coarctation of the aorta

I. Introduction
Aortic KOARTASIO is a disorder that occurs in the form of narrowing of the aorta near the branching of the left subclavian artery from the aortic arch and the base of the duct arteriousus battoli.
Koartasio aorta is a narrowing of the aorta, which usually occurs at the point where the ductus arteriosus is connected to the aorta and aortic turned down.
The aorta is the main artery of the body. The aorta circulate oxygen-rich blood throughout the body, except the lungs. The first branch of the aortic blood flow to the upper body (arms and head). Then the blood flow to the lower body (abdomen and legs). Coarctation is derived from the Latin coartatio (tension or pressure). Aortic coarctation is defined as a narrowing of the aortic lumen and cause obstruction of blood flow. Paris was first described in 1791 coarctation of the aorta, although Meckel in 1750 and Morgagni in 1760 has reported the discovery of a narrowing of the aorta at autopsy. In 1944 Crafoord perform corrective surgery for the first time with coarctation resection and end-to-end anastomosis.

Location classic coarctation is usually found on the thoracic aorta distal to the left subclavian artery, at the site of insertion of the ductus arteriosus (jukstaduktal). Although rare, coarctation segment can also be found in the area thorakal lower aorta or abdominal aorta. Aortic coarctation is often associated with other congenital cardiac defects especially, PDA, VSD, bicuspid aortic valve, subaortik obstruction and mitral valve disease.
II. epidemiology
In the United States, the incidence of coarctation of the aorta approximately 5-8% of all congenital heart defects. Internationally, the incidence of coarctation appears to be low (<2%) in Asian countries than in Europe and North America.
Approximately 90% of deaths due to uncorrected aortic coarctation occurred at the age of 50 years with an average age of 35 years. Race has no effect on the prevalence of aortic coarctation. Comparison between men and women is 2: 1, but in the abdominal aortic coarctation, women have a higher risk. The ratio between the thoracic and abdominal aortic coarctation is 1000: 1. In terms of age, most of the patients had symptoms and signs in the first years of life due to congestive heart failure, or at an older age because of hypertension.

III. Anatomy and Fetal Circulation

1. The walls of the arteries
Arterial wall consists of three tunica, tunica adventitia (outer), tunica media and tunica intima (in). The tunica adventitia contains nerves and blood vessels (vasa vasorum) that supply the walls of the arteries and is composed of connective tissue that gives full power to the artery wall. The tunica media is composed of collagen, smooth muscle fibers, and elastin. which takes full responsibility in regulating blood vessel diameter when dilation and constriction. Tunica intima is smooth endothelial cells that provide nontrombogenik surface for blood flow. The tunica media and intima got food from the diffusion process of arterial blood flow.

A) Pieces of transverse normal aorta in newborns right at the site of insertion of the ductus arteriosus (DA). Colored duct network brighter than aortic tissue due to relatively fewer contains elastin. A third part of the aortic elastin layer (Ao) fused to the internal elastic lamina (iel) duct, and two-thirds of the outside of the tunica adventitia meyatu (ad), resulting in a picture of a fish-like tail (asterisk) on the relations between the two walls of the blood vessels. Duct network does not exceed one third of the total circle aorta.

B) Pieces of transverse aorta in infants with coarctation of the site of insertion of the ductus arteriosus. Bright colors of duct tissue is clearly visible around the lumen of the aorta. Little bearing intima appears on the specimen.

2. The aorta and its main branches

Aorta berjalalan across chest and abdomen cavity, and the segments of the aorta is named according to its location. The aorta is divided into several segments torasika anaomi the following: 1) the ascending aorta torasika, 2) the transverse aortic arch, and 3) the descending aorta torasika. Starting from the ascending aorta and aortic valve estuary extends to some blood vessels that supply the head, neck and upper extremities. Blood vessels are collectively called brachiocephalic veins, which originate from the aortic arch. Brachiocephalic vessels including arteries inominata (brachiocephalic trunk), the left common carotid artery and the left subclavian artery. Inominata artery divides into the right common carotid artery and the right subclavian artery. Axillary artery originating from the subclavian artery and the brachial artery continues to be, that later branched into the radial and ulnar arteries. Vertebral artery originating from the subclavian arteries bilaterally.
Torasika descending aorta originated from the distal part of the left subclavian artery and continues to the diaphragm. Originated from the abdominal aorta below the diaphragm and branches after walking a few centimeters to supply the abdominal organs. Posterior part of the aorta running towards the lungs, diaphragm, small intestine, spleen, stomach and intestines. Major visceral branches of the abdominal aorta, including the celiac axis, superior mesentrika arteries, and renal arteries. The abdominal aorta continues as the aortic bifurcation to the level of the pelvis. The aorta is the terminal part of the aorta between the renal arteries and the aortic bifurcation; mesentrika inferior artery is the main branch of the terminal aorta.
The abdominal aorta branching into common iliac artery. Common iliac artery branched into the external iliac artery and hypogastric or internal iliac artery. External iliac artery continues into the femoral artery communist. Communist femoral artery has many branches, among others, superficial femoral artery and the deep femoral artery. Superficial femoral artery continues down as the popliteal artery branching into the posterior tibial artery, peronealis artery and the anterior tibial artery. The anterior tibial artery continues into the dorsalis pedis artery.

3. Fetal Circulation
All the needs of the fetus, including the oxygen needed for survival was obtained from the mother through the placenta, as well as any materials that are not required include carbonic acid expelled again through the placenta. Blood from the placenta contains 60-70% O2. From the umbilical vein, most of the blood flow through the heart and the rest through the ductus venosus reach vana cava and then arrives in the right atrium. Most of the blood is then passed through the foramen ovale into the left atrium and the left ventricle further. From the left ventricle, blood is pumped to the pressure ± 60 mmHg in the ascending aorta. In this place there is a separation that most of the blood flowing through blood vessels to supply O2 to the coronary circulation, the head and upper limbs, and then to and arriving at the superior vena cava. The rest are in the ascending aorta flows into the descending aorta.
Of the superior vena cava, blood flows into the right atrium, then to the right ventricle and onward into the pulmonary artery. Blood pressure in the pulmonary artery is the same as the pressure in the ascending aorta. Given the lung capillaries and alveoli fetus still does not work (high pulmonary vascular resistance, the muscles are still constricting the pulmonary artery and pulmonary artery tunica media is thicker), so most of the blood that is in the pulmonary artery (85%) will flow through the ductus botalli into the descending aorta, so most of the blood to the tool in the stomach and lower limbs, and partly through the umbilical artery flow back to the placenta.
The first inspiration that occurs soon after birth causes open alveoli and pulmonary artery vasoconstriction disappeared. This process resulted in a slight widening of the blood vessels in the lungs so that the function of the pulmonary circulation with blood from the pulmonary artery. Botalli close the duct wall (thrombosis and fibrosis) even in the days or weeks early is open again if the baby cries, straining or hypoxia. Closure of the foramen ovale occurs, placental circulation stops, and the left ventricle, with the amount of blood is approximately equal to the amount of blood before the baby is born, to supply all the needs of the body. Immediately after birth, the levels of arterial O2 dampai increased 100%, while the levels in venous blood depends on the place where it was taken for examination.
Diagram of fetal circulation to normal. A) superior vena cava blood flow (VKS) (blue arrow) directly through the tricuspid valve into the pulmonary artery through the ductus arteriosus to the lower body segment. The flow of the inferior vana cava (VKI) (red arrow) carries oxygenated blood from the placenta directly through the foramen ovale into the left ventricle, the ascending aorta and the upper body segment, with a little stream that passes through the isthmus which lies between the left subclavian artery and the ductus arteriosus. B) koaktasio aorta in the fetus will not affect the pattern of blood flow. C) After birth, there is a decrease in pulmonary resistance and increased blood flow in the pulmonary artery (blue arrow) and forward blood flow from the aortic arch to the descending aorta. D) As the closure of the ductus, the narrowing is more severe in areas koarktasi, and increased obstruction depends on the gradient.

IV. aetiopathogenesis
The risk of aortic koartasio increased in some genetic circumstances, such as Turner syndrome (gonadal disgenosis: a state in girls, which one of the chromosomes Xnya missing part or missing entirely). Koartasio aorta is also associated with congenital abnormalities of the aortic valve (eg bicuspid valve). This disorder is found in 1 out of 10,000 people. Usually diagnosed in childhood or adult under 40 years. Mechanisms underlying aortic coarctation remains unclear unknown. There are two hypotheses that are often put forward the theory ductal tissue and reduced flow theory.

1. Theory Ductal Tissue
Network ductus arteriosus invaded the descending aorta distal to the aortic isthmus. When the ductus arteriosus constrict, there coarctation. This theory is supported by the fact that neonatal coarctation manifest after the closure of the ductus arteriosus (infantile type) and usually have more severe symptoms. Obstruction appears as indentation on the side of the posterolateral thoracic descending aorta dealing with the insertion of the ductus arteriosus. Nevertheless this theory fails to explain the occurrence of coarctation of the aorta in some other places.
2. Reduced Flow Theory
Berdasarkang this theory, a defect develops due to hemodynamic disturbances such as decreased blood flow on the side of the coarctation. In normal fetuses, the left ventricle pumps 30% of all ventricular output, but the aortic isthmus receives only 10% led isthmus diameter smaller than the descending aorta. If the flow farther left ventricular pressure, the narrowing isthmus More severe coarctation. This explains the relationship of coarctation with obstruction of the left side of the heart to another.
In addition to these two theories, is now increasing evidence that genetic factors play a role in the development of coarctation and arch hypoplasia. Relationship with Turner syndrome and coarctation of events in the family gave evidence of genetic involvement. Coarctation may occur in 15-36% of patients with Turner syndrome and inherited in an autosomal dominant with incomplete penetration.
There is a significant role and interconnected between genetic factors, abnormal blood flow patterns, and the expansion of abnormal ductal tissue, which led to the breadth of the spectrum of anatomical abnormalities that can be found in patients with koarktasi.

V. Pathophysiology
Koartasio aorta may occur as solitary or jukstaduktal obstruction or tubular hypoplasia of the transverse aortic started on one of the blood vessels of the head or neck and extends into the area of ​​the duct (koartasio preduktal). Often both komponem there. Koartasio formulated that began in the life of a fetus in the presence of a heart defect that causes the flow of blood through the aortic valve is reduced (eg, biskupid aortic valve, VSD).
After birth, the jukstaduktal koartosio separate, ascending aortic blood will menggalir through a narrow segment to reach the descending aorta. though it will result in hypertension and hypertrophy of the left ventrkel. In the first few days of age, patent ductus arterius may contribute to widen the area of ​​aortic obstruction justadukal and provide temporary fixes. In infants it occurs from left to right ductal shunt and they do not cyanosis. Preferably, the koartasio justadukal heavier or when there is hypoplasia of the transverse arch, right ventricular blood through the ductus to memasuk terejeksi descending aorta, as was done during fetal life. Perfusion of the lower body then depends on the bulk of the right ventricle. In these circumstances, can pemoralis palpable pulse, and blood pressure differences may not be helpful in making the diagnosis. But the right-left ductal shunt will manifest as differential cyanosis with upper extremity red (pink) and the lower extremities blue (cyanosis).
Baby - Baby thus may suffer pulmonary hypertension and pulmonary high paskuler prisoners. Signs - a real sign of heart failure. Sometimes - sometimes, segment hypoplastic aortic isthmus can be artetik severe, producing ternganggu aortic arch with left subclavian artery proximal or distal exit disorders. In the past, koartasio accompanied with arch hypoplasia referred to as infantile type, "as he used to. Adult type called koartasio jukstadukal course, that if the light does not usually come until the end of the child. This term has been dinganti with more precise anatomical terms of the former describing the location and keparah defect.
Blood pressure rises in the blood vessel that comes out next proximal koartasio. Blood pressure in pulse pressure below the lower konstrisi. Hypertension is not due to mechanical obstruction alone, but also involves the renal mechanisms. If not operated on in infancy, koartasi aorta usually cause extensive collateral circulation growth, particularly from the branch - branch of the axillary artery in the thoracic and subkapiler can also be enlarged as collateral channels. These blood vessels joining the branch - the inferior epigastric branch of the femoral artery in the arterial blood vessels to form a local bypass koartasio. The blood vessels that helped shape the collateral circulation may be very large and winding - winding in early adulthood.

The hemodynamic effects of aortic coarctation varies and depends on the degree of obstruction, the relationship with other cardiac lesions and compensation mechanisms. In the fetus, there was minimal hemodynamic disturbance because only 10% of the combined ventricular output passes isthmus. However, after birth, the ductus closure causes a variety of disorders, ranging from mild systemic hypertension until congestive heart failure and shock. Aortic obstruction suppress output of the left ventricle, leading to pressure overload and increased end diastolic left ventricular pressure.
Koartasio aorta may occur as solitary or jukstaduktal obstruction or tubular hypoplasia of the transverse aortic started on one of the blood vessels of the head or neck and extends into the area of ​​the duct (koartasio preduktal). Often both komponem there. Koartasio formulated that began in the life of a fetus in the presence of a heart defect that causes the flow of blood through the aortic valve is reduced (eg, biskupid aortic valve, VSD).
After birth, the jukstaduktal koartosio separate, ascending aortic blood will menggalir through a narrow segment to reach the descending aorta. though it will result in hypertension and hypertrophy of the left ventrkel. In the first few days of age, patent ductus arterius may contribute to widen the area of ​​aortic obstruction justadukal and provide temporary fixes. In infants it occurs from left to right ductal shunt and they do not cyanosis. Preferably, the koartasio justadukal heavier or when there is hypoplasia of the transverse arch, right ventricular blood through the ductus to memasuk terejeksi descending aorta, as was done during fetal life. Perfusion of the lower body then depends on the bulk of the right ventricle. In these circumstances, can pemoralis palpable pulse, and blood pressure differences may not be helpful in making the diagnosis. But the right-left ductal shunt will manifest as differential cyanosis with upper extremity red (pink) and the lower extremities blue (cyanosis).
Baby - Baby thus may suffer pulmonary hypertension and pulmonary high paskuler prisoners. Signs - a real sign of heart failure. Sometimes - sometimes, segment hypoplastic aortic isthmus can be artetik severe, producing ternganggu aortic arch with left subclavian artery proximal or distal exit disorders. In the past, koartasio accompanied with arch hypoplasia referred to as infantile type, "as he used to. Adult type called koartasio jukstadukal course, that if the light does not usually come until the end of the child. This term has been dinganti with more precise anatomical terms of the former describing the location and keparah defect.
Blood pressure rises in the blood vessel that comes out next proximal koartasio. Blood pressure in pulse pressure below the lower konstrisi. Hypertension is not due to mechanical obstruction alone, but also involves the renal mechanisms. If not operated on in infancy, koartasi aorta usually cause extensive collateral circulation growth, particularly from the branch - branch of the axillary artery in the thoracic and subkapiler can also be enlarged as collateral channels. These blood vessels joining the branch - the inferior epigastric branch of the femoral artery in the arterial blood vessels to form a local bypass koartasio. The blood vessels that helped shape the collateral circulation may be very large and winding - winding in early adulthood.
Aortic coarctation determine significant afterload of the left ventricle which causes increased cardiac wall stress and ventricular hypertrophy kompensatoar. Cardiac output formed arrived - arrived that occurred following the closure of the ductus arteriosus in neonates with severe coarctation. In babies - babies who experience this, congestive heart failura (CHF) and shock can occur. Rapid constriction of the ductus arteriosus produces severe aortic obstruction arrived - arrived. During ductal constriction, left ventricular afterload increase rapidly which results in an increase in left ventricular pressure (systolic and diastolic). This causes an increase in left atrial pressure can open foramen ovale, causing left-to-right shunt and right atrial dilatation and right ventricle. If the foramen ovale does not open, arterial pressure and pulmonary veins increases, causing dilatation of the right ventricle. Cardiomegaly can be seen from examination of thoracic images and right ventricular hypertrophy can be seen on ECG and echocardiography.
Left ventricular afterload also increases gradually leading to formation of collateral vessels in children - children with severe coarctation. In children - children are usually asymptomatic until symptoms - symptoms of hypertension and other complications arise.
Other congenital heart defects also contribute to the occurrence of coarctation of the aorta, such as ventricular septal defect (VSD), aortic stenosis can increase left ventricular afterload
In neonates, as constriction of the ductus, left ventricular afterload increased rapidly, with a resultant increase in pressure (systolic and diastolic). It also causes an increase in pressure in the left atrium that can open foramen ovale, resulting in left-to-right shunt and right atrial and ventricular dilatation. When the foramen ovale fails to open, the pressure increases venous and pulmonary artery, right ventricular dilatation. Due to severe obstruction of myocardial dysfunction, decreased stroke volume and cardiogenic shock. Compensatory mechanisms that aim to improve cardiac output is activated, including the Frank Starling mechanism, renin angiotensin and sympathetic systems. However, this mechanism may not be effective in the immature neonatal myocardium due to a lack of β-adrenergic receptors and the low compliance of the left ventricle myocardium when compared with adults
Left ventricular afterload may also increase gradually led to children with milder degrees of obstruction have collateral arteries developing partially "cut" aortic obstruction. Compensatory mechanisms are activated, including left ventricular hypertrophy. These patients can be asymptomatic until the detected presence of hypertension or other complications. collateral blood flow is divided into two, namely the anterior and posterior collateral. Collateralization anterior starting from the subclavian artery to the internal mammary arteries leading to the epigastric artery to supply blood to the lower body or the lower extremities. Collateralization of the posterior intercostal arteries leading to supply blood to the abdomen.
Some vascular abnormalities develop in patients with coarctation of the aorta at the proximal and distal vascular obstruction. Neonates and children with coarctation decreased distensibility and increased reactivity to norepinephrine in the proximal side koarktasi. Plasma renin activity increased significantly and baroreceptor reflex is set to higher blood pressure. These abnormalities may persist long after the surgical repair and contribute to the onset of systemic hypertension and premature death from coronary and cerebrovascular disorders.VI. diagnosis
The diagnosis of coarctation of the aorta is done on the basis of history, physical examination and diagnostic investigations.
1. History
Patients with coarctation of the aorta in the isthmus narrowing the type of the early weeks of life will be reluctant to drink, tachypnea, lethargy and grow progressively toward congestive heart failure. Discrete type of coarctation of the aorta in children usually does not cause symptoms due to the formation of collateral vessels. With the growth of the child, coarctation of the aorta becomes relatively more narrow so that the child complained weak, chest pain, headaches and intermittent claudication.
New symptoms may arise in adolescence, but it can also arise when the baby, depending on the severity of the resistance to blood flow.
Highly dependent on the degree of aortic coarctation and concomitant cardiovascular abnormalities. In untreated patients, 60% without concomitant severe aortic coarctation and 90% were accompanied by concomitant cardiac abnormalities, will die in the year - the first year of life. Although the average age expectations - mean aortic coarctation was 35 years old, there are surviving into old age.
Patients who survive to adulthood without treatment usually had abnormal post-ductal aortic coarctation is mild, usually asymptomatic for a long time. Is not often found hypertension, therefore, a new diagnosis is made after adulthood.
In severe stenosis, narrowed ascending aorta, the blood circulation is reduced and consequently the blood pressure in the lower limbs (legs) is very low compared with the pressure in the upper limbs. (5)
Problems that may arise later may be and probably is the cause of death as heart failure left (28%), intracranial hemorrhage (12%), bacterial endocarditis (18%), aortic rupture or dissection (21%), and coronary heart disease more the beginning.
Adult patients with hypertension and can usually be found on the noisy although frequently asymptomatic adults. Typical symptoms are a result of high blood pressure at the top can be a headache, nose bleeding, float, tinnitus, cold limbs, abdominal angina, leg fatigue on exercise even intracranial hemorrhage. Claudication of the legs can describe abdominal aortic coarctation.
Symptoms include:
1. dizziness
2. fainting
3. leg cramps during activity
4. High blood pressure is localized (only in the upper body)
5. foot or leg felt cold
6. shorthanded
7. throbbing headache
8. nosebleed
9. The leg pain during activity.
At the age of a few days to 2 weeks, after ateriosus ductus closes, some babies have heart failure. Severe respiratory distress occurs, the baby looked very pale and blood tests showed increased acid in the blood (metabolic acidosis).

2. Physical Examination
On physical examination can ditemukaan higher systolic blood pressure in the arms to the legs, but the same diastolic pressure, therefore the pressure pulse in the arm would be great. Femoral arterial pulsation is weak and delayed compared to the radial artery. Systolic thrill may be palpable in the suprasternal region. When accompanied by a bicuspid aortic, can rough systolic murmur audible ejection type along the left sternal and back, especially in the area of ​​the coarctation. The existence of collateral may lead to continuous noise. Clinical manifestations of patients with coarctation of the aorta varies but in general can be classified into early onset in neonates and infants, usually in the form of congestive heart failure and late onset in children and adolescents, more common form of hypertension.
1. In infants heart failure can occur
2. Generally no complaints, usually found incidentally
3. Palpation: touch the radial artery and femoral secra simultaneously
· In the radial artery is stronger
· At the femoral artery palpable weaker
4. Auscultation:
· There was bisng koartasio on the back of a noisy obtruksi
· If a severely narrowed aortic lumen continuous murmur in the aorta.

a. Early onset
· Neonates may experience tachypnea, tachycardia, and more effort to breathe may even develop into shock. The key to mediagnosis that measures the difference in blood pressure between the superior and inferior extremities as well as the lack or loss of pulsation on palpation of the lower extremities. However, infants in a state of severe heart failure, all of pulsation would be perceived decline. In patients where the right subclavian artery is located in the aorta distal to the obstruction, the difference in blood pressure may not be found in spite of the lower extremity pulses will be lower than the pulsation karotisSianosis differential (kemerehana color on the upper extremities and cyanosis of the lower extremities) can occur when the right-to-left patent ductus arteriosus shunt blood to the lower body. Although usually not too visible on inspection, differential cyanosis can be found by examination and pulse oximetry preduktal postduktal aorta and careful inspection. However, the presence of lesions of the left-to-right shunt is large (eg, VSD), pulmonary saturation can approach the saturation of the aorta, causing saturation oksimetrik minimal differentiation.
· Noise associated with coarctation of the aorta are usually non-specific, such as noisy systolic left infraclavicular area and under the left scapula. Additional noise due to the presence of other abnormalities such as VSD or aortic valve stenosis can also be detected. ejection
Sign in
  significantly indicating a bicuspid aortic valve and gallop rhythm indicating ventricular dysfunction.
In infants under 6 months with koarkasio aorta and heart failure diagnosis can be confused with sepsis or lung disease.

b. Slow onset
· Older infants and children, may be referred for evaluation of hypertension or heart murmur discovered. Hypertension in infants and children can cause agitation. It is important to compare the blood pressure in all four extremities. Sometimes the pressure on the lower left arm than the right arm, when a. left subclavian involved in koarktasi. The same thing happens when the arteries are in the distal right subcalvia koarktasi that can cause dropped or loss of pulsation in the right brachial artery. Simultaneous palpation carefully pulsation of all four limbs can confirm suspected koarktasi
· In older children, adolescents and adults, coarctation of the aorta are best diagnosed clinically by palpation simultaneous femoral and brachial arterial pulsation. Blood pressure in both arms and legs to be determined, the difference in pressure above 20 mmHg may be evidence of coarctation of the aorta.
· Noise on the left infraclavicular area and under the left scapula can be noisy systolic, but continuous noise can also arise as a result of multiple collateral vessels or severe coarctation.
· Can be found abnormalities in the blood vessels of the retina and prominent pulsation in the suprasternal. Thrill can be felt in the suprasternal basin or the precordial region due to aortic valve stenosis. In rare cases of coarctation of the abdominal, abdominal bruit may be heard.

3. Examination Support
a. Electrocardiographic (ECG)
ECGs in neonates or infants with coarctation of the aorta from the beginning will show a picture right ventricular hypertrophy. This is achieved because the intrauterine life of the ventricle is the dominant role of the right ventricle. In early onset (infantile type), mainly due to coarctation aort arch hypoplasia with coarctation of the left so this will increase the load of the right ventricle. EKG in children after the age of 1 year can be normal and may be accompanied by a picture of left ventricular hypertrophy. Children who had no symptoms of coarctation of the aorta showed left ventricular hypertrophy which is comparable to high blood pressure upper body. If there are other disorders, usually accompanied by other abnormalities.
ECGs in neonates aged 20 days with severe aortic coarctation which manifests as cardiogenic shock. There are tall R in V1 and deep S in lead I indicating the presence of ventricular hypertrophy kanan.8

b. Chest Roentgenogram
Two markers of coarctation of the aorta is a lesion on the lower edge of the rib (rib notching) and picture number 3 (figure 3 sign) in the proximal descending aorta. Rib Notching, which occurs due to compression of the ribs posterior collateral arteries are dilated, tortuous and pulsating. Angled ribs usually occur on the lower edge of the third to eighth ribs. It is widely seen in adults (75%) whereas in children are rare. Picture number 3 shows koarktasionya. The curve of the upper part of figure 3 shows dilatation of the left subclavian artery or the proximal part of the aortic coarctation, while the lower curve illustrates the post-dilatation of coarctation of the distal part of aortic stenosis. In patients with early aortic coarctation, there are descriptions of cardiomegaly, pulmonary edema, and other signs of congestive heart failure. In patients with a slower onset, would seem cardiomegaly, indented arch coarctation area and rib notching is rarely seen in children younger than 10 know.
Examination conducted by the esophagus is filled with barium. Projection important is postero - anterior (PA). Esophagus at the top of the arch is depressed by wide. In this section of the esophagus pushed to the right. In a more prominent esophageal stenosis. Under the pressure of esophageal stenosis right again by poststenostik dilatation of the aorta. Thus, it has depressed the esophagus as a form of letter E
Heart enlarged to the left with the apex downward, due to hypertrophy of the left ventricle. Waist normal heart. Pulmonary vasculature appeared normal
Looks rib knotching on ribs - ribs, especially ribs - rib top. Bottom edge of the ribs - rib is shaped like a sawtooth. (5)
Intercostal arteries are dilated and are collateral to grow at the bottom of the bleeding can only be seen on arteriography.

c. Echocardiography
Describe the anatomy of intracardiac echocardiography along with other anomalies. In infants, the aortic isthmus and proximal descending aorta can be displayed by using the parasternal view, parasagittal or suprasternal view. Aortic coarctation is almost always occurs distal to the left subclavian artery branching. The sign is a typical curve of the aortic side of the posterior and lateral distal segment coarctation is usually dilated. Suprasternal view of the aortic arch can be seen for the evaluation of the transverse aortic arch, isthmus and assess the severity of coarctation. Transverse aortic arch should be demonstrated and measured using suprasternal view. The diameter of less than 4 mm is associated with differences settled although pure coarctation was repaired. The transducer used was a small transducer that can be inserted deeper without feeling uncomfortable.
Fetal echocardiography showed hypoplasia of the transverse aortic arch (white arrow) and coarctation of the aorta (yellow arrow). AAO: ascending aorta; DAO: the descending aorta.

d. Magnetic Resonance Imaging (MRI)
MRI can depict the exact location and degree of narrowing, the anatomy of the aortic arch and the presence of collateral flow. Measurements using cardiac MRI correlates well with cardiac catheterization gradients and determine whether the patient requiring transcatheter or surgical treatment.
e. Cardiac catheterization
Non-invasive diagnostic tools such as echocardiography and cardiac MRI has many shifts as supporting the diagnosis of cardiac catheterization. The main use today is cardiac catheterization as transcatheter therapy. Sometimes, more information is needed in neonates and infants when no other lesions related or arch hypoplasia, as an indication of surgical therapy. Therapy is indicated when the gradient aortic coarctation obstruction cardiac catheterization subs reach ≥20-30 mmHg.
f. computed Tomography
Cardiac examination with Computed Tomography easier than with echocardiography or angiography. CT scans can show all segments of the aorta and heart valve abnormalities so that there will be clearly visible. In addition, the characteristics of cardiac masses can be visualized well as the ability of CT scan to assess the characteristics of the network. In addition, this modality can measure the dimensions and position of the aorta and arteries prestenosis and poststenosis - collateral arteries.
CT Scan in coarctation of the aorta showed a narrowing of the aorta in the presence of arterial dilation - collateral artery and left internal mammary arteries.

VII. Management of
Surgery, with the goal of eliminating stenosis and strain in the wall of the aorta, as well as maintaining the patency of the aorta. Repair immediately after diagnosis at a young age have a lower risk than older age. After 30-40 years of high intraoperative mortality due to the degeneration of the aortic wall.
Interventions such as angioplasty with or without stent implantation is a good alternative treatment in children - children and adults.
In rekoarktasio conditions, there is a consensus that more choice to act either angioplasty with or without stent.
After birth, after the diagnosis of coarctation of the aorta has been established, neonates followed up carefully. In neonates with partial obstruction, regular assessment of femoral pulse and a fourth limb to be done to clear if the coarctation is really there. Once the obstruction has been ruled out by clinical examination and there has been a closure of the ductus, the neonate may be sent back to be re-examined at the age of 6 months where the slow onset of aortic coarctation may arise
Infusion of PGE1 must be done quickly in neonates with heart failure or shock. These patients often require mechanical ventilation, correction of acidosis and the use of inotropic agents. PGE1 dilates and minimizing duct obstruction in 80% of neonates up to 28 days. Lack of response to PGE1 may be caused secondary to have a complete closing of the duct or irreversible functional closure due to lack of PGE1 receptor number and sensitivity. PGE1 effective dose varied between 0.002-0.1 mg / kg / min, and it remains unclear whether the magnitude of ductal dilatation depends on the dose. Recent observations show that above the age of 28 days and weighing less than 4 kg PGE1 associated with failure. Side effects that may occur in the form of a wide variety of types of congestive heart failure. Side effects occur in 12-16% of infants and is associated with low birth weight (LBW) <2 kg, the use of long-term (> 48 days), and arterial infusion of high doses (> 0.1μg / kg / min). The most frequent effects are respiratory depression (11%), cutaneous vasodilation (7%) rhythm disorders (7%), cramps (7%), and hypothermia (4.5%). The act operative, with the goal of eliminating stenosis and strain in the wall of the aorta, and to maintain the patency of the aorta. Repair immediately after diagnosis at a young age have a lower risk than older age. After 30-40 years of high intraoperative mortality due to the degeneration of the aortic wall.
Interventions such as angioplasty with or without stent implantation is a good alternative treatment in children - children and adults.
In rekoarktasio conditions, there is a consensus that more choice to act either angioplasty with or without stent.

When the patient was hemodynamically stable, definitive action needs to be handling operatively. or transcatheter.
1. Measures Operative
Operative action is the very first modality, since more than 50 years ago. Surgical options include resection of the narrowed segment with end-to-end anastomosis, interposition prosthesis, the flap with the left subclavian artery (left subclavian artery / LSCA) or synthetic materials. Among the different techniques, end-to-end anastomosis is the most commonly used, especially in neonates and have a long survival rate. In this technique the resection of the narrowed aortic segment and then re-direct anastomosis. Can also be done using a prosthetic interposition.
LSCA flap aortoplasty produce collateral flow to the left upper extremity, resulting in growth suppression arm, or a decrease in blood flow from the artery vertebasilar. When used synthetic materials, there is a risk of aneurysm formation at the repair side.
The operation is usually performed through left lateral thoracotomy without cardiopulmonary bypass. However, median sternotomy generate Tampakan better in situations where there are other cardiac lesions, such as VSD or extensive arch reconstruction. During surgery, the proximal transverse arch in the clamp, resulting in uncontrolled ischemia and carotid artery and vertebral artery distal. Blood supply to the left hemisphere is maintained by collateral vessels were clamped on the proximal side. Although in general it is well tolerated, Azaki et al shows the oxygen supply to the left hemisphere of the brain failed during clamping arch.
No surgical technique that can be applied to all patients. Although resection techniques can be used in almost all patients, each case must be evaluated on an individual basis in order to choose the most optimal method. Each patient requires long-term follow-up because of frequent postoperative complications and the number is increasing over time.
2. transcatheter
Interventional therapy of coarctation of the aorta has been accepted as an alternative to surgery. This therapy has a good level of security, and has a success rate that is not much different from the operative action especially in the case of re-koarktasi.
Lock et al was first successfully performed balloon angioplasty in aortic coarctation segment narrowing in humans. Since then, balloon angioplasty became a standard method in primary and recurrent coarctation of the aorta. Balloon Angiplasty produce injury to the intima and media thicken the narrowed aortic segment, dilate the obstruction. However, this can be extended to the side of the healthy aorta causing rupture or aneurysm formation. Balloon Angioplasty is generally not performed until the age of 6-12 months in primary koartasio because rekoarktasi high risk of occurrence (57%), aneurysm formation (17%) and damage to the femoral artery (39%).

Stent placement
The balloon is equipped with stents have been used successfully since the early '90s to open an obstruction in experimental animals and patients with coarctation. Stents support the integrity of the blood vessel wall during balloon dilatation and result in a more controlled wound. This minimizes expansion and continuation wound dissection or aneurysm formation. Aneurysms occur in 4-7% after balloon angioplasty or stenting for coarctation. However, the results of long-term stenting disappointing. Stent implant at a young age is limited because of its small size and lack accommodate somatic growth. Therefore, stenting is the primary therapy of coarctation there teens and adults. 2,8,12

VIII.Komplikasi
Patients were operated on before the age of one year have a higher risk of experiencing a re-coarctation than older patients. Re-koarktasi insufficiency may occur as a result of the growth of the anastomosis, the presence of the rest of the ductus arteriosus in severe coarctation prone to retraction, or technical errors such insufficiency resection or synthetic flap size estimation error
· Hypertension is a frequent complication in aortic coarctation. It is linked to the age of the patient during surgery is the first time that an older age have a higher probability of experiencing persistent hypertension even in patients who did not undergo re-koarktasi. All patients with hypertension require antihypertensive therapy, but even with this therapy, there is still an increased risk of coronary heart disease, brain hemorrhage, aneurysm of the aorta or the aortic dissection.
· Stenting can lead to complications including a fatal rupture of blood vessels, forming pseudoaneurisma, stent dislocation, and the formation of thromboembolism.

IX. prognosis
Aortic coarctation is a disease with a poor prognosis. Fixing obstruction, control of hypertension, recurrent obstruction supervise, and monitor other related disorders is an action that needs to be done. Patients with persistent hypertension, untreated obstruction, or other complications have the appropriate degree of disease prognosis. The mortality rate associated with rekoarktasio, in the area of coarctation aneurysm, congestive heart failure, bacterial endocarditis, and hypertension.
Despite the overall improvement in operations and transcatheter is quite satisfactory, but the long-term morbidity and mortality is still quite high. Cohen et al in the Mayo clinic showed 571 patients operated between 1946 to 1981. In the middle of the follow-up of 20 years, 11% of patients required subsequent surgery (3% because rekoarktasi), 25% progress to hypertension and 15% had cardiac-related deaths. Survival analysis showed 91% of patients were still alive in 10 years, and decreased to 72% within 30 years after repair. Death occurs at an average age of 38 years, and correlated with repair older age (> 9 years) and postoperative hypertension. 44% of all pending death secondary to coronary artery disease or cerebrovascular injury, indicating accelerated vascular disease. Similar results were reported by Toro-Salazar et al in 274 patients followed up over 50 years. The risk of death after repair koarktasi estimated to be approximately 3.8 / 1000 patients a year. Important risk factor for death was persistent hypertension, associated circuitry repair older age and residual obstruction. Hypertension occurs in 7-28% of patients treated in infancy, compared with 38% who were treated at the age of 4 years. The incidence of infective endocarditis after repair koarktasi was 1.2 / 1000 patients per year, causing antibiotic prophylaxis should continue to be provided.