Presentation
Abstract Ebstein's anomaly is known for its varied presentations and exceptionally long survival in some cases. [ncbi.nlm.nih.gov]
[…] as pain or heaviness in chest, difficulty in breathing or cough. 4,5 We present a case in a middle-aged man who presented with feeling of heaviness in chest. [pjms.com.pk]
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.de]
Here the authors describe a case of previously undiagnosed Ebstein's anomaly in a patient who presented with massive pericardial effusion. Over the next 6 months, the patient was hospitalised twice for pericardiocentesis. [casereports.bmj.com]
Symptoms, if present, are usually because of compression by the cystic mass. [annalspc.com]
Entire Body System
- Asymptomatic
Patients can be asymptomatic, but they may experience chest pain, myocardial ischemia, emboli, arrhythmia, and sudden death. In this report, we present an 8-month-old boy with pericardial defect and right atrial appendage herniation. [ncbi.nlm.nih.gov]
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]
They are usually asymptomatic are often discovered incidentally on chest X-ray. [omicsonline.org]
These are quite rare in childhood and are mostly asymptomatic. Symptoms, if present, are usually because of compression by the cystic mass. [annalspc.com]
The exact incidence of these lesions, however, is not known because antenatal detection has been infrequent and many affected neonates are asymptomatic [3,6]. [medcraveonline.com]
- Atrial Septal Defect
The portion of the right ventricle above the displaced leaflets is composed of atrial tissue: referred to as the "atrialized" portion of the right ventricle Associated Anomalies Atrial septal defect (75% with a right-to-left shunt) Ventricular septal [echocardiology.org]
Defect Myocardial Infarction in Childhood Ostium Primum Atrial Septal Defects Ostium Secundum Atrial Septal Defects Partial Anomalous Pulmonary Venous Connection Patent Ductus Arteriosus (PDA) Pediatric Aortic Valve Insufficiency Pediatric Atrial Ectopic [klikdokterjantung.wordpress.com]
We describe the management of a patient with this condition, atrial Septal defect (ASD) and also with history of Protein S deficiency undergoing caesarean section (C-section). [ispub.com]
septal defect asymmetrical septal hypertrophy hypertrophic cardiomyopathy heart dissection myocardial Fabry disease basophilic degeneration of the myocardium cardiomyocytic disarray rheumatic heart disease cardiomyocytic lipofuscin deposits 1996 - 2019 [humpath.com]
Frequently, Ebstein's Anomaly is associated with an Atrial Septal Defect (ASD). The ASD may be closed with a patch made of pericardium (part of the membrane that covers the heart) or of a synthetic material. [pted.org]
- Congestive Heart Failure
heart failure Cardiac Auscultation Widely split S1 "Sail" sound Loud S2 Tricuspid regurgitation Electrocardiogram Wolff-Parkinson-White type B Atrial and or ventricular arrhythmias Chest X-ray Cardiomegaly Treatment Plication with closure of atrial septal [echocardiology.org]
heart failure congestive rheumatic heart failure cor biloculare cor triatriatum coronary artery anomaly coronary artery fistula cyanotic heart disease dextrocardia double aortic arch double outlet right ventricle ebstein anomaly eisenmenger syndrome [washington.providence.org]
Heart Failure Pediatric Cor Triatriatum Pediatric Dilated Cardiomyopathy Pediatric Ebstein Anomaly Pediatric Fungal Endocarditis Pediatric Holt-Oram Syndrome Pediatric Hypertension Pediatric Hypertrophic Cardiomyopathy Pediatric Hypoplastic Left Heart [klikdokterjantung.wordpress.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]
Other complications, such as congestive heart failure, right ventricular outflow tract obstruction, infection and pulmonary stenosis have been reported. [omicsonline.org]
- Coarctation of the Aorta
Pediatric Cardiology Acquired Mitral Stenosis Anomalous Left Coronary Artery From the Pulmonary Artery Aortopulmonary Septal Defect Atrioventricular Node Reentry Supraventricular Tachycardia Bicuspid Aortic Valve Coarctation of the Aorta Commotio Cordis [klikdokterjantung.wordpress.com]
Cohen M, Foster V, Steeler PM, et al., Coarctation of the aorta: long-term follow-up and prediction of outcome after surgical correction, Circulation, 1989;80:840–45. [radcliffecardiology.com]
Conditions Associated with NIH (This list is not exhaustive) Cardiac Cardiomyopathy, Ebstein's anomaly, pulmonary atresia, coarctation of the aorta, hypoplastic left heart, complete AV canal, left sided obstructive lesions, premature closure of the foramen [perinatology.com]
Respiratoric
- Tachypnea
Hemorrhage-Intraventricular Hemorrhage Polycythemia of the Newborn Polyhydramnios and Oligohydramnios Prematurity Pulmonary Interstitial Emphysema Respiratory Distress Syndrome Retinopathy of Prematurity Shock and Hypotension in the Newborn Transient Tachypnea [klikdokterjantung.wordpress.com]
VSD Rİ>20Ü/m² Qp/Qs 13 Patients with large VSD and increased Qp/Qs Weak peripheral pulses Symptoms and signs Patients with large VSD and increased Qp/Qs Weak peripheral pulses Tachypnea, subcostal drawings, profuse sweating Hepatomegaly, high jugular [slideplayer.com]
Cardiovascular
- Hypertension
Double Outlet Right Ventricle With Normally Related Great Arteries Double Outlet Right Ventricle With Transposition Double-Chambered Right Ventricle Endocardial Fibroelastosis Heterotaxy Syndrome and Primary Ciliary Dyskinesia High Altitude Pulmonary Hypertension [klikdokterjantung.wordpress.com]
This typically happen in constrictive pericardits and any isolated RV failure without pulmonary hypertension. [drsvenkatesan.com]
Increased red blood cell production by the spleen and liver leads to hepatic circulatory obstruction (portal hypertension) Anti-D, anti-E, and antibodies directed against other Rh antigens comprise the majority of antibodies responsible for hemolytic [perinatology.com]
Analysis of the ascitic fluid indicated that portal hypertension had caused the ascites. Although RHF could have caused it, we first had to ensure that it was not due to cirrhosis. [revespcardiol.org]
- Pansystolic Murmur
(Eisenmenger sendrome) 16 CHEST X-RAY Large VSD and light PVR Large VSD and high PVR 17 Patients with Moderate size VSD Pansystolic murmur Light – moderate left and right ventricular enlargement. 18 small VSD There is harsh pansystolic murmur due to small [slideplayer.com]
- Vascular Disease
disease Large VSD can have serious pulmonary resistance (Rp) in first 2 years of life 24 Pulmonary Vascular disease (Heath Edwards Classification) Grade 1: Medial hyperthrophy. [slideplayer.com]
Liver, Gall & Pancreas
- Hepatomegaly
(Elevated JVP, hepatomegaly, edema legs ) The mechanism of fluid retention are many .But, traditionally we have given importance to venous back pressure (Hydrostatic pressure). Equally important (if not more !) [drsvenkatesan.com]
VSD Rİ>20Ü/m² Qp/Qs 13 Patients with large VSD and increased Qp/Qs Weak peripheral pulses Symptoms and signs Patients with large VSD and increased Qp/Qs Weak peripheral pulses Tachypnea, subcostal drawings, profuse sweating Hepatomegaly, high jugular [slideplayer.com]
There was no hepatomegaly or pedal edema. [heartviews.org]
- Jaundice
Shock in Pediatrics Traumatic Brain Injury in Children Treatment of Sepsis and Septic Shock in Children Neonatology Anemia of Prematurity Apnea of Prematurity Benign Neonatal Sleep Myoclonus Birth Trauma Bowel Obstruction in the Newborn Breast Milk Jaundice [klikdokterjantung.wordpress.com]
Neurologic
- Asthenia
Clinical picture was not specific on clinical field: atypical chest pain, asthenia, syncope, cystolic murmur 1/6 Levine on the second left interspace were found. [rwjms1.umdnj.edu]
Workup
[…] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL). [4] Recognition of the disease requires a high index of suspicion and an extensive workup [en.wikipedia.org]
X-Ray
- Pericardial Effusion
To the best of our knowledge, a case of Ebstein's anomaly with recurrent massive pericardial effusion has not been described previously. 2016 BMJ Publishing Group Ltd. [ncbi.nlm.nih.gov]
To the best of our knowledge, a case of Ebstein's anomaly with recurrent massive pericardial effusion has not been described previously. [casereports.bmj.com]
Interpretation: Patients with moderate to large pericardial effusion at least 7 days after cardiac surgery are at risk of tamponade. Unfortunately, colchicine did not improve pericardial effusion grade or the incidence of tamponade. [acc.org]
Pulmonary hypoplasia may be a consequence of pericardial effusion during fetal life. [medcraveonline.com]
Blocks
- Right Bundle Branch Block
Intermediate Atrioventricular Septal Defects Pediatric Patent Foramen Ovale Atrial Septal Defects Pediatric Restrictive Cardiomyopathy Pediatric Rheumatic Heart Disease Pediatric Right Bundle Branch Block Pediatric Second-Degree Atrioventricular Block [klikdokterjantung.wordpress.com]
ECG at 18 years old showed sinus rhythm, atrial anomalies; incomplete right bundle branch block. Figure 4. [escardio.org]
ECG showed clockwise de viation of axis in the precordial leads and incomplete right bundle branch block. Chest X-ray showed marked levocardia and dilatation of pulmonary trunk. Levocardia increased with the patient on the left side. [rwjms1.umdnj.edu]
Treatment
Nonsteroidal anti-inflammatory drug treatment for postoperative pericardial effusion: a multicenter randomized, double-blind trial. [clinicaltrials.gov]
Initial treatment shortly after birth may be necessary to treat a baby's cyanosis because of inadequate pulmonary artery blood flow. [pted.org]
[…] of Infections of the Lung, Pleura, and Mediastinum Surgical Treatment of Pediatric Hypoplastic Left Heart Syndrome Systemic to Pulmonary Artery Shunting for Palliation Tetralogy of Fallot With Pulmonary Stenosis The Fontan Procedure for Pediatric Tricuspid [klikdokterjantung.wordpress.com]
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.de]
Organellar and miscellaneous neurodegenerative disorders 413 Lysosomal enzyme deficiencies 414 mucopolysaccharidoses 420 Mitochondrial disorders 425 Peroxisomal diseases 430 Miscellaneous metabolic disorders 434 Metabolic dysplasias susceptible to dietary treatment [books.google.com]
Prognosis
Treatment and prognosis As the anomaly is of variable severity, so is the prognosis. Severity is related to the amount of RVOT dysfunction and tricuspid regurgitation. Sudden death from arrhythmia may occur. [radiopaedia.org]
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]
When only the left coronary artery is anomalous, prognosis is relatively better. Again early death usually occurs from CHF & arrhythmia. Anomalous origin of the right coronary artery only carries the best prognosis. [cardiologyforyou.blogspot.com]
McElhinney DB, Silverman N (1999) Left ventricular aneurysm inthe fetus: a diagnosis with a mixed prognosis. Cardiol Young9: 123-126. [criticalcare.imedpub.com]
Etiology
CT chest is used as a diagnostic tool to establish the etiology. In our patient the pericardial cavity was large; the sternal wound had grown Acinetobacter baumannii. [elynsgroup.com]
The etiological study found no other causes in addition to those proposed a priori (alcohol and congestion), and the clinical, histopathological, and laboratory data indicated that congestion was the primary cause. 1 The echocardiogram still indicated [revespcardiol.org]
The etiology of these two t^^pes of congenital dextrocardia differs widely. [embryology.med.unsw.edu.au]
The pericardial effusion in our case was acquired and was tubercular in etiology. [heartviews.org]
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.de]
Epidemiology [ edit ] Lymphangiomatosis can occur at any age, but the incidence is highest in children and teenagers. [en.wikipedia.org]
Pathophysiology
However, as all that glitters is not gold.. all restrictive pathophysiology is not a restrictive-infiltrative cardiomyopathy.. let’s understand why! [escardio.org]
Pathophysiology: During fetal life: while PA pressure is higher than AO pressure, the anomalous coronary artery gets anterograde flow from PA. [cardiologyforyou.blogspot.com]
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.de]
[…] 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]
The indications for resection of pericardial cysts include large size, symptoms, patient concern, uncertainty of malignant potential, and prevention of the life threatening emergencies. [ctsnet.org]
The indications for resection of pericardial cysts are large size, symptoms, uncertainty of malignant potential and prevention of life-threatening emergencies. [annalspc.com]