Coarctation

The coarctation is a narrowing of the aorta, leading to an arterial hypertension in the upper extremities, left ventricular hypertrophy and a reduced blood flow to the viscera and lower extremities. Symptoms vary with the severity of deformity and can range from headache, rich chest pain, cold extremities, fatigue and claudication of the legs, to fulminant heart failure and shock. A faint sound is often heard about the coarctation. The diagnosis is made by echocardiography, CT or MRI. The treatment is balloon angioplasty with placement of a stent or surgical correction.

The coarctation accounts for 8-10% of congenital heart defects. It occurs in 10-20% before the patients with Turner syndrome. The ratio of male to female is 2: 1.

The coarctation is a narrowing of the aorta, leading to an arterial hypertension in the upper extremities, left ventricular hypertrophy and a reduced blood flow to the viscera and lower extremities. Symptoms vary with the severity of deformity and can range from headache, rich chest pain, cold extremities, fatigue and claudication of the legs, to fulminant heart failure and shock. A faint sound is often heard about the coarctation. The diagnosis is made by echocardiography, CT or MRI. The treatment is balloon angioplasty with placement of a stent or surgical correction. The coarctation accounts for 8-10% of congenital heart defects. It occurs in 10-20% before the patients with Turner syndrome. The ratio of male to female is 2: 1. Pathophysiology coarctation normally found arteriosus at the proximal thoracic aorta after the origin of the left subclavian artery and prior to the opening of the duct. Rarely the abdominal aorta is involved. Thus bypasses – in utero and before the open ductus arteriosus closes (PDA) – a large proportion of cardiac output stenosis on the PDA. The coarctation of the aorta can be alone or with other congenital malformations occur (eg. B. bicuspid aortic valve, VSD, aortic stenosis, patent ductus arteriosus, and Mitralklappenfehler intracerebral aneurysms). The physiological consequences include two phenomena: pressure overload in the arterial circulation proximal to the stenosis hypoperfusion distal to the stenosis, the pressure overload causes left ventricular hypertrophy and hypertension in the upper part of the body, including the brain. Hypoperfusion affects the abdominal organs and lower extremities. The malperfusion the gut increases the risk of sepsis due to enteric pathogens. Ultimately, the pressure drop increases the collateral circulation to the stomach and to the lower limbs on the arteries of the intermediate ribs of the inner breast, shoulder blade, and others. If untreated coarctation may lead to left ventricular failure, a rupture of the aorta, intracranial hemorrhage, hypertensive encephalopathy and hypertensive cardiovascular disease in adulthood. Symptoms and complaints If the coarctation is pronounced, it can lead to circulatory failure with renal insufficiency (oliguria and anuria) and metabolic acidosis in the first 7-10 days of life and resemble other systemic diseases such as sepsis. Infants with critical (severe) stenosis are probably acutely ill as soon as the ductus arteriosus constricts or closes. Less severe coarctation may be asymptomatic during childhood. Nonspecific symptoms (eg. As headaches, chest pain, fatigue and claudication in the legs during exercise) can occur in children as they grow older. High blood pressure in the upper extremities is often present, but heart failure often develops after the newborn period. Rarely rupture intracranial aneurysms and cause a subarachnoid or intracerebral hemorrhage. The typical findings on physical examination are high pulses and high blood pressure in the upper extremities, decreased or hardly palpable femoral pulse and a blood pressure drop with a low or a barely measurable blood pressure in the lower extremities. Systolic heart sounds of the Grades 2-3 / 6 are often present in the upper left sternal, in the left armpit and sometimes most clearly in the left interscapular region. Apical systoler ejection click is when a bicuspid aortic valve is present. The dilated intercostal arteries causing a continuous noise in the intercostal space. Affected women may one Turner syndrome have a congenital malformation with lymphedema of the legs, neck wings, a shield chest, valgisierten elbow and widely spaced nipples (Turner syndrome). Diagnostic x-ray thorax and ECG echocardiography, CT or MR angiography The diagnosis is suspected in the clinical examination (including the RR on all four limbs); supported by a chest X-ray absorption and an ECG and by two-dimensional Farbechokardiographie and Doppler sonography or in elderly patients secured with a suboptimal echocardiographic windows with CT or MR angiography. The chest X-ray shows a “3” in the left upper mediastinum. The heart size is normal, unless there is a failure before. The dilated intercostal collateral arteries can erode the 3rd to 8th ribs and cause so-called rib notching. These rib notching are rarely seen before age 5. The ECG usually shows left ventricular hypertrophy, but can also be normal. In neonates and young children in the ECG rather a right ventricular hypertrophy is seen as a left ventricular hypertrophy. In symptomatic treatment of newborns: infusion of prostaglandin E1 with high blood pressure: ?-blockers Surgical correction or balloon angioplasty (sometimes with stent implantation) Symptomatic newborns need a stabilization of the circulation with an infusion of prostaglandin E1 (0.01-0.10 g / kg / min, titrated) to the lowest effective dose to open the ductus arteriosus again. The opening of the duct and its aortic ampulla provides some relief by allowing the blood in the pulmonary artery to bypass the Aortenobstruktion through the ductus and increase the perfusion of the descending aorta, resulting in the improvement of systemic perfusion and to reverse the metabolic acidosis leads. Diuretics may help in the treatment of symptoms of heart failure. Additional O2 should be used in newborns with caution because the resulting decrease in pulmonary vascular resistance may increase the pulmonary blood flow at the expense of systemic blood flow. Intravenous cardioactive drugs (eg. As milrinone, dopamine, dobutamine) may be useful under selected circumstances (e. As children with heart failure and significant Linksventrikeldysfunktion). In non-acute situations patients can be treated with hypertension with ?-blockers. ACE inhibitors may have a negative effect on renal function. After a correction of Aortenverengung may persist high blood pressure or develop after years. This can be treated with ?-blockers, ACE inhibitors, angiotensin II receptor blockers or calcium channel blockers. The definitive treatment is controversial. Some centers prefer the balloon angioplasty with or without stent placement, but most prefer the surgical correction and reserve angioplasty for restenosis after surgical therapy or as first-line therapy with discreet Aortenverengung in older children or adolescents before. The initial success rate after balloon angioplasty is about 73% in patients with congenital stenosis and about 80% in patients with recurrent stenosis. Subsequent catheterization can dilate the stent when the children grow. The surgical options are resection and end-to-end anastomosis, an aortic patch plastic and an interposition between the left subclavian artery and the aorta with a Aortenplastik. With a heavy Aortenverengung that manifests early in life, the tranverse aorta and the isthmus are often hypoplastic, and this area of ??the aorta must be widened surgically. The decision on the surgical technique depends on the anatomical conditions and the preference of the center. The mortality for the surgical procedure is <5% in symptomatic infants and in <1% in older children. A residual stenosis is frequently observed (6-33%). On rare occasions by the disconnection of the aorta during surgery to paraplegia. Endocarditis prophylaxis is not necessary before surgery, but rather in the first 6 months after surgery. The important points coarctation is a localized narrowing of the lumen, typically in the proximal thoracic aorta after the origin of the left subclavian artery and prior to the opening of the ductus arteriosus. The manifestations depend on the severity of the stenosis, but typically include a pressure overload proximal to coarctation of the aorta, leading to heart failure, and hypoperfusion distal to the coarctation. Severe coarctation of the aorta may be in the neonatal period with acidosis, renal failure and shock manifest little coarctation may not be recognizable before a juvenile or adult is examined for hypertension. It is typically a blood pressure gradient between the upper and lower extremities in front of, and a degree of 2 to 3/6 systolic ejection noise is loudest sometimes left in the interscapular region. In symptomatic newborns prostaglandin E1 is infused to open the restricted ductus arteriosus again. Coarctation of the aorta is repaired surgically or using a balloon angioplasty with or without stent placement.

Health Life Media Team

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