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Fontan Procedure
The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, and finally published in 1973. Indications The Fontan Kreutzer procedure is used in pediatric patients who possess only a single functional ventricle, either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex ...
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Palliative
Palliative care (derived from the Latin root , or 'to cloak') is an interdisciplinary medical caregiving approach aimed at optimizing quality of life and mitigating suffering among people with serious, complex, and often terminal illnesses. Within the published literature, many definitions of palliative care exist. The World Health Organization (WHO) describes palliative care as "an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial, and spiritual." In the past, palliative care was a disease specific approach, but today the WHO takes a more broad approach, that the principles of palliative care should be applied as early as possible to any chronic and ultimately fatal illness. Palliative care is appropriate for individuals with ...
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Kawashima Procedure
The Kawashima procedure is used for congenital heart disease with a single effective ventricle and an ''interrupted inferior vena cava'' (IVC). It was first performed in 1978 and reported in 1984. __TOC__ Procedure Technically it is very similar to the bidirectional Glenn procedure used to direct half the body's venous blood flow into the lungs. However, in patients with interrupted IVC, most of the blood from the lower body actually joins the blood from the upper body before returning to the heart via the superior vena cava (SVC). Therefore, the redirection of SVC blood to the lungs (as in the Glenn) results in much more than half the venous blood flow being diverted. After Kawashima, the only de-oxygenated blood returning to the heart is from the abdominal organs (via the hepatic vein In human anatomy, the hepatic veins are the veins that drain venous blood from the liver into the inferior vena cava (as opposed to the hepatic portal vein which conveys blood from the gastr ...
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Heart Transplantation
A heart transplant, or a cardiac transplant, is a surgical transplant procedure performed on patients with end-stage heart failure or severe coronary artery disease when other medical or surgical treatments have failed. , the most common procedure is to take a functioning heart, with or without both lungs, from a recently deceased organ donor (brain death is the standard) and implant it into the patient. The patient's own heart is either removed and replaced with the donor heart ( orthotopic procedure) or, much less commonly, the recipient's diseased heart is left in place to support the donor heart (heterotopic, or "piggyback", transplant procedure). Approximately 3,500 heart transplants are performed each year worldwide, more than half of which are in the US. Post-operative survival periods average 15 years. Heart transplantation is not considered to be a cure for heart disease; rather it is a life-saving treatment intended to improve the quality and duration of life for a reci ...
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Hepatic Fibrosis
Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue repair and subsequent formation of scar tissue, which over time can replace normal functioning tissue, leading to the impaired liver function of cirrhosis. The disease typically develops slowly over months or years. Early symptoms may include tiredness, weakness, loss of appetite, unexplained weight loss, nausea and vomiting, and discomfort in the right upper quadrant of the abdomen. As the disease worsens, symptoms may include itchiness, swelling in the lower legs, fluid build-up in the abdomen, jaundice, bruising easily, and the development of spider-like blood vessels in the skin. The fluid build-up in the abdomen may become spontaneously infected. More serious complications include hepatic encephalopathy, bleeding from dilated veins ...
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Plastic Bronchitis
Plastic bronchitis (PB) is a disorder in which branching casts of the airways are expectorated. PB is not a single disease with a defined mechanism that explains the cast formation in all conditions. Examples of diseases associated with expectoration of casts, and which sometimes are labeled PB include tuberculosis, atypical mycobacterial disease, allergic bronchopulmonary aspergillosis, and asthma. When casts are very large with many branches, an abnormal communication or leakage of lymphatic fluid into the airway is often the cause. This entity is termed lymphatic plastic bronchitis (LPB). LPB is a lymphatic flow disorder characterized by the recurrent formation of branching, rubbery bronchial casts composed primarily of proteinaceous and sometimes chylous material and lymphocytes. Lymphatic fluids deposited into the airspaces become gelatinous as they cool, forming large string cheese-like casts of the airways, which can obstruct airflow. Attempts to expectorate casts can ...
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Protein Losing Enteropathy
Protein losing enteropathy refers to any condition of the gastrointestinal tract (e.g. damage to the gut wall) that results in a net loss of protein from the body. Signs and symptoms The signs/symptoms of protein losing enteropathy are consistent with diarrhea, fever, and general abdominal discomfort. Swelling of the legs due to peripheral edema can also occur, however, if the PLE is related to a systemic disease such as congestive heart failure or constrictive pericarditis, then the symptoms could be of the primary disease development. Causes The causes of protein-losing enteropathy can include GI conditions (among other causes), like the following: Mechanism The pathophysiology of protein losing enteropathy is a result of plasma proteins loss, which enters GI tract ( lumen). PLE is a complication of a disorder, be it lymphatic obstruction or mucosal injury. In ''pediatric protein losing enteropathy'' there are several changes in epithelial cells causing PLE by augmenting the ...
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Chylothorax
A chylothorax is an abnormal accumulation of chyle, a type of lipid-rich lymph, in the space surrounding the lung. The lymphatics of the digestive system normally returns lipids absorbed from the small bowel via the thoracic duct, which ascends behind the esophagus to drain into the left brachiocephalic vein. If normal thoracic duct drainage is disrupted, either due to obstruction or rupture, chyle can leak and accumulate within the negative-pressured pleural space. In people on a normal diet, this fluid collection can sometimes be identified by its turbid, milky white appearance, since chyle contains emulsified triglycerides. Chylothorax is a rare but serious condition, as it signals leakage of the thoracic duct or one of its tributaries. There are many treatments, both surgical and conservative. About 2–3% of all fluid collections surrounding the lungs (pleural effusions) are chylothoraces. It is important to distinguish a chylothorax from a pseudochylothorax (a pleural ...
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Children's Hospital Of Philadelphia
The Children's Hospital of Philadelphia (CHOP) is a children's hospital in Philadelphia, Pennsylvania, with its primary campus located in the University City neighborhood of West Philadelphia in the campus of the University of Pennsylvania. The hospital has 594 beds and more than 1 million outpatient and inpatient visits each year. It is one of the largest and oldest children's hospitals in the world, and United States' first hospital dedicated to the healthcare of children. CHOP has been ranked as the best children's hospital in the United States by '' U.S. News & World Report'' and ''Parents Magazine'' in recent years. As of 2020, it was ranked number one in the nation by U.S. News for three out of ten specialties. The hospital treats infants, children, teens, and young adults aged 0–21. The hospital also treats adults that would benefit from advanced pediatric care. The hospital is located next to the University of Pennsylvania and its physicians serve as the pediatrics depa ...
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Interventional Cardiologist
Interventional cardiology is a branch of cardiology that deals specifically with the catheter based treatment of structural heart diseases. Andreas Gruentzig is considered the father of interventional cardiology after the development of angioplasty by interventional radiologist Charles Dotter. Many procedures can be performed on the heart by catheterization. This most commonly involves the insertion of a sheath into the femoral artery (but, in practice, any large peripheral artery or vein) and cannulating the heart under X-ray visualization (most commonly fluoroscopy). The radial artery may also be used for cannulation; this approach offers several advantages, including the accessibility of the artery in most patients, the easy control of bleeding even in anticoagulated patients, the enhancement of comfort because patients are capable of sitting up and walking immediately following the procedure, and the near absence of clinically significant sequelae in patients with a normal Allen ...
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Hypoxia (medical)
Hypoxia is a condition in which the body or a region of the body is deprived of adequate oxygen supply at the tissue level. Hypoxia may be classified as either '' 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 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 lungs, reduced capacity of the blood to carry oxygen, compromised general ...
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Chest Tube
A chest tube (also chest drain, thoracic catheter, tube thoracostomy or intercostal drain) is a surgical drain that is inserted through the chest wall and into the pleural space or the mediastinum in order to remove clinically undesired substances such as air (pneumothorax), excess fluid (pleural effusion or hydrothorax), blood (hemothorax), chyle ( chylothorax) or pus (empyema) from the intrathoracic space. An intrapleural chest tube is also known as a Bülau drain or an intercostal catheter (ICC), and can either be a thin, flexible silicone tube (known as a "pigtail" drain), or a larger, semi-rigid, fenestrated plastic tube, which often involves a flutter valve or underwater seal. The concept of chest drainage was first advocated by Hippocrates when he described the treatment of empyema by means of incision, cautery and insertion of metal tubes. However, the technique was not widely used until the influenza epidemic of 1918 to evacuate post-pneumonic empyema, which was first ...
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