A. DEFINITIONS
Ebstein Anomaly (AE) is a congenital heart defect characterized by the migration of the location of the leaf and the leaf posterior septal tricuspid valve (KT) downward to the right ventricle and right heart split into two rooms, the atrium space with room air-join atrialisasi right ventricle, located at the proximal KT and right ventricular functional space located in the distal part of the small KT. Clinical symptoms vary depending on the location and severity of displacement KT right ventricular outflow obstruction. Cyanosis can manifest at birth, in the neonatal period or in infancy youth. Some AE manifest symptomatic neonates with tachypnea, cyanosis, and difficulty drinking. Chest X-ray showed cardiomegaly and a box or balloon pulmonary vascularity is reduced. Diagnosis can be koni rm with ekokardiograi. Cyanotic neonates with symptoms sometimes do not need to be treated, unless great cyanosis due to severe hypoxia, because usually improves with reduced pulmonary vascular resistance and heart failure kongesif usually improves with anti-congestive medication. Babies severe hypoxia can be given intravenous prostaglandin to open ductus arteriosus and pulmonary blood vascularization and improve saturation of arterial blood. If there are dependencies prostaglandins, quite overcome by the manufacture or the closure of the shunt aortopulmonal KT, septektomi fibrillation, and shunt removal aortopulmonal. Prognosis is poor when severe AE and infants born with symptoms of cyanosis and other symptoms are not severe and resolved with conservative measures or surgery, the baby can usually survive to adulthood when AE light.
B. ANATOMY PHYSIOLOGY
Ebstain anomaly has characteristics of any interruption to the right side of heart, this disorder is formed when Triskuspid valve separates the right atrium and right ventricle is abnormally formed, in which the tricuspid valve is formed too far down into the ventricles. As a result of this disorder, the right ventricle becomes smaller than normal and also weaker than it should be. It also found the presence of an abnormal connection between the right atrium and left atrium (Atrial Septal Defect / ASD (2)). Valve Triskuspid the EA one or two parts of the valve leaflets "Moored" to the heart wall and causing it to not move properly, so that the blood can be returned to the atrium in systole (regurgitation Triskuspid
C. Epidemiology
Ebstein's anomaly is a very rare case, allegedly 0.3% sampai1% of all cases of congenital heart disease. In the United States the incidence obtained Ebstein anomaly of about 1: 210,000 live births. The disorder is found 1000 times 1dari autopsies of patients with suspected congenital heart disease. Angkakejadian women comparable to men, usually sporadic, but has been reported in a kinship. In general, 40% to 60% of patients died in the early year of life.
D. CLASSIFICATION
Ebstein Anomaly can be grouped into two categories based on the level of the disease is of being and Weight
Medium level: the tricuspid valve disorders and can be addressed immediately so as not to experience severe complications.
More severe level; when the tricuspid valve opens wide (sail shape) and can not be shut down or re-keposisinya when diastole ends or when the right ventricle begins to contract, Things like this can impede blood flow to the lungs which will pass through the valve Pulmonary valve pulmunal.dengan thus deprived or does not get the blood which in turn will be pulmunal atresia. So the lungs that causes lack of blood throughout the body is deprived of oxygen carried in the blood and the next phase will be had cyanosis and respiratory distress as well as extensive experience complications.
E. Etiology
The cause of congenital heart disease can not be known with certainty, but there are several factors suspected to have an influence on the increase in the incidence of congenital heart disease. In most cases, the cause of heart disease is not known for certain bawaa. allegedly due to endogenous and exogenous factors.
1) Various types of genetic diseases chromosomal abnormalities
2) the mother's pregnancy history, had previously participated in an oral or injectable birth control program, taking medication without a prescription (benzodiazepines, thalidmide, aminopterin, herbs)
3) Perinatal factors: the mother has an infection: rubella, alcoholism, maternal age over 40 years, the mother has diabetes that requires insulin diseases, maternal herbal drink.
4) Genetic factors: children born before suffering from congenital heart disease. Father and mother mendertia congenital heart disease, chromosomal abnormalities such as sidrom down.
5) Most cases of congenital heart defects have no known cause.
F. RISK FACTORS
In Ebstein's anomaly diseases have risk factors such as patients who have had infectious diseases, diabetes mellitus who are already insulin drug dependence, pregnant women who consume obattan antidepressant drugs without a prescription. Clients mempuyai drinking herbs, alcohol beverages.
G. Pathophysiology
In AE, the location of the tricuspid valve moves downward, the lower of the actual position and into the ventricular cavity. This defect may be due to the failure of tar-separation process tricuspid valve from the right ventricle myocardium. Becomes very large right atrium, right ventricle functional small, and usually occurs from mild to severe tricuspid regurgitation. Effective cardiac output from the right heart to be reduced in addition to the size of the right ventricular functional so it does not function normally and trikuspida-lis regurgitation causes blood to flow back into the right atrium through the tricuspid valve, as well as right ventricular outflow obstruction due to anterior cusps can be very large to shaped screen (sail-shape). The anatomical abnormalities causing blood flow resistance through the right ventricle so that most of the blood will flow from the right atrium to the left atrium through the foramen ovale, or persistent atrial septal defect. There are times when right ventricular function is reduced so that is not strong enough to open the valve trikuspidal during right ventricular systolic, may cause pulmonary atresia-finalists functional even in some infants become anatomic pulmonary atresia. During the cardiac cycle, namely during atrial systolic, systemic venous blood returns to the heart in addition to entering into the right atrium into the chamber also atrialisasi right ventricle and only a little blood goes into the right ventricle functional; whereas during ventricular systole, contraction of the right ventricle function in harmony with section atrialisasi right ventricle causing a lot of blood back into the right atrium for tricuspid regugitasi and only a little blood to flow into the lungs. Space atrialisasi although anatomically right ventricle is part of the right atrium but follow along with the contraction and relaxation of the right ventricle. The lack of harmony contraction causes stagnation of blood in the right atrium. The burden of pressure and volume load in the atrium space ka-nan overcome by right to left shunt through the foramen ovale or atrial septal defect persistent causing symptoms of cyanosis.
H. Clinical manifestations
The main problem of the EA is the tricuspid valve located not in the right place. Not only misplaced but also there is a leak because part of the tricuspid valve is not moving as it should. Leaky valve can cause backflow throughout the body (systemic) causes swelling in the legs and abdomen (ascites), liver enlargement (hepatomegaly) and difficulty breathing (dyspnea). If blood flows backward through the ASD will be there mixing of oxygen-poor blood (Unoxygenated) and oxygen-rich (Oxygenated) in the left atrium, as a result of this process there will be bluish fingers and lips, which we refer to as cyanosis. Hole between the left and right atrium can be track the passage of a stroke or blood clot that cardiac tissue damage may occur. Sometimes patients with EA may experience heart rhythm abnormalities (arrhythmias) that complain of dizziness, feeling faint even drift can occur, in addition to the feeling of palpitations also often complain about. Rhythm disturbances that can appear in the EA is Wolff Parkinson White Syndrome (WPW) and Supraventricular Tachycardia (SVT). Space atrialisasi although anatomically right ventricle is part of the right atrium but follow along with the contraction and relaxation of the right ventricle. The lack of harmony contraction causes stagnation of blood in the right atrium. Pressure load and load volume in the right atrium space solved by right to left shunt through the foramen ovale or atrial septal defect persistent causing symptoms of cyanosis. Clinical symptoms may manifest after birth (50%) or during the neonatal period, including cyanosis, tachypnea, and drinking difficulties. Cyanosis due to reduced oxygenation caused by right-to-left shunt through the foramen ovale, or persistent atrial septal defect. Most babies are born with symptoms of mild cyanosis and some will asymptomatic manifest upon reaching the age of children, adolescents or young adults with symptoms of fatigue or palpitations due to cardiac dysrhythmias. But if the weight of the AE babies born with symptoms of severe cyanosis, cardiomegaly massive and noisy continuous systolic and death can occur due to congestive heart failure and hypoxemia; although spontaneous improvement may occur in some babies when pulmonary vascular resistance can be lowered and improved the ability of the right ventricle to fill the pulmonary circulation. Most babies survive when the ductus arteriosus remains open resulting in improved pulmonary vascularization. The results of the examination contents vary from mild to severe. Babies born with cyanosis initially mild, but with age can be more severe cyanosis with finger-clubbing, distended neck veins, and pulsation in the neck because presistolik full contraction of the right atrium. There may be a mild deformity of the left side of the chest and a palpable thrill in some cases at the apex of the heart or the left border of the sternum due to tricuspid regurgitation. The liver is usually enlarged, but no pulsation presistolik. Cool clammy extremities accompanied by a small pulse due to poor cardiac output. On auscultation of the heart sounds rupture width, two normal heart sounds, heart sounds sometimes heard III and IV, diastolic and noisy noisy holosystolic at the lower left stenum limit. When there is right heart failure will appear urging increased central venous, tachycardia, tachypnea, hepatomegaly, leg edema, and ascites.
I. DIAGNOSTIC EXAMINATION
1. In the case of severe cyanosis and heart failure can occur in the first days of life, the less severe cases shortness of breath, fatigue, cyanosis on exertion is often reported.
2.Auskultasi: There is a second heart sounds wide and separated (Widely Split s2), Pansystolic murmur (PSM) in the lower left sternal Limit / Lower Left Sternal Border (LLSB)
3.ECG: RBBB, RAH, WPW syndrome, SVT and AV block first degree
4.Rontgen thorax: Cardiomegaly with a predominance of right heart enlargement and reduction in bronkovaskular markings due to reduced blood flow to the pulmonary.
5. Echocardiography: There Apical displacement of the tricuspid valve, TR, RV Cavity small, large and ASD RA Cavity
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6. Cyanosis increased when pulmonary vascular resistance decreased
7. CHF, brain abscesses, endocarditis Infective
Cardiomegaly on chest x-ray looks berben-tuk box (box-shaped cardiomegaly) or balloon (balloon-shaped cardiomegaly) pembesa-ran due to the right atrium, the left side of the heart to the posterior terdo-rong, a shadow of the pulmonary and aortic smaller upstream, and reduced pulmonary vascularity . (Figure 2). At l uoroskopi look weak heartbeat, enlarged heart shaped globu-ler (globular-shape) because the right atrium-Ngat sa big, vascular stalk at the base of the heart narrows, and decreased pulmonary vascularity. Overall cardiac shadow and aktivi-bag resembles a picture of a massive pericardial effusion or congestive heart failure with pulmonary stenosis. Radiological picture above is proi l severe AE cases with symptoms of complete (full-blown cases); some ka-sus mild AE only provide clinical symptoms and mild heart enlargement. Examination ekokardiograi me rupakan standard criteria for the diagnosis of AE. In M-mode ekokardiogai sep-tum movements seem paradoxical, right ventricular dilatation, tricuspid valve closes later than 65 ms after the closing of the mitral valve and tricuspid regurgitation. In ekokar-diograi two-dimensional, visible displacement of the septal leaf leaf apical KT more than 8 mm / m2 (which is a sign spesii k AE), leaves an incompetent KT failed to join the normal KT leaf and its free end will enter into the right atrium when the systolic, morphological abnormalities and early perlekat septum septum on leaves and leaf tri-kuspidalis anterior, dilated right atrium and right ventricle atrialisasi, small functional right ventricle and right ventricular contraction is reduced. (Figure 3) In ekokardiograi
doppler
looks mild to severe tricuspid regurgitation and right-to-left shunt through a persistent forame ovale or atrial septal defect. In general, the picture shows the displacement ekokardiograi KT leaf layout, right atrial dilatation, tirkuspidalis regurgitation, and right ventricular outflow obstruction, and in severe cases the pulmonary valve does not move and pulmonary blood flow appears fallow-sal of patent ductus arteriosus. Celenmayer et al introduced ekokardiograi index (IE) AE has prognostic value in neonates and children.
Space + space atrialisasi right atrium right ventricle
IE = -----------------------
Right ventricular chamber of the left ventricle + atrium + left
IE high value means poor prognosis and a high risk of infant mortality. When the functional volume of the right ventricle <35% of the total volume of the right ventricle of normal or ra-sio atrialisasi chamber right ventricle: right ventricular functional space> 1.0 or regurgi-tation of severe tricuspid or pulmonary atresia atrial septal defect functional or ≥4 mm mean a poor prognosis . Cardiac catheterization showed atrial cardiac kaKateterisasi shows ka-nan large atrium with an average pressure of the right atrium rising, KT abnormal interatrial right to left shunt (25%), pulse pressure may be normal or dominant V wave, low systemic cardiac output, flow reduced pulmonary blood under systemic pressure in the case of the right-to-left shunt, and easy entry and curved katater da-lam a large atrium space.
J. MANAGEMENT
The management of AE depends on the degree of cardiac dysfunction. In neonates with mild cyanosis in the first days of birth, may be given oxygen to reduce pulmonary vascular resistance and drug ACE blockers, diuretics, and digoxin when there are signs of congestive heart failure, antibiotic prophylaxis to prevent bacterial endocarditis, an anti-arrhythmia drugs when no arrhythmias, treatment with radiofrequency ablation option if there is supraventricular and giving prostglandin takikadia to open ductus arteriosus so pembe-limb action may be delayed until adolescence or young adulthood. In neonates with cyanosis and severe clinical symptoms, it is recommended actions differentiator-han to keep foramen ovale and the ductus arteriosus remains open while when neonates with severe hypoxic-densi Depen prostaglandins, just done aortopulmonalis shunt or surgically closing KT, septektomi fibrillation, and pembu -atan aortopulmonalis shunt, which aims to create functional tricuspid atresia, se-added improved with the creation of the Glenn shunt and Fontan technique. In cases of severe tricuspid regurgitation, abnormal KT repairs in conjunction with the closure of ASD and in some cases are carried out with a Glenn shunt technique of anastomosis between the superior vena cava and pulmonary artery-finalists. This will reduce the volume of blood pumped by the right ventricle not functioning. Improvements biventrikuler AE neonatal severe pain can be done very well to conventional treatments se-ill neonate should be replaced with early surgery. Surgery consists of the reconstruction of the expansion of KT monokuspid competent right ventricle, ASD closure subtotal, atrioplasti Penor forecasting right atrium space, and repair of heart defects that exist.
K. PROGNOSI
Definition of a likelihood estimates of travel, duration and effect of a disease based on general knowledge and the pathogenesis of disease and the presence of risk factors. AE The prognosis varies depending on the severity of the disease and the available treatment options are usually bad prognosis, babies born with cyanosis and other symptoms are severe. AE poor prognosis forecast clicking when the baby naturally severe cyanosis or attacks of paroxysmal tachycardia, male gender, manifest at a young age, CTI> 0.65 (cardiomegaly), the ratio of leaf attachment of the septum (the ratio of the distance between the AV ring and the distal attachment of the leaf septum to septum leaf length)> 0.45, and an increase in the ratio of the volume of the right atrium + right ventricle atrialisasi than de-ngan functional right ventricular volume - level 1 <0.5 to 4 degrees> 1,5.8 life expectancy estimated mean AE case average of 30 years; 1/4 of cases died within the first 10 years of life. AE classic clinical cases usually die during the second decade of life. no reported cases of the meninges AE-gal in the decade to eight even once until the age of 60-85 years. Early death is usually associated with cases of AE-rat be caused by congestive heart failure, cardiac arrhythmias, central nervous system disease, and myocardial infarction. Surgical closure of ASD then cyanosis and other symptoms reduced, the manufacture of the superior vena cava anastomosis de-ngan right pulmonary artery repair right heart volume load, and penggan-tian KT can improve the function of the right ventricle so that the prognosis improves. AE high neonatal mortality rate; although there is no defect in the hemo-dynamic signii other, only 70% of infants live to the age of 2 years and 50% die before reaching the age of 13 years; only 5 of the total cases reported AEs live up to the 23-25 decade Survival (Kaplan-Meier) was 61% at 1 week of age, 48% at 1 month of age, and 36% at 1 and 5 years. Ekokardiograi volume ratio (right atrium + right ventricle atrialisasi): volume (functional right ventricle + left atrium + left ventricle)> 1.0 indicates 100% mortality prediction.
L. DIAGNOSIS
1. The anatomic pulmonary stenosis. Clinical symptoms of cyanosis and distinguishable darifoto thorax which showed cardiomegaly shaped box, a ball, or balloon, a small right ventricle and the pulmonary vascularity is reduced in AE right sedangkanventrikel normal and reduced pulmonary vascularity pulmonalanatomik stenosis.
2. Pericardial effusion: Photo AE piston similar to the effusion pericardial effusion but perikardtidak found cyanosis, noisy, and gallop rhythm.
3. Total anomalous pulmonary venous drainage: clinical symptoms similar to AE but the chest x-ray looks transposition total pulmonary veins, right ventricular hypertrophy and increased pulmonary vascularity.
4. Tetralogy of Fallot and L-transposition of the great vessels. Clinical symptoms of cyanosis, and can be distinguished from the results of radiological examinations.
5. Isolated severe tricuspid regurgitation.
6. Differentiated from a congenital heart defect characterized by a hypoplastic right ventricle segments with normal KT.
M. Complications
1) Endocarditis
2) pulmonary vascular obstruction
3) CHF
4) Hepatomegaly (rare in premature infants)
5) necrotizing enterocolitis
6) Pulmonary Disorders that occur simultaneously (eg, respiratory distress syndrome or pulmonary dysplasia bronkko) gastrointestinal bleeding (GI), a decrease in platelet count
7) Hyperkalemia (decreased urine output).
8) Arrhythmias
9) Failure to thrive
Thus both circulation (pulmonary and systemic) apart. Patients can only live if there is a relationship between the two circulations. In infants patent foramen ovale and the ductus arteriosus is still open.
Clinical symptoms seen in infants after birth is cyanosis. At first light, and cyanosis increased on subsequent days. I sound normal heart sounds and heart sounds terdengan II single and keras.Bising usually not heard, unless there are other abnormalities. CXR shows increased vascularity of the lung (plethora) with a heart like an egg (eggshaped).
N. PREVENTION
Prevention is important in order to reduce the incidence of congenital heart disease, Ebstein's anomaly In the disease to be prevented in the disease are:
1) habits for pregnant women trimester ie drinking two or three drugs labeled made hardware purchase without a prescription medicine that is an example of one of the sala of the type benzodiazepine antidepressant drugs which work by pressing the central nervous system
2) The habit of drinking alcohol
3) Animals which is an agent for the rubella virus can be avoided from as infants or pregnant women.
4) maternal age over 40 years to the risk of congenital heart disease.
5) Avoid beverages such as herbal medicine and others.
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