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Total Anomalous Pulmonary Venous Connection
Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital heart defect of the pulmonary veins. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned. Signs and symptoms * Right ventricular heave * Loud S1 * Fixed split S2 * S3 gallop * Systolic ejection murmur at left upper sternal border * Cardiomegaly * Right ventricular hypertrophy and right axis deviation on ECG * Cyanosis, tachypnea and dyspnea, since the overloaded pulmonary circuit can cause pulmonary edema * ''Cottage-loaf sign'', that is, chest X-ray appearance similar to a cottage loaf, also known as the 'snow man' sign or 'figure of 8' sign. Total anomalous pulmonary venous connection (TAPVR) ''Total anomalous pulmonary venous connection'', also known as total ' ...
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Pulmonary Vein
The pulmonary veins are the veins that transfer Blood#Oxygen transport, oxygenated blood from the lungs to the heart. The largest pulmonary veins are the four ''main pulmonary veins'', two from each lung that drain into the left atrium of the heart. The pulmonary veins are part of the pulmonary circulation. Structure There are four main pulmonary veins, two from each lung – an inferior and a superior main vein, emerging from each lung hilum, hilum. The main pulmonary veins receive blood from three or four feeding veins in each lung, and drain into the left atrium. The peripheral feeding veins do not follow the bronchial tree. They run between the pulmonary segments from which they drain the blood. At the root of the lung, the right superior pulmonary vein lies in front of and a little below the pulmonary artery; the inferior is situated at the lowest part of the lung hilum. Behind the pulmonary artery is the bronchus. The right main pulmonary veins (contains oxygenated bloo ...
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Congenital Heart Defect
A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, 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 are variable and 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. Congenital heart defects are the most common birth defect. In 2015, they were present in 48.9 million people globally. They affect between 4 and 75 per 1,000 live births, depending upon how they are diagnosed. In about 6 to 19 per 1,000 they cause a moderate to severe degree of problems. Congenital heart defects are t ...
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Echocardiography
Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an echocardiogram, a cardiac echo, or simply an echo. Echocardiography is routinely used in the diagnosis, management, and follow-up of patients with any suspected or known heart diseases. It is one of the most widely used diagnostic imaging modalities in cardiology. It can provide a wealth of helpful information, including the size and shape of the heart (internal chamber size quantification), pumping capacity, location and extent of any tissue damage, and assessment of valves. An echocardiogram can also give physicians other estimates of heart function, such as a calculation of the cardiac output, ejection fraction, and diastolic function (how well the heart relaxes). Echocardiography is an important tool in assessing wall motion abnorma ...
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Angiography
Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is performed by injecting a radio-opaque contrast agent into the blood vessel and imaging using X-ray based techniques such as fluoroscopy. With time-of-flight (TOF) magnetic resonance it is no longer necessary to use a contrast. The word itself comes from the Greek words ἀνγεῖον ''angeion'' 'vessel' and γράφειν ''graphein'' 'to write, record'. The film or image of the blood vessels is called an ''angiograph'', or more commonly an ''angiogram''. Though the word can describe both an arteriogram and a venogram, in everyday usage the terms angiogram and arteriogram are often used synonymously, whereas the term venogram is used more precisely. The term angiography has been applied to radionuclide angiography and newer vascular ima ...
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CT Scan
A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or radiology technologists. CT scanners use a rotating X-ray tube and a row of detectors placed in a gantry (medical), gantry to measure X-ray Attenuation#Radiography, attenuations by different tissues inside the body. The multiple X-ray measurements taken from different angles are then processed on a computer using tomographic reconstruction algorithms to produce Tomography, tomographic (cross-sectional) images (virtual "slices") of a body. CT scans can be used in patients with metallic implants or pacemakers, for whom magnetic resonance imaging (MRI) is Contraindication, contraindicated. Since its development in the 1970s, CT scanning has proven to be a versatile imaging technique. While CT is most prominently used in medical diagnosis, i ...
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Turner Syndrome
Turner syndrome (TS), commonly known as 45,X, or 45,X0,Also written as 45,XO. is a chromosomal disorder in which cells of females have only one X chromosome instead of two, or are partially missing an X chromosome (sex chromosome monosomy) leading to the complete or partial deletion of the pseudoautosomal regions (PAR1, PAR2) in the affected X chromosome. Typically, people have two sex chromosomes (XX for females or XY for males). The chromosomal abnormality is often present in just some cells, in which case it is known as Turner syndrome with mosaicism. 45,X0 with mosaicism can occur in males or females, but Turner syndrome without mosaicism only occurs in females. Signs and symptoms vary among those affected but often include additional skin folds on the neck, arched palate, low-set ears, low hairline at the nape of the neck, short stature, and lymphedema of the hands and feet. Those affected do not normally develop menstrual periods or mammary glands without hormone trea ...
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Total Anomalous Pulmonary Venous Connection
Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital heart defect of the pulmonary veins. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned. Signs and symptoms * Right ventricular heave * Loud S1 * Fixed split S2 * S3 gallop * Systolic ejection murmur at left upper sternal border * Cardiomegaly * Right ventricular hypertrophy and right axis deviation on ECG * Cyanosis, tachypnea and dyspnea, since the overloaded pulmonary circuit can cause pulmonary edema * ''Cottage-loaf sign'', that is, chest X-ray appearance similar to a cottage loaf, also known as the 'snow man' sign or 'figure of 8' sign. Total anomalous pulmonary venous connection (TAPVR) ''Total anomalous pulmonary venous connection'', also known as total ' ...
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Left Atrium
The atrium (; : atria) is one of the 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 mitral and tricuspid heart 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, previously 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''. Auricles in this modern terminology are distinguished by having thicker mus ...
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Patent Ductus Arteriosus
Patent ductus arteriosus (PDA) is a medical condition in which the ''ductus arteriosus'' fails to close after childbirth, birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs from the aorta, which has a higher blood pressure, to the pulmonary artery, which has a lower blood pressure. 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 thrive, 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 circulation, fetal blood vessel that normally closes soon after birth. This closure is caused by vessel constriction immediately after birth as circulation changes occur, followed by the occlusion of the vessel’s lumen in the following days. In a PDA, the vessel does not close, but remains ''patent'' (ope ...
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PGE1
Prostaglandin E1 (PGE1) is a naturally occurring prostaglandin with various medical uses. Alprostadil and misoprostol are synthetic forms of prostaglandin E1 used as medications. Lubiprostone, a derivative of prostaglandin E1, is also used as a medication. Prostaglandin E1 is a vasodilator. It has various effects in the body that include opening blood vessels, relaxing smooth muscle, inhibiting clotting, and causing uterine contractions. In infants with certain congenital heart defects, alprostadil is delivered by slow injection into a vein to maintain a patent ductus arteriosus until surgery can be carried out. By injection into the penis or placement in the urethra, alprostadil is used to treat erectile dysfunction. Common side effects when given to babies include decreased breathing, fever, and low blood pressure. When injected into the penis for erectile dysfunction; side effects may include penile pain, bleeding at the site of injection, and prolonged erection (priapi ...
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Hypoxia (medical)
Hypoxia is a condition in which the body or a region of the body is deprived of an adequate oxygen supply at the tissue (biology), tissue level. Hypoxia may be classified as either ''Generalized hypoxia, generalized'', affecting the whole body, or ''local'', affecting a region of the body. Although hypoxia is often a pathological condition, variations in arterial oxygen concentrations can be part of the normal physiology, for example, during strenuous physical exercise. Hypoxia differs from hypoxemia and anoxemia, in that hypoxia refers to a state in which oxygen present in a tissue or the whole body is insufficient, whereas hypoxemia and anoxemia refer specifically to states that have low or no Oxygen saturation (medicine), oxygen in the blood. Hypoxia in which there is complete absence of oxygen supply is referred to as anoxia. Hypoxia can be due to external causes, when the breathing gas is hypoxic, or internal causes, such as reduced effectiveness of gas transfer in the lung ...
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Congenital
A birth defect is an abnormal condition that is present at childbirth, birth, regardless of its cause. Birth defects may result in disability, disabilities that may be physical disability, physical, intellectual disability, intellectual, or developmental disability, developmental. The disabilities can range from mild to severe. Birth defects are divided into two main types: structural disorders in which problems are seen with the shape of a body part and functional disorders in which problems exist with how a body part works. Functional disorders include metabolic disorder, metabolic and degenerative disease, degenerative disorders. Some birth defects include both structural and functional disorders. Birth defects may result from genetic disorder, genetic or chromosome abnormality, chromosomal disorders, exposure to certain medications or chemicals, or certain vertically transmitted infection, infections during pregnancy. Risk factors include folate deficiency, alcohol drink, d ...
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