms and the different cardiac imaging modalities used to confirm the diagnosis of this defect.

Congenital Absence of pericardium

Nepalese Heart Journal, 2013

A 30-year-old physically active male presented to us for evaluation of left sided chest pain which was non-exertional in nature. He described it as being of pricking type and non-radiating .His past history was not significant. Physical examination revealed a non-palpable apical impulse with no other abnormal findings.

Partial Congenital Absence of Pericardium

Revista Brasileira de Cardiologia Invasiva English Version, 2012

Agenesis of the pericardium is a rare congenital anomaly, which is difficult to diagnose. We report a case of a 42-yearold patient, with atypical chest pain, electrocardiogram with an incomplete right bundle branch block, chest X-ray with a counterclockwise heart axis deviation and echocardiogram suggesting right ventricle volume overload. Cardiac catheterization showed no intracardiac shunts, and rotation and displacement of the heart into the left hemithorax. Magnetic nuclear resonance imaging confirmed the diagnosis by revealing partial absence of the left pericardium, with interposition of lung parenchyma between the main pulmonary artery and the aorta.

A case report of congenital absence of the pericardium that was diagnosed by cardiac computed tomography angiogram (CCTA)

Radiology Case Reports

This case report describes a 40-year-old male who presented to the emergency department (ED) with chest pain. Initial diagnostic workup was concerning for a congenital cardiac anomaly, further imaging revealed complete congenital absence of the pericardium (CAP) which is a rare condition. Multimodality cardiac imaging including cardiac computed tomography angiogram (CCTA) was used to confirm the diagnosis of CAP. We briefly discuss various clinical presentations of CAP along with potential complications and other anomalies that could be associated with pericardial agenesis.

Does echocardiography play a role in the clinical diagnosis of congenital absence of pericardium? A case presentation and a systematic review

Journal of Cardiovascular Medicine

Congenital absence of pericardium is an uncommon cardiac defect with variable clinical presentations. The detection of this malformation is clinically relevant because of potential complications such as fatal myocardial strangulation, myocardial ischemia and sudden death. Physical examination, chest radiograph and ECG are not helpful for the diagnosis. Echocardiography may accurately identify abnormalities in myocardial wall motion and in cardiac silhouette that may strongly suggest the diagnosis that is confirmed by magnetic resonance imaging (MRI) or computed tomography scan. A case presentation and a review of the literature with emphasis on the role of echocardiography are presented.

Congenital complete absence of pericardium presenting with chest pain: MRI characteristics

International Archives of Medicine, 2016

Congenital absence of the pericardium is a very rare anomaly which has an incidence of 1 in 14000 cases in autopsy studies. Defects in the pericardium are either partial or complete with complete defects having a much benign course than partial defects. Partial defects are associated with complications like strangulation of a herniated cardiac chamber or coronary artery stenosis from external compression. Absence of the left pericardium is the most frequently encountered type of defect which accounts for 2/3 of the cases. We herein report a case of complete absence of the entire pericardium which is an extremely rare subtype with associated Cardiac magnetic resonance images.

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

Revista Portuguesa de Cardiologia, 2014

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 and partial absence of the left pericardium

Wiener Medizinische Wochenschrift, 2013

Kongenitale komplete und partielle Absenz des Perikardiums Zusammenfassung Das kongenitale Fehlen des Perikards ist eine seltene Fehlbildung. Wir zeigen diese Diagnose anhand der Kasuistiken zweier junger Männer. Bei der posteroanterioren Röntgendarstellung der Brust wurde eine Verschiebung der linken Herzgrenze in den linken Hemitorax festgestellt. Ungewöhnliche akustische Fenster und abnormale Herz-und septale Bewegungen während der Echokardiographie wiesen auf die Diagnose der kongenitalen Absenz des Perikardiums hin. Die Magnetresonanzuntersuchungen bestätigten endgültig die Diagnose.

Complete Congenital Absence of the Left Pericardium in Elderly Patient: A Case Report

International medical case reports journal, 2024

Congenital pericardial absence is an uncommon heart condition that is usually undiagnosed due to a lack of symptoms and awareness. In this case study, we present a 72-year-old patient who initially presented for medical evaluation due to unexplained weight loss, a displaced cardiac apex, and poor echocardiographic windows. An extracardiac tumor was suspected at first, but it was later determined that the patient had a congenital complete absence of the left pericardium. This case illustrates the possibility for benign pathology to present abnormally and details the clinical features, imaging findings, and management of congenital absence of the pericardium.

Congenital defect of the pericardium

Journal of Clinical Pathology, 1962

The morbid anatomy and histopathology is described as found at necropsy on a 7-year- old coloured girl who suffered from partial defect of the pericardium with prolapse and partial incarceration of the cardiac apex. Because of the absence of any history of illness during life the defect is considered to have been congenital, and because of the lack of any other significant necropsy findings, it is considered to have been the cause of death.

Congenital absence of the pericardium: A rare cause of right ventricular dilatation and levoposition of the heart

Cardiology Journal, 2012

Congenital absence of the pericardium is a rare cardiac defect with variable clinical presentations and is usually discovered incidentally. The pathology may lead to serious complications such as incarceration of cardiac tissue, myocardial ischemia, aortic dissection or valvular insufficiency. Diagnosis is not difficult so long as some tips are remembered. We present the cases of two patients with congenital absence of left pericardium.

Congenital partial absence of the pericardium in a young man with atypical chest pain

World journal of cardiology, 2013

Pericardial defects are infrequent congenital anomalies due to agenesis caused by premature atrophy of the common cardinal vein or Cuvier duct during the 5(th) or 6(th) week of embryonic life. These congenital defects are rare, typically observed as an incidental finding and usually remain asymptomatic. Nevertheless, the more widespread use of modern imaging techniques has contributed to an increase of its incidence in recent years. There is currently no consensus regarding therapeutic options, all of which are based on small retrospective studies that evaluate the risk of developing a life-threatening complication such as herniation and incarceration of the myocardium. We report on a 22-year-old male who presented with sudden onset of sharp chest pain and dyspnea. Computed tomography and cardiac magnetic resonance scan revealed a pericardial defect adjacent to the lateral free wall of the left atrium with associated herniation of the left atrial appendage. The patient was managed c...

Congenital partial absence of the pericardium presenting with a rare concurrent abnormality of vascular ring diagnosed by cardiac magnetic resonance imaging

Congenital absence of the pericardium is a rare abnormality that can be diagnosed by cardiac imaging procedures. A 49-year-old male needed medical attention due to the appearance of palpitation with a systolic murmur, and a notable aortic arch deviation was seen in the chest X-ray. In the echocardiogram, a poor echo window was detected. A cardiac magnetic resonance imaging (MRI) showed a rare concomitant anomaly of partial absence of the pericardium including a rare defect of the right-sided aortic arch. Using cardiac MRI, the pericardium can be easily visualized, and thus, its absence more easily detected, aiding appropriate clinical decision-making.

Absence of the left pericardium diagnosed by computed tomography

International Journal of Cardiology, 1995

A case report of left pericardial absence in a 12-year-old boy is described. The suspected diagnosis was not shown either by cross-sectional echocardiography or angiography, but was correctly established by computed tomography (CT), showing the effectiveness of this diagnostic test for diagnosis of pericardial defects.