What is endocardial Fibroelastosis?
What is endocardial Fibroelastosis?
Endocardial fibroelastosis (EFE) is a rare heart disorder that affects infants and children. It is characterized by a thickening within the muscular lining of the heart chambers due to an increase in the amount of supporting connective tissue (inelastic collagen) and elastic fibers. The normal heart has four chambers.
What does Fibroelastosis mean?
Medical Definition of fibroelastosis : a condition of the body or one of its organs (as the left ventricle of the heart) characterized by proliferation of fibroelastic tissue — see endocardial fibroelastosis.
What is a endocardial?
The endocardium is the innermost layer of the heart and lines the chambers and extends over projecting structures such as the valves, chordae tendineae, and papillary muscles.
What is Efe in Ultrasound?
Introduction. Fetal endocardial fibroelastosis (EFE) is a type of rare fetal cardiac malformation characterized by the diffuse thickness of the ventricular endocardium. The fundamental pathological changes of EFE include hyperplasia of endocardium elastic fibers and collagenous fibers.
What are endocardial tubes?
The endocardial tubes are paired regions in the embryo that appear in its ventral pole by the middle of the third week of gestation and consist of precursor cells for the development of the embryonic heart.
What are endocardial cushions?
The endocardial cushions are two thicker areas that develop into the walls (septum) that divide the four chambers of the heart. They also form the mitral and tricuspid valves. These are the valves that separate the atria (top collecting chambers) from the ventricles (bottom pumping chambers).
What causes Pleuroparenchymal Fibroelastosis?
The cause of idiopathic pleuroparenchymal fibroelastosis is unknown, but clinical data suggest a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms are also thought to play a role in this disease.
What is pleural parenchymal Fibroelastosis?
Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upper-lobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough.
What is epicardial and endocardial?
This procedure involves both Epicardial (surgical) and Endocardial (catheter) ablation. (Ablation treats irregular heartbeat by creating scar tissue in the area of the heart that is causing the problem.)
What is endocardial infarction?
In myocardial infarction, ischemia of the myocardium starts at the endocardium and might extend up to the epicardium, disrupting the entire heart wall (“transmural” infarction). Less extensive infarctions are often “subendocardial” and do not affect the epicardium.
When is fetal echo done?
Fetal echocardiography is a test that is done while the baby is still in the womb. It is most often done during the second trimester of pregnancy. This is when a woman is about 18 to 24 weeks pregnant. The procedure is similar to that of a pregnancy ultrasound.
What is a dating ultrasound?
A dating scan is an ultrasound scan done between 8 and 14 weeks of pregnancy to help estimate your baby’s due date. In early pregnancy, most babies of the same gestational age are about the same size.
Is endocardial tube the same as heart tube?
As folding of the embryo in the horizontal plane initiates in the 4th week of gestation, the endocardial tubes meet in the midline to form the primitive heart tube, which will eventually develop into the histologically definitive endocardium.
What tissue is formed by endocardial tubes?
Later, the endocardial tubes fuse to form part of the primordial heart tube (with the myocardium). The non-fused posterior portions of the endocardium become the openings of the vitelline veins, which feed into the heart, via the sinus venosus.
How common is endocardial cushion defect?
The frequency rate of endocardial cushion defect (atrioventricular [AV] canal or septal defects) is about 3% of children with congenital heart disease.
How many endocardial cushions are there?
two
Causes. ECD occurs while a baby is still growing in the womb. The endocardial cushions are two thicker areas that develop into the walls (septum) that divide the four chambers of the heart. They also form the mitral and tricuspid valves.
What is Pleuroparenchymal Fibroelastosis?
What is idiopathic Pleuroparenchymal Fibroelastosis?
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial …
What does pleural thickening indicate?
Pleural Thickening and Asbestos. Pleural thickening is a disease that causes thickening of the lung lining, or pleura. Symptoms of pleural thickening may include chest pain and difficulty breathing. Pleural thickening can be a sign of significant asbestos exposure and may indicate pleural mesothelioma or lung disease.
How serious is pulmonary fibrosis?
Pulmonary fibrosis is a serious, lifelong lung disease. It causes lung scarring (tissues scar and thicken over time), making it harder to breathe. Symptoms may come on quickly or take years to develop. No cure exists.
What is endocardial fibroelastosis (EFE)?
Endocardial fibroelastosis (EFE) is primarily a disease of infants and children, but can rarely present in adulthood as well.[1]
How is the diagnosis of endocardial fibroelastosis confirmed?
The diagnosis of endocardial fibroelastosis is confirmed by a thorough clinical evaluation, including a physical examination that may reveal signs of respiratory distress (i.e., moist rales) and galloping heart rhythms.
What is the prevalence of endocardial fibroelastosis?
Endocardial fibroelastosis is a rare disorder that affects males and females in equal numbers. Fewer than 1 percent of infants and children with congenital heart disease are diagnosed with this disorder. A 1964 study suggested an incidence of 1 in 5,000 live births.
What is the history of endocardial fibroelastosis?
The term “endocardial fibroelastosis” was introduced by Weinberg and Himmelfarb in 1943. In their pathology laboratory they noted that usually the endocardium was pearly white or opaque instead of normally thin and transparent and microscopically showed a systematic layering of collagenous and elastic fibers.
