Lutembacher's Syndrome
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Lutembacher's Syndrome
Lutembacher's syndrome is a very rare form of congenital heart disease that affects one of the chambers of the heart (commonly the atria) as well as a valve (commonly the mitral valve). It is commonly known as both congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). Congenital (at birth) atrial septal defect refers to a hole being in the septum or wall that separates the two atria; this condition is usually seen in fetuses and infants. Mitral stenosis refers to mitral valve leaflets (or valve flaps) sticking to each other making the opening for blood to pass from the atrium to the ventricles very small. With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. Septal defects that may occur with Lutembacher's syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent. Lutembacher's syndrome affects females more often than males. It can affect children or adults; the person ca ...
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Congenital Heart Disease
A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure. The cause of a congenital heart defect is often unknown. Risk factors include certain infections during pregnancy such as rubella, use of certain medications or drugs such as alcohol or tobacco, parents being closely related, or poor nutritional status or obesity in the mother. Having a parent with a congenital heart defect is also a risk factor. A number of genetic condition ...
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Ventricular Septal Defect
A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes. The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children in the United States. It is also the type that will most commonly require surgical intervention, comprising over 80% of cases. Membranous ventricular septal defects are more common than muscular ventricular septal defects, and are the most common congenital cardiac anomaly. Signs and symptoms Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth. VSD is an acyanotic congenital heart defect, aka a lef ...
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Congenital Heart Defects
A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure. The cause of a congenital heart defect is often unknown. Risk factors include certain infections during pregnancy such as rubella, use of certain medications or drugs such as alcohol or tobacco, parents being closely related, or poor nutritional status or obesity in the mother. Having a parent with a congenital heart defect is also a risk factor. A number of genetic condition ...
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EUROCAT (medicine)
EUROCAT is a European organization that describes itself as "a network of population-based registries for the epidemiological surveillance of congenital anomalies, covering 1.5 million births in 20 countries of Europe". They were founded in 1979, with the goal of improving the collection of data about congenital disorders, and the standardization of that data. They have published extensions to ICD-10 Chapter Q, which helps to provide unique codes for individual conditions. , approximately a quarter of new births in the European Union are reported to EUROCAT. See also * Rare disease A rare disease is any disease that affects a small percentage of the population. In some parts of the world, an orphan disease is a rare disease whose rarity means there is a lack of a market large enough to gain support and resources for discove ... References External links * * * * * * Databases in the United Kingdom International medical associations of Europe Genetic diseases and ...
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Mitral Valvuloplasty
Mitral stenosis is a valvular heart disease characterized by the narrowing of the opening of the mitral valve of the heart. It is almost always caused by rheumatic valvular heart disease. Normally, the mitral valve is about 5 cm2 during diastole. Any decrease in area below 2 cm2 causes mitral stenosis. Early diagnosis of mitral stenosis in pregnancy is very important as the heart cannot tolerate increased cardiac output demand as in the case of exercise and pregnancy. Atrial fibrillation is a common complication of resulting left atrial enlargement, which can lead to systemic thromboembolic complications like stroke. Signs and symptoms Signs and symptoms of mitral stenosis include the following: * Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea (PND) * Palpitations * Chest pain * Hemoptysis * Thromboembolism in later stages when the left atrial volume is increased (i.e., dilation). The latter leads to increase risk of ...
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Ventricular Septal Defect
A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes. The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children in the United States. It is also the type that will most commonly require surgical intervention, comprising over 80% of cases. Membranous ventricular septal defects are more common than muscular ventricular septal defects, and are the most common congenital cardiac anomaly. Signs and symptoms Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth. VSD is an acyanotic congenital heart defect, aka a lef ...
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Mitral Regurgitation
Mitral regurgitation (MR), also known as mitral insufficiency or mitral incompetence, is a form of valvular heart disease in which the mitral valve is insufficient and does not close properly when the heart pumps out blood.Mitral valve regurgitation
at Mount Sinai Hospital
It is the abnormal leaking of blood backwards – regurgitation from the , through the mitral valve, into the

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Blausen 0648 MitralValveStenosis
Blausen Medical Communications, Inc. is the creator and owner of a library of two- and three-dimensional medical and scientific images and animations, a developer of information technology allowing access to that content, and a business focused on licensing and distributing the content. It was founded by Bruce Blausen in Houston, Texas, in 1991, and is privately held. Background Blausen Medical Communications, Inc. (BMC) is a privately held company founded by Bruce Blausen in Houston, Texas in 1991. BMC created and owns a library of medical and scientific images and animations, and has developed information technology tools allowing access to the library; as well, it licenses and otherwise works to distribute the content. As of this date, BMC's animation library comprised approximately 1,500 animations and over 27,000 two- and three-dimensional images designed for point-of-care patient education, which could be accessed by consumers or professional caregivers (primarily via h ...
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Blausen 0069 AtrialSeptalDefect 02
Blausen Medical Communications, Inc. is the creator and owner of a library of two- and three-dimensional medical and scientific images and animations, a developer of information technology allowing access to that content, and a business focused on licensing and distributing the content. It was founded by Bruce Blausen in Houston, Texas, in 1991, and is privately held. Background Blausen Medical Communications, Inc. (BMC) is a privately held company founded by Bruce Blausen in Houston, Texas in 1991. BMC created and owns a library of medical and scientific images and animations, and has developed information technology tools allowing access to the library; as well, it licenses and otherwise works to distribute the content. As of this date, BMC's animation library comprised approximately 1,500 animations and over 27,000 two- and three-dimensional images designed for point-of-care patient education, which could be accessed by consumers or professional caregivers (primarily via h ...
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Septum Primum
During heart development of a human embryo, the single primitive atrium becomes divided into right and left by a , the septum primum. The septum primum () grows downward into the single atrium. Development The gap below it is known as the ostium primum (), and becomes increasingly small. The septum primum eventually fuses with the endocardial cushion, closing the ostium primum off completely. Meanwhile, perforations appear in the superior part of the septum primum, forming the ostium secundum (). The septum primum will eventually form part of the fossa ovalis. Blood flow between atria will continue through the foramen ovale (heart). Clinical significance Failure of the septum primum to fuse with the endocardial cushion can lead to an ostium primum atrial septal defect. This is the second most common type of atrial septal defect and is commonly seen in Down syndrome. Typically this defect will cause a shunt to occur from the left atrium to the right atrium. Children born with t ...
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Ebstein's Anomaly
Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. It is classified as a critical congenital heart defect accounting for less than 1% of all congenital heart defects presenting in around per 200,000 live births. Ebstein anomaly is the congenital heart lesion most commonly associated with supraventricular tachycardia. Signs and symptoms The annulus of the valve is still in the normal position. The valve leaflets, however, are to a varying degree, attached to the walls and septum of the right ventricle. A subsequent "atrialization" of a portion of the morphologic right ventricle (which is then contiguous with the right atrium) is seen. This causes the right atrium to be large and the anatomic right ventricle to be small in size. * S3 heart sound * S4 heart sound * Triple or quadruple gallop due to widely split S1 and S2 sounds plus a loud S3 and/or S4 ...
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Mitral Regurgitation
Mitral regurgitation (MR), also known as mitral insufficiency or mitral incompetence, is a form of valvular heart disease in which the mitral valve is insufficient and does not close properly when the heart pumps out blood.Mitral valve regurgitation
at Mount Sinai Hospital
It is the abnormal leaking of blood backwards – regurgitation from the , through the mitral valve, into the