Presentation
The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. [books.google.com]
Finally, a review of literature focusing on congenital absence of the pericardium, as well as a discussion of clinical presentation, imaging techniques and therapeutic options, is also presented. [ncbi.nlm.nih.gov]
Entire Body System
- Asymptomatic
The patient is asymptomatic at the present time. [revportcardiol.org]
In most cases they are asymptomatic and are usually diagnosed incidentally by chest radiograph. Some cases present with symptoms of dyspnea and chest pain. [scielo.br]
The patient is currently asymptomatic, we scheduled a clinical follow-up to evaluate potential complications. [scmr.org]
May be detected at any age but most commonly in low 20’s Location: Foraminal defect on left side 35% Complete absence of left side 35% —levoposition of heart Diaphragmatic surface 17% Total bilateral absence 9% Right sided 4% Clinical findings Mostly asymptomatic [learningradiology.com]
- Pain
[…] that appears suddenly in a previously asymptomatic adult and that varies from mild and occasional to frequent, prolonged, and debilitating. 4 The pain is stabbing; left-sided; brief, if not fleeting; unrelated to exertion but aggravated by position, [clinicalgate.com]
Congenital partial absence of the pericardium in a young man with atypical chest pain. [wjgnet.com]
However, some patients are symptomatic, reporting symptoms that include chest pain, palpitations, dyspnea, and syncope. [heartviews.org]
Case Presentation A 16-year-old girl with no previous cardiac problems came to the outpatient clinic with ill-defined chest pain. She declared that she had this pain for many months. She complained of dyspnoea but not easy fatigability. [ispub.com]
A constrained heart: a case of sudden onset unrelenting chest pain. Circulation 2014;130:1626. [academic.oup.com]
- Inflammation
Pericardial Inflammation Inflammation of the pericardium ( inflammatory pericarditis ) presents in many clinical settings and has a wide range of causes [ 2 ]. [jcmr-online.biomedcentral.com]
Characterization of Pericardial Masses Differentiation of tumor from thrombus in the Atrium, Ventricle or Pericardium Pericardial Diseases Detection of pericardial thickening Diagnosis of constrictive pericarditis Identification of active pericardial inflammation [upstate.edu]
In this disease, there are overlapping features of cardiac tamponade and constriction, the latter chiefly due to inflammation and fibrosis of the visceral pericardium. [appliedradiology.com]
Calcification and inflammation seemed related to use in an area of high pressure and flexion (ventricular septum and left ventricle) and possibly duration in situ. [icvts.oxfordjournals.org]
Other reported complications include right ventricular outflow obstruction, inflammation and infection, pulmonary stenosis, partial erosion into adjacent structures, atrial fibrillation, and congestive heart failure[2,5,6,7]. [ctsnet.org]
- Surgical Procedure
Cardiac surgical procedures. INTRODUCTION The cysts of the heart and pericardium are rare [1]. [scielo.br]
Postpericardiotomy syndrome is a common reported complication following these surgical procedures[ 1 - 4 ]. In summary, we report a case of a left side partial pericardial defect, a rare cardiac anomaly. [wjgnet.com]
Discussion Congenital absence of the pericardium (CAP) is a rare entity that is difficult to diagnose and is usually identified incidentally in clinical autopsies or surgical procedures performed for other reasons. 1–5 Its pathogenesis is multifactorial [revportcardiol.org]
2013/14/1/29/107118 Congenital absence of the pericardium (CAP) is a rare cardiac condition usually identified incidentally on autopsies and surgical procedures. [1] The prevalence, including cases with other cardiopulmonary anomalies, is approximately [heartviews.org]
- Congestive Heart Failure
Most patients in whom the origin of the left coronary artery (LCA) is the main pulmonary artery manifest congestive heart failure and myocardial ischaemia in the first four months of life. 2 This coronary anomaly is rarely seen in adults. [ecrjournal.com]
Other reported complications include right ventricular outflow obstruction, inflammation and infection, pulmonary stenosis, partial erosion into adjacent structures, atrial fibrillation, and congestive heart failure[2,5,6,7]. [ctsnet.org]
[…] valve prolapsed •Congestive heart failure •Compression of lung: Obstruction of right main stem bronchus, Compression of adjacent lobes 2. [interventional-cardiology.imedpub.com]
A variable degree of hypoplastic right ventricle with hypertrophic wall, dilatation of the right atrium, and tricuspid insufficiency may be present, while congestive heart failure and hydrops may occur in severe stenosis. [intechopen.com]
Respiratoric
- Dyspnea
However, some patients are symptomatic, reporting symptoms that include chest pain, palpitations, dyspnea, and syncope. [heartviews.org]
The most common symptoms are chest pain, dyspnea, cough, and palpitations. [scielo.br]
Occasionally, these defects may cause symptoms such as chest pain, dyspnea, arrhythmias, syncope, and even sudden death. Arrhythmias, including atrial fibrillation (AF), are uncommonly found in cases of CPD [ 1 ]. [e-sciencecentral.org]
Usually asymptomatic and discovered incidentally although occasionally may present with chest pain and dyspnea. They are thought to often result from aberrations in the formation of coelomic cavities. [radiopaedia.org]
We report on a 22-year-old male who presented with sudden onset of sharp chest pain and dyspnea. [wjgnet.com]
- Pleural Effusion
Pleural effusions may also occur. CCAM may occasionally remain undiagnosed until it is discovered later in life. [ 6 ] Recurrent chest infections may be a feature later in life. [patient.info]
The pleural effusion proved to be transudative. Following multiple recurrences, a permanent intrapleural catheter was placed to drain the effusions as needed. Analysis of the ascitic fluid indicated that portal hypertension had caused the ascites. [revespcardiol.org]
Pericardial effusion. Pericardial effusion. PA chest radiograph Pleural effusion. Pleural effusion. PA chest radiograph. Pneumothorax. Pneumothorax. PA chest film (Image and Description) Image Review: Pneumothorax. Pneumopericardium. [bcm.edu]
Distention of the inferior vena cava and hepatic veins is usually present and pleural effusions or ascites may be present. [appliedradiology.com]
Presence of a pericardial effusion (*) with several nodular masses (arrowhead). Note the presence of multiple pulmonary metastases. Presence of pectus excavatum and right-sided pleural effusion. [jcmr-online.biomedcentral.com]
Gastrointestinal
- Vomiting
The disorder is characterized by profoundly low levels of blood sugar (hypoglycemia), scarring (fibrosis) of the liver, failure to thrive, and cyclic vomiting. [rarediseases.org]
Liver, Gall & Pancreas
- Hepatomegaly
One individual had milder symptoms including hepatomegaly, protein-losing enteropathy and kidney disease. This disorder was formerly known as CDG-Ih. [rarediseases.org]
[…] disease of liver Congenital polycystic disease of liver Fibrocystic disease of liver 751.69 Other anomalies of gallbladder, bile ducts, and liver Absence of: gallbladder, congenital liver (lobe) Accessory: hepatic ducts liver Congenital: choledochal cyst hepatomegaly [theodora.com]
Cardiovascular
- Chest Pain
Congenital partial absence of the pericardium in a young man with atypical chest pain. [wjgnet.com]
However, some patients are symptomatic, reporting symptoms that include chest pain, palpitations, dyspnea, and syncope. [heartviews.org]
In one study half the patients were asymptomatic; others had dyspnea after brisk effort, palpitation and atypical chest pain 11. [ispub.com]
We report a case of a 42-year-old 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 [elsevier.es]
A constrained heart: a case of sudden onset unrelenting chest pain. Circulation 2014;130:1626. [academic.oup.com]
Neurologic
- Stroke
Case of Ferumoxytol (Feraheme®) Prompting Critical Modification to Our Patient Prebreast Magnetic Resonance Imaging Questionnaire Naziya Samreen, Asha A Bhatt, James Glockner, Christine U Lee J Clin Imaging Sci 9 (6) 1-3 (28 March 2019) Case report: Stroke [clinicalimagingscience.org]
(1)拍動 (2)卒中、発作〈脳の〉 stroke index (SI)一回拍出係数 stroke volume (SV)一回拍出量 stunned myocardium気絶心筋 subacute bacterial endocarditis (SBE)亜急性細菌性心内膜炎 subclavian artery鎖骨下動脈 subendocardial心内膜下 【形】 sudden death急死〈頓死〉、突然死 superior vena cava (SVC)上大静脈 supraventricular [tokyo-med.ac.jp]
Additional symptoms include liver abnormalities, heart abnormalities such as disease of the heart muscle (cardiomyopathy), stroke-like episodes, and excessive loss of proteins from the gastrointestinal tract (protein-losing enteropathy), which can cause [rarediseases.org]
Because the stretching capacity of the pericardial layers is limited, acute accumulation of pericardial contents (e.g., fluid, blood, air) can lead to cardiac chamber compression with decreased cardiac filling and subsequently impaired stroke volume, [jcmr-online.biomedcentral.com]
Heart disease and stroke statistics—2015 update: a report from the American Heart Association. Circulation. 2015;131(4):e29–322. pmid:25520374. View Article PubMed/NCBI Google Scholar 5. Peterson C, Grosse SD, Oster ME, Olney RS, Cassell CH. [journals.plos.org]
- Dizziness
[…] see Figure 5-2 ) is believed to originate from torsion of the thoracic inlet. 20 In light of evidence of transient compression of coronary arteries, myocardial ischemia is also a consideration. 21, 22 Additional symptoms include dyspnea, palpitations, dizziness [clinicalgate.com]
The patient was referred to the cardiology department of our institution for episodes of dizziness lasting for seconds without spinning sensation, accompanied by cold sweats. [revportcardiol.org]
- Seizure
ST3GAL5-CDG – Affected individuals (some 46 reported) have developed infantile-onset seizures (epilepsy). [rarediseases.org]
Workup
A brief discussion on this rare congenital defect with its clinical presentation, diagnostic workup and management is provided at the end of the case report. [wjgnet.com]
FIGURE 2 Proposed diagnostic workup for a child presenting with symptoms suggestive of CTS. LPA, left pulmonary artery. [pediatrics.aappublications.org]
Further history and workup for deep vein thrombosis (DVT) and a hereditary hypercoagulable state failed to identify a cause for PE. The patient was discharged, but later on readmitted to the hospital with chest pain and palpitations. [heartviews.org]
X-Ray
- Pericardial Effusion
A cardiac ultrasound done at birth showed the same hyper echoic structure, surrounded by a new pericardial effusion. [criticalcare.imedpub.com]
There has been a progressive reduction of effusion (3 mm 6 months after surgical intervention). Figure 1. Echocardiography (subcostal view). Moderate anterior basal pericardial effusion. Figure 2. Chest x-ray. [revespcardiol.org]
Whenever pericardial masses are encountered, the amount of pericardial effusion and any evidence of tamponade effect should be reported. [appliedradiology.com]
Pericardial effusion. MayoClinic.com [On-line information]. Available online at http://www.mayoclinic.com/health/pericardial-effusion/DS01124/METHOD=print. Accessed June 2012. Hoit, B. (Modified 2011 June). Pericarditis. [labtestsonline.it]
It is believed that effusive-constrictive pericarditis most likely represents an intermediate transition from acute pericarditis with pericardial effusion to pericardial constriction [ 57 ]. [jcmr-online.biomedcentral.com]
Pleura
- Pleural Effusion
Pleural effusions may also occur. CCAM may occasionally remain undiagnosed until it is discovered later in life. [ 6 ] Recurrent chest infections may be a feature later in life. [patient.info]
The pleural effusion proved to be transudative. Following multiple recurrences, a permanent intrapleural catheter was placed to drain the effusions as needed. Analysis of the ascitic fluid indicated that portal hypertension had caused the ascites. [revespcardiol.org]
Pericardial effusion. Pericardial effusion. PA chest radiograph Pleural effusion. Pleural effusion. PA chest radiograph. Pneumothorax. Pneumothorax. PA chest film (Image and Description) Image Review: Pneumothorax. Pneumopericardium. [bcm.edu]
Distention of the inferior vena cava and hepatic veins is usually present and pleural effusions or ascites may be present. [appliedradiology.com]
Presence of a pericardial effusion (*) with several nodular masses (arrowhead). Note the presence of multiple pulmonary metastases. Presence of pectus excavatum and right-sided pleural effusion. [jcmr-online.biomedcentral.com]
Treatment
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
Initial treatment shortly after birth may be necessary to treat a baby's cyanosis because of inadequate pulmonary artery blood flow. [pted.org]
Treatment Continuous suction of the upper pouch and a feeding gastrostomy constitute initial treatment. The patient then is followed closely with contrast radiographs until primary esophagus repair can be performed, usually at an age of 2-3 months. [emedicine.medscape.com]
Prognosis
Prognosis [ edit ] The prognosis of ectopia cordis depends on classification according to three factors: [1] Location of the defect Cervical Thoracic Thoracoabdominal Abdominal Extent of the cardiac displacement Presence or absence of intracardiac defects [en.wikipedia.org]
Prognosis CCAM is a rare fetal lung disease with an excellent prognosis in the absence of fetal hydrops. Resection of the CCAM generally leads to full recovery. [patient.info]
Morbidity after cardiovascular surgery is much higher if the patient has cirrhosis, and the prognosis is influenced by the functional state of the liver disease. [revespcardiol.org]
Signal characteristics are those of fluid and include 4 : T1: typically low signal (occasionally can be high signal if contains proteinaceous material) T2: high signal T1 C+ (Gd): no enhancement Treatment and prognosis They are benign lesions. [radiopaedia.org]
McElhinney DB, Silverman N (1999) Left ventricular aneurysm inthe fetus: a diagnosis with a mixed prognosis. Cardiol Young9: 123-126. [criticalcare.imedpub.com]
Etiology
[…] most cases, the heart protrudes outside the chest through a split sternum. [1] Pathology [ edit ] Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall formation during embryonic development. [2] The exact etiology [en.wikipedia.org]
Etiology of pericardial cyst6 1.Congenital 2.Inflammatory: Rheumatic pericarditis, Bacterial infection particularly tuberculosis, Echinococcosis 3.Traumatic 4.Post cardiac surgery Table 2 : Etiology of pericardial cyst6. [interventional-cardiology.imedpub.com]
Congestive cardiac failure (non-cardiac etiology). Congestive cardiac failure secondary to glomerulonephritis. PA chest film Lateral chest film Congestive cardiac failure secondary to sickle cell anemia. [bcm.edu]
Despite an extensive work-up, the etiology remained unclear. [ncbi.nlm.nih.gov]
Epidemiology
The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. [books.google.com]
In general, the prognosis for ectopia cordis is poor—most cases result in death shortly after birth due to infection, hypoxemia, or cardiac failure. [4] Epidemiology [ edit ] The occurrence of ectopia cordis is 8 per million births. [2] It is typically [en.wikipedia.org]
Congenital cytomegalovirus infection: review of the epidemiology and outcome. Obstet Gynecol Surv. 2002; 57 :245–256. [ PubMed ] [ Google Scholar ] 8. Fowler KB, Stagno S, Pass RF. [ncbi.nlm.nih.gov]
Epidemiology No data are available regarding the frequency of this lesion but it is a rare condition. [patient.info]
Pathophysiology
Crossref | PubMed Angelini P, Velasco JA, Flamm S, Coronary anomalies: incidence, pathophysiology, and clinical relevance, Circulation, 2002;105:2449–54. Crossref | PubMed [ecrjournal.com]
Today, despite improved knowledge of pathophysiology of pericardial diseases and the availability of a wide spectrum of diagnostic tools, the diagnostic challenge remains. [jcmr-online.biomedcentral.com]
This review will firstly discuss the anatomy and pathophysiology of CTS and outline the various clinical presentations associated with the disorder. [pediatrics.aappublications.org]
Based on this case report the clinical, pathophysiological and diagnostic features of this condition are described Pulmonary and systemic venous flow patterns assessed by transesophageal Doppler echocardiography in congenital absence of the pericardium [rwjms1.umdnj.edu]
Prevention
The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. [books.google.com]
Glycoprotein-D-adjuvant vaccine to prevent genital herpes. N Engl J Med. 2002; 347 :1652–1661. [ PubMed ] [ Google Scholar ] 36. [ncbi.nlm.nih.gov]
[…] intervention Most authors advise leaving incidental defects untreated Symptomatic foraminal defect may require surgery because of herniation and strangulation of left atrial appendage or herniation of LA/LV Surgery can be enlargement of the defect to prevent [learningradiology.com]
Techniques to prevent arrhythmias and sudden death should be considered in some cases when large amounts of adjacent structures are removed during surgery. Acknowledgements None. [jtd.amegroups.com]
Patients with large pericardial effusions or cardiac tamponade undergo pericardiocentesis and may require drain placement or pericardial window to prevent recurrence. [appliedradiology.com]