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Referat

First Recognizition of AcyanoticCongenital Heart Disease

DIETER ALYONA, Sked0961050054

Background

Various congenital abnormalities (congenital anomaly).Congenital Heart Disease (CHD) is the most common abnormality.

In the United States, the prevalence of congenital heart disease around 8-10 of 1,000 live births, with a third of them manifest in critical condition in the first year of life and 50% of the gravity of the first month of life ends with death.

In Indonesia, with a population of 200 million inhabitants and a live birth rate of 2%, it is estimated there are about 30,000 patients with CHD.

Etiology and Epidemiology

Acyanotic congenital heart disease is a disease with structural abnormalities and cardiac or circulatory function due to interruption or failure of development of cardiac structures in the early phase of fetal development, without symptoms of cyanosis.

Acyanotic congenital heart disease are 75% of the prevalence of congenital heart defects. Consists of the effects of structural heart defects, with or without gout from left to right (Left to Right Shunt = ltrs).

A-CHD

- Increased pulmonary blood flow

Ventricular Septal Defect (VSD)

Atrial Septal Defect (ASD)

Patent Ductus Arteriosus (PDA)

Endocardial cushion defects (ECD)

- Normal pulmonary blood flow

Pulmonary Stenosis (PS)

Aortic Stenosis (AS)

Coarctatio Aorta (CoA)

Increased pulmonary blood flow

The increase in pulmonary blood volume will decrease the flower (compliance) lung and improve breathing effort. Leakage of fluid into the interstitial space and alveoli, may cause pulmonary edema.

Cardiac remodeling can occur, with cardiac dilatation and hypertrophy of the heart muscle in mild scale. If the condition remains untreated, the pulmonary resistance will continue to rise, and at a time of gout will turn from right to left (Right-to-left shunt) or also called Eisenmenger's syndrome.


A defect in the form of one or more holes that are on the wall that separates the left and right ventricles.

(25-30%)


Anamnesis Physical Examination

Dyspnoe Heart sounds : normal

Impaired growth Thrill palpable and noisy pansystolic

Lung Infection (sindrom eisenmenger)

In Eisenmenger: cyanotic (right-left shunt), the child fails to grow,

heart sounds II hardened

More to VSDMore to VSD

Thorax: cardiomegaly, increased pulmonary vascularity, hilar blood vessels thicken

ECG: an increase in the activity of the left ventricle, mitral P (enlargement of the left atrium), when pulmonary hypertension → right ventricular hypertrophy

Echocardiography: the direction of the shunt, the pressure a.pulmonalis

Cardiac catheterization and angiography: to prove the increase in oxygen saturation in the right ventricle. Angiography shows the magnitude and direction of the shunt, and detecting aortic regurgitation


Congenital heart disease form holes (defects) in the interatrial septum (septum between the porch) that occurs due to failure of fusion of the fetus - the interatrial septum.


Primum ASD

There is a gap at the bottom of the atrial septum, namely the atrial septum primum.


ASD secundum

There are holes in place fossa ovalis pathological. Defects can be small to very large so that it covers most of the septum. The result is a shunt from the right atrium to the left atrium, with a load volume in the atrium and right ventricle


Clinical features:

Dyspnoe and recurrent respiratory tract infections

Physical examination:

There noisy "systolic ejection" on the left second intercostal space


Supporting investigation

Torax Photo: AP chest radiograph showed right atrial prominent, and with prominent pulmonary conus, right atrial and ventricular enlargement. Dilated pulmonary arteries, and increased pulmonary vascularity

ECG obtained right axis deviation, normal atrial complex, normal conduction, and left ventricular hypertrophy. Primum defect showed left axis deviation (left axis deviation) first-degree AV block (PR interval extenders)


The ductus arteriosus open after birth (5-10%)

Due to higher aortic pressure, blood flow around 5-10 mm distal to the left subclavian artery through the duct runs from the aorta to the pulmonary artery generally funnel-shaped with a smaller orifices that lead to pulmonary artery.


Mild Medium Heavy

Anamnesis asimptomatik feeding difficulties, recurrent respiratory tract infections

Difficulty eating and stunted growth

Physical examination

Palpable vibration noise in the second intercostal left sternal

-Palpableof Noisy vibrations in the second intercostal space left sternal-- On auscultation: continuous noisy II-III intercostal left sternal

-Wide pulse pressure-Palpable vibration noise in the second -intercostal space left sternal noisy systolic-Middiastolik noisy at apex

Radiology Normal EKG and Ekokardiografi

- Photo thorax: cardiomegaly, increased pulmonary vascularity, enlarged hilar vesselsECG: left ventricular hypertrophyEchocardiography: left atrial dilation and a.pulmonalis


Endocardial cushion defects (ECD) or AVSD In this disorder does not happen the separation between the mitral valve ring and tricuspid valve so that there is a big hole atrioventricular valve ring connecting the two atria and two ventricles simultaneously, complete Form consists of the posterior inlet VSD, ostium primum ASD that may progress to ventricular defect, and the anterior portion of the mitral valve.


On history, ECD can occur without symptoms.

In extreme cases, patients may indicate poor feeding, chronic of upper respiratory tract infections, pneumonia, and growth failure.

Might also be obtained information from mothers of children who often cry, often stops when eating, and their nostril breathing. In older children, the manifestation of ECD is more toward a state of congestive heart failure, with reduced activity and play, fatigue, spasms, and edema.

Increasing Pressure Load

Babies can experience a critical situation a few hours after birth. Severe pulmonary stenosis in the newborn period will show symptoms of right heart failure (hepatomegaly, peripheral edema) and can occur right-to-left shunt through a patent foramen ovale were not closed, so cyanosis may occur later.

Severe aortic stenosis in the newborn period will show symptoms of left heart failure (pulmonary edema, poor perfusion), and may be accompanied by progressive total circulatory collapse.


The presence of an obstruction in the road or a right ventricular outflow tract. Pulmonary and branches. Narrowing of the pulmonary stenosis can occur under the valve, which is in the infundibulum (Subvalvular or infundibular stenosis), the valve alone (valvular).


Pulmonary valvular stenosis

One such disorder is a bicuspid pulmonary valve (only consists of two cusps), or thick and each valve attached to the end that causes pulmonary valvular stenosis.

Infundibular pulmonary stenosis

Tissue resorption disruption bulb cardiac hypertrophy occurs during development infundibulum which will cause the infundibular pulmonary stenosis


Physical Examination:

On palpation of the chest, ribs palpable vibration noisy II interrupted the left edge of the sternum

The first heart sound normal ejection followed click

Split second heart sound heard widening with increased severity of obstruction, due to increased right ventricular ejection duration

Noisy systolic pulmonary stenosis always sounded on, rough nature, punctum maximum in the second intercostal space left parasternal and spread along the left sternal line and apex

Supporting Investigation:

X-ray: dilatation pascastenosis on a.pulmonalis, heart size is normal but who have experienced heart failure

ECG: QRS axis deviation to the right with the right ventikel hypertrophy, which is indicated by the high R wave in the conduction of the right chest with deep S waves in V5 and V6


Narrowing of the aorta which can occur at the level of Subvalvular, valvular, or supravalvular

SA valvular:

Most of the aortic valve has 2 pieces valve (bicuspid) which will usually cause mild or moderate aortic stenosis.


SA Subvalvular:

Subaortic stenosis, similar in membrane below the aortic valve

SA SUPRAVALVULAR:

From the outside of the aorta appeared normal, but the walls are very thick, which is composed of fibrous tissue. Aortic lumen becomes smaller, can be up to 1/3 the normal lumen. Ventricular hypertrophy is often found.


Anamnesis

Substernal pain

Dyspnoe

Dizziness or syncope during

the activity


With increasing degree of obstruction, pulse intensity will be reduced, enlarged heart, and the apex will be pushed to the left

In aortic valvular stenosis is mild to moderate tone will sound "early systolic ejection", the most audible at the apex and left sternal line.

Systolic noisy vibrations are in the crook suprasternal or in a.karotis. on auscultation

mild aortic stenosis split second heart sounds were normal in the second degree heart sounds heard are single

In severe aortic stenosis will sound paradoxical split second heart sound.



ECG: may show left ventricular hypertrophy due to left ventricular pressure load

ECHO: clearly indicate the type of stenosis (Subvalvular, valvular, supravalvular) and aortic valve biskuspid. With Doppler can determine the degree of stenosis


Localized narrowing of the aorta which generally occurs at the ductus arteriosus (juxtaductal coarctation).


Clinical manifestations

shortness of breath, hepatomegaly, with a small pulse, oliguria or anuria

headache, pain in the legs and feet, or occur epistaxis (in older children)


Palpable of brachial pulse that normal or strong, while the femoral and dorsalis pedis pulse was not palpable or impalpable small

On auscultation of the heart I and II are generally normal, and to be found in the area noisy subtle systolic pulmonary



X-ray: left ventricular hypertrophy, rib notcing, picture 3 reverse

ECG: look right ventricular hypertrophy. In older children there are descriptions of left ventricular hypertrophy with an inverted T at V3 and V6

General handling

- ASI / High calories of formula milk.

- NGT / OGT (with heart failure - low water and salt)

- Profilactic antibiotic of any dental prosedure.

Conservative - MM

The objective medical therapy also to avoid the development of pulmonary vascular obstructive disease or the development of congestive heart failure. If it turns out heart failure and circumstances related to lung problems still occur, diuretics and digoxin is the indication.

Diuretics can be given is furosemide (Neonates is 0.5-1 mg/kg/day in 1-3 a day). In infants and children, the dose given was 0.5-2 mg/kg/day, in 2-4 feedings.

Digitalis in children is 0.25 mg/kg/day given in 4 doses every 6 hours. In premature infants, the maximum dose is 20 mg/kg/day orally. In children, the recommended maintenance dose is 8-12 mg/kg/day divided into two doses.

Conservative - MM

ACE Inhibitor such as captopril. Dose for neonates is 0.05-0.1 mg/kg/dose given 1-4 times per day. For infants, the recommended dose is 0.15-0.3 mg/kg/dose every 6-24 hours. For older children, the dose of captopril is 0.3-0.5 mg/kg/dose in 1-4 feedings.

PDA - Indomethacin, at a dose of 0.1 mg/kg IV every 12 hours in 3 doses for infants < 48 hours. In infants aged 2-7 days the dose of 0.2 mg/kg iv / 12 hours divided into 3 doses. For infants > 7 days is given a dose of 0.25 mg/kg iv / 12 hours in 3 doses.

CoA - prostaglandin-E : 0.05-0.15 mcg/kg/min IV, then the dose is gradually lowered to 0.02-0.05 mcg/kg/min.

Surgical therapy

Conclusion

Congenital heart disease (CHD) is a common heart defect that is caused by the progress of CV (cardiovascular) system during embryo.

Generally caused by two factors: endogenous factors (heredity) and exogenous factors (environmental). Hereditary disorder can be caused by abberasi chromosome and gene mutations, whereas the environmental causes mainly due to viruses or other rubella in the mother during pregnancy, drugs such as thalidomide and other can be caused by radiation.

Thank You…!!!

dieter pjb asianotik.ppt

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