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Neonatal Lupus Erythematosus
Neonatal lupus erythematosus is an autoimmune disease in an infant born to a mother with anti-Ro/SSA and with or without anti-La/SSB antibodies. The disease most commonly presents with a rash resembling subacute cutaneous lupus erythematosus and can have systemic abnormalities such as complete heart block or hepatosplenomegaly. Neonatal lupus is usually benign and self-limited. Many of the clinical manifestations are transient, but certain heart problems can be permanent. Diagnosis is based on maternal antibodies and clinical manifestations. Treatment and management is mainly supportive and focused on preventing complete heart block if possible. Pathogenesis Ro/SSA and La/SSB are proteins found inside cells. Anti-Ro/SSA and anti-La/SSB are antibodies that form against these proteins. These antibodies can be seen in autoimmune diseases, the most common being Lupus and Sjögren's. Mothers can have these antibodies circulating in their blood without having any signs or symptoms of ...
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Neonatology
Neonatology is a subspecialty of pediatrics that consists of the medical care of newborn infants, especially the ill or premature newborn. It is a hospital-based specialty, and is usually practised in neonatal intensive care units (NICUs). The principal patients of neonatologists are newborn infants who are ill or require special medical care due to prematurity, low birth weight, intrauterine growth restriction, congenital malformations (birth defects), sepsis, pulmonary hypoplasia or birth asphyxia. Historical developments Though high infant mortality rates were recognized by the medical community at least as early as the 1860s, advances in modern neonatal intensive care have led to a significant decline in infant mortality in the modern era. This has been achieved through a combination of technological advances, enhanced understanding of newborn physiology, improved sanitation practices, and development of specialized units for neonatal intensive care. Around the mid-19th ce ...
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Atrium (heart)
The atrium ( la, ātrium, , entry hall) is one of two upper chambers in the heart that receives blood from the circulatory system. The blood in the atria is pumped into the heart ventricles through the atrioventricular valves. There are two atria in the human heart – the left atrium receives blood from the pulmonary circulation, and the right atrium receives blood from the venae cavae of the systemic circulation. During the cardiac cycle the atria receive blood while relaxed in diastole, then contract in systole to move blood to the ventricles. Each atrium is roughly cube-shaped except for an ear-shaped projection called an atrial appendage, sometimes known as an auricle. All animals with a closed circulatory system have at least one atrium. The atrium was formerly called the 'auricle'. That term is still used to describe this chamber in some other animals, such as the ''Mollusca''. They have thicker muscular walls than the atria do. Structure Humans have a four-chambered ...
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Ostium Secundum
The foramen secundum, or ostium secundum is a in the septum primum, a precursor to the interatrial septum of the human heart. It is not the same as the foramen ovale, which is an opening in the septum secundum. Development The foramen secundum () is formed from small perforations that develop in the septum primum. The septum primum is a that grows down between the single primitive atrium of the developing heart to separate it into left and right atria.Schoenwolf, Gary C., and William J. Larsen. Larsen's Human Embryology. 4th ed. Philadelphia: Churchill Livingstone/Elsevier, 2009. 363. Print. Closure Once a baby is born, blood should flow through the lungs, which now function to provide oxygen to the blood. The foramen secundum and foramen ovale act as a shunt where blood bypasses the lungs and does not become oxygenated. To provide proper blood flow as a newborn, the foramen secundum and foramen ovale must close at birth. Since the lungs now require a significant amount ...
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Chordae Tendineae
The chordae tendineae (tendinous cords), colloquially known as the heart strings, are inelastic cords of fibrous connective tissue that connect the papillary muscles to the tricuspid valve and the mitral valve in the heart. Structure The chordae tendineae connect the atrioventricular valves (tricuspid and mitral), to the papillary muscles within the ventricles. Multiple chordae tendineae attach to each leaflet or cusp of the valves. Chordae tendineae contain elastin in a delicate structure notably at their periphery. Tendon of Todaro The ''tendon of Todaro'' is a continuation of the Eustachian valve of the inferior vena cava and the valve of the coronary sinus. Along with the opening of the coronary sinus and the septal cusp of the tricuspid valve, it makes up Koch's triangle. The apex of Koch's triangle is the location of the atrioventricular node. Function During atrial systole, blood flows from the atria to the ventricles down the pressure gradient. Chordae tendineae are ...
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Pulmonic Stenosis
Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood. Signs and symptoms Cause Pulmonic stenosis is usually due to isolated valvular obstruction (pulmonary valve stenosis), but it may be due to subvalvular or supravalvular obstruction, such as infundibular stenosis. It may occur in association with other congenital heart defects as part of more complicated syndromes (for example, tetralogy of Fallot). Pathophysiology When pulmonic stenosis (PS) is present, resistance to blood flow causes right ventricular hypertrophy. If right ventricular failure develops, right atrial pressure will increase, and this may result in a persistent opening of the foramen ovale, shunting of unoxygenated blood from the right atrium into the left atrium, and systemic cyanosis. If pulmonary stenosis is severe, congestive heart failure occurs, and systemic venous engorgement will be noted. A ...
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Patent Foramen Ovale
Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO). It is common in patients with a congenital atrial septal aneurysm (ASA). After PFO closure the atria normally are separated by a dividing wall, the interatrial septum. If this septum is defective or absent, then oxygen-rich blood can flow directly from the left side of the heart to mix with the oxygen-poor blood in the right side of the heart; or the opposite, depending on whether the left or right atrium has the higher blood pressure. In the absence of other heart defects, the left atrium has the higher pressure. This can lead to lower-than-normal oxygen levels in the arterial blood that supplies the brain, organs, and tissues. However, an ASD m ...
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Patent Ductus Arteriosus
''Patent ductus arteriosus'' (PDA) is a medical condition in which the ''ductus arteriosus'' fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta, which has a higher pressure, to the pulmonary artery. Symptoms are uncommon at birth and shortly thereafter, but later in the first year of life there is often the onset of an increased work of breathing and failure to gain weight at a normal rate. With time, an uncorrected PDA usually leads to pulmonary hypertension followed by right-sided heart failure. The ''ductus arteriosus'' is a fetal blood vessel that normally closes soon after birth. In a PDA, the vessel does not close, but remains ''patent'' (open), resulting in an abnormal transmission of blood from the aorta to the pulmonary artery. PDA is common in newborns with persistent respiratory problems such as hypoxia, and has a high occurrence in premature newborns. Premature newborns are ...
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Endocardial Fibroelastosis
Endocardial fibroelastosis (EFE) is a rare heart disorder usually occurring in children two years old and younger. It may also be considered a reaction to stress, not necessarily a specific disease. It should not be confused with endomyocardial fibrosis. Signs and symptoms EFE is characterized by a thickening of the innermost lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibres. It is an uncommon cause of unexplained heart failure in infants and children, and is one component of HEC syndrome. Fibroelastosis is strongly seen as a primary cause of restrictive cardiomyopathy in children, along with cardiac amyloidosis, which is more commonly seen in progressive multiple myeloma patients and the elderly. Cause A review cites references to 31 different diseases and other stresses associated with the EFE reaction. These include infections, cardiomyopathies, immunologic diseases, congenital malformations, even ...
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Third-degree Atrioventricular Block
Third-degree atrioventricular block (AV block) is a medical condition in which the electrical impulse generated in the sinoatrial node (SA node) in the atrium of the heart can not propagate to the ventricles. Because the impulse is blocked, an accessory pacemaker in the lower chambers will typically activate the ventricles. This is known as an ''escape rhythm''. Since this accessory pacemaker also activates independently of the impulse generated at the SA node, two independent rhythms can be noted on the electrocardiogram (ECG). * The P waves with a regular P-to-P interval (in other words, a sinus rhythm) represent the first rhythm. * The QRS complexes with a regular R-to-R interval represent the second rhythm. The PR interval will be variable, as the hallmark of complete heart block is the lack of any apparent relationship between P waves and QRS complexes. Presentation People with third-degree AV block typically experience severe bradycardia (an abnormally low measured heart ...
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Second-degree Atrioventricular Block
Second-degree atrioventricular block (AV block) is a disease of the electrical conduction system of the heart. It is a conduction block between the atria and ventricles. The presence of second-degree AV block is diagnosed when one or more (but not all) of the atrial impulses fail to conduct to the ventricles due to impaired conduction. It is classified as a block of the AV node and is categorized in between first-degree (slowed conduction) and third degree blocks (complete block). Signs and symptoms Most people with Wenckebach (Type I Mobitz) do not show symptoms. However, those that do usually display one or more of the following: * Light-headedness * Dizziness * Syncope (fainting) Types There are two non-distinct types of second-degree AV block, called ''Type 1'' and ''Type 2''. In both types, a P wave is blocked from initiating a QRS complex; but, in Type 1, there are increasing delays in each cycle before the omission, whereas, in Type 2, there is no such pattern. Typ ...
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First-degree Atrioventricular Block
First-degree atrioventricular block (AV block) is a disease of the electrical conduction system of the heart in which electrical impulses conduct from the cardiac atria to the ventricles through the atrioventricular node (AV node) more slowly than normal. First degree AV block does not generally cause any symptoms, but may progress to more severe forms of heart block such as second- and third-degree atrioventricular block. It is diagnosed using an electrocardiogram, and is defined as a PR interval greater than 200 milliseconds. First degree AV block affects 0.65-1.1% of the population with 0.13 new cases per 1000 persons each year. Causes The most common causes of first-degree heart block are AV nodal disease, enhanced vagal tone (for example in athletes), myocarditis, acute myocardial infarction (especially acute inferior MI), electrolyte disturbances and medication. The medications that most commonly cause first-degree heart block are those that increase the refractory ti ...
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Complete Heart Block
Third-degree atrioventricular block (AV block) is a medical condition in which the electrical impulse generated in the sinoatrial node (SA node) in the atrium of the heart can not propagate to the ventricles. Because the impulse is blocked, an accessory pacemaker in the lower chambers will typically activate the ventricles. This is known as an ''escape rhythm''. Since this accessory pacemaker also activates independently of the impulse generated at the SA node, two independent rhythms can be noted on the electrocardiogram (ECG). * The P waves with a regular P-to-P interval (in other words, a sinus rhythm) represent the first rhythm. * The QRS complexes with a regular R-to-R interval represent the second rhythm. The PR interval will be variable, as the hallmark of complete heart block is the lack of any apparent relationship between P waves and QRS complexes. Presentation People with third-degree AV block typically experience severe bradycardia (an abnormally low measured heart ...
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