Congenital heart pathology

2021-01-28 12:00 AM

Diseases acquired by the mother during pregnancy: Viral infections are mainly rubella (Rubella), Rubella syndrome often includes deafness, cataracts, baby's head and possibly in combination with the ductus arteriosus, Pulmonary valve stenosis, ventricular septal defect.

Outline

Congenital heart disease is defects in the heart and large blood vessels that are caused by abnormalities in the fetus during the 2-3 months of pregnancy, during the period of the formation of large blood vessels from the primitive ductus. The rate of about 5% in newborns according to French and American documents, in Vietnam currently does not have any statistics.

Reason

Diseases acquired by the mother during pregnancy: Viral infections are mainly rubella (Rubella), Rubella syndrome often includes deafness, cataracts, baby's head and possibly in combination with the ductus arteriosus, Pulmonary valve stenosis, ventricular septal defect.

Genetic factors with or without chromosomal disorders such as congenital heart disease in Marfan syndrome, hip dislocation, Down syndrome, and single-gene mutations are also mentioned in the family genetics of common disease. ventricle, atrial communication, visceral island ...

Recently, it has been found that in pregnant women who are alcohol addicted, they can give birth to babies with birth defects called fetal alcohol syndrome, including baby's head, small eyes, small forehead, small, slow jaw. fetal development, ventricular septal, atrial septal ...

Classify

Shunt left-right, right-left or without a Shunt

Left - Right Shunt: A type of heart that is congenital without purple. Blood flowing from the left high pressure to the right with lower pressure. However, over time due to severe pulmonary hypertension that can reverse the shunt at that time, it is called Eisenmenger syndrome. In this group often see atrial septal, ventricular septal, and the ductus arteriosus.

Type with right-left shunt: Usually, the defects cause increased pressure in the heart cavity to make blood from right to left causing mixing of arterial blood and venous blood, purple, appearing when Hb> 5g / 100ml and degree O2 saturation <70%, this is congenital purple heart disease. Diseases of this group such as Fallot quartet, Fallot trilogy,

Shunt-free type: A birth defect in the heart or large blood vessels but without a shunt such as aortic stenosis, aortic stenosis or a lung ...

Congenital heart disease with left and the right flow

Atrial septal defect

The most common congenital heart disease (15-20% of cases). The simple form accounts for only about 20% of all cases of the atrial septal defect. The ratio of female/male in

Clinical form:

Secondary hole: Located in the centre also known as the Bottle hole. Holes round or oval, sometimes elongated or window-shaped. The average diameter is about 20-30mm.

The venous sinus cavities are located above or below:

The hole is above the entrance to the superior vena cava, which almost always includes incomplete pulmonary return abnormality (either directly into the right atrium or in the lower part of the superior vena cava).

Hole in the lower wall: extends to the inferior vena cava (coronary orifice).

Primary loss: Rarer than the above. The opening is located at the base of the anterior coronary cavity. It can be either alone or in combination with the mitral septum leaf funnel.

Pathophysiology:

This type has left-right flow due to higher left atrial pressure. The magnitude of the shunt depends on the size of the hole and the resistance of the pulmonary arterioles. Although the shunt is large, the pulmonary artery pressure does not increase or slightly increase. Volumetric overload dilates the right heart chamber and pulmonary blood vessels. In contrast, the left heart chamber as well as the aorta are small in size. Later damage to the pulmonary arterioles (endothelial hyperplasia, embolism) increases the resistance to pulmonary hypertension and decreases the shunt volume. Eventually, when the right chamber pressure increases too it will reverse the shunt and the patient will have purple.

Diagnostic symptoms:

Secondary loss:

In 90% of cases, an ejection-type systolic blow will be heard in the pulmonary valve drive. The sound spreads along the left sternum but maximal in the 2 or 3 sides of the left sternum. The sound of a murmur is of moderate intensity and has a smooth timbre but can also have a strong coarse sound similar to that found in pulmonary valve stenosis. T2 split sound fixed according to the respiratory cycle. In severe pulmonary hypertension, a diastolic murmur can be heard due to a pulmonary valve opening.

X-ray images highlight the right cardiac dilatation and pulmonary artery. The electrocardiogram often has the right-deflection QRS axis and especially the right bundle branch block imaging is often not complete.

Ultrasound is a very effective diagnostic tool, on two-plane ultrasound images show that the right ventricle increases in size due to diastolic volume overload. It shows a direct image of the atrial septal defect and with colour ultrasound showing the shunt flow through the septum, contrast ultrasound shows the wash zone in the right atrium if the left atrium shunt is right and in the case of shunting, the cardiac contrast can be seen. left.

Cardiac catheterization: There are two of the following symptoms that confirm the diagnosis.

The catheter runs from the right atrium to the left atrium easily (7-8 times / 10).

The oxygen concentration in the atrium must be richer than in the aorta.

In addition, cardiac catheterization also allows assessing pulmonary artery pressure, measuring shunt flow, measuring pulmonary artery resistance.

Evolution:

Depends on vent diameter i.e., shunt flow. Average life expectancy is 35-40 years old. Long-term patients will develop right heart failure, arrhythmias (or atrial fibrillation), when

Primary loss:

Account for 20% of cases of the atrial septal defect. Lower average life expectancy (30 years), surgery more difficult.

Clinically, in addition to symptoms of the atrial septal defect, there is also systolic blowing in the tip due to mitral valve abnormality. Left-axis ECG while having right-branch bloc. Cardiac catheterization has a low atrial septal defect as well as a backflow of blood to the atrium due to mitral regurgitation. Doppler ultrasound also allows for a similar diagnosis.

Combined atrial septal return to pulmonary venous abnormalities:

Or an abnormal return of the right upper lobe or right middle lobe vein to the end of the superior vena cava (sinus venosus form).

Angiographic catheterization allows very good diagnosis.

Lautenbacher syndrome:

Combination of mitral stenosis and atrial septal defect.

Ventricular communication

The most common type of congenital heart accounts for 20-30% of congenital heart diseases. If you include congenital heart diseases with combined ventricular septal combination it accounts for 50%. This type of heart is congenital with left and right shunt (pure form).

Pathology:

The most common is membrane glomerulus.

In the high and anteriorly below the pulmonary valve.

At the posteriorly lower wall of the tricuspid valve.

The rheum is located in the muscular part of the ventricular wall.

The left ventricle may be open to the right atrium (not really ventricular septal defect), often in association with tricuspid valve abnormality.

Pathophysiology:

Since left ventricular pressure is higher than right ventricular pressure, there will be left and right shunt through the septum. The magnitude of the shunt depends on the size of the opening and the resistance of the pulmonary arterioles. In children with a hole diameter> 1.2cm2 / m2 body (or> 1/2 aortic valve hole), there is no difference between the two ventricles and large shunt volume if low lung resistance creates hypertension. lung "supply". The opening can either close on its own over time or the shunt will decrease (or improve at 2 years) due to an increase in the aortic valve aperture.

In ventricular septal defect, the vent muscle becomes smaller in systole. Increased pulmonary arterial resistance can be attributed to the following factors:

Post-capillary pulmonary hypertension: Causes due to excessive blood flow to the left atrium while the mitral valve is closed and when the blood is opened, it is not in time to create mitral stenosis.

The latter increase in pulmonary flow has the consequence of thickening of the mantle between the small blood vessels (stage I), endothelial proliferation (stage II), hyaline degeneration and fibrosis (stage III), dilated necrosis. localized (phase IV). The shunt flow will decrease, but the pulmonary artery pressure will increase.

The pathophysiology will be different if there is pulmonary artery stenosis (congenital or acquired due to secondary adaptation) for a situation similar to the quadruple Fallot.

It is divided into the main ventricular septal groups as follows:

Small ventricular septal defect, left and right shunt normal pulmonary artery pressure, this is Roger disease.

Enlargement of the hole, normal or slightly increased pulmonary arterial resistance.

Group II a: Systolic arterial pressure <70% of system pressure.

Group II b: Pulmonary systolic pressure> 70% systemic pressure.

Group III: Pulmonary hypertension and severe pulmonary resistance, small left and right shunt, mild bidirectional shunt possible. When the shunt is called Eisenmenger syndrome.

Group IV: Lung protective (stenosis of the pulmonary valve or funnel), bounded by the quadruple Fallot (except the left and right shunt).

Diagnostic symptoms:

Clinical: Symptoms of muscle function vary depending on the group of the ventricular septal system as well as the patient's age, which can be asymptomatic or difficult to breathe. Children can grow slowly, deformed chest. Examination with a large systolic blow with mIU fibrillation (not always), maximum rough intercostal tone of the left thymus four sides spread in all directions. Normal or strong chest T2 sounds. In addition, ejection-type systolic blows can be heard due to increased pulmonary flow heard in the left thoracic 2-3 intercostal space, diastolic head fibrillation due to audible mitral stenosis at the apex of the heart. When the pulmonary arterial resistance increases, the murmur will decrease in intensity or disappear, and diastolic fibrillation is no longer heard at the tip.

X-ray: May or may not see the enlarged left cardiac balloon as well as pulmonary artery dilatation.

ECG: left ventricular thickening, left atrium, when there is pulmonary hypertension you can see signs of right ventricular thickening.

Doppler ultrasound: Allows the ventricular to be seen as well as the shunt flow through the vent, assessing the magnitude of the shunt as well as pulmonary hypertension.

Angioplasty: Left ventricular scan allows to accurately see the ventricular septum. In addition, cardiac catheterization also shows the pulmonary artery pressure as well as the pulmonary arterioles resistance, shunt flow.

And the ductus arteriosus

Anatomy: The location of the tube originates from the aortic aorta to the left pulmonary artery sometimes at the pulmonary branching. Rarely enters the right pulmonary artery. Diameter in children 7-10 mm long and 5-7mm wide, tubes may or may not grow with age.

Pathophysiology: The ductus arteriosus creates the left and right shunt. If the shunt is large, the pressure is equal to either side of the circulatory system. Left atrial and pulmonary venous hypertension causes reversible contraction of the pulmonary arterioles for a long time and reduces shunt flow. For a long time, the arterial resistance increases irreversibly and approaches the shunt island or loses the shunt.

In rare cases, there is pulmonary hypertension as well as an immediate increase in pulmonary resistance due to fetal structural abnormality of the pulmonary artery.

Diagnostic symptoms:

Clinical: Usually found by chance on physical examination because the disease is usually well tolerated. Cardiac listening is fundamental to making a diagnosis with a continuous blow in the left subclave. Breastfed babies sometimes only hear a systolic murmur.

If the shunt is large, diastolic fibrillation can be heard due to mitral stenosis, the arterial pressure increases.

X-ray: Heart balloon is normally normal, in the case of the large left ventricular shunt can relax.

ECG: Normal if the shunt is small or imaging left ventricular thickening if the shunt is large.

Cardiac catheterization: allows confirmation of the diagnosis when the catheter is shaped from the pulmonary artery into the aorta (but is usually not required.

Colour doppler ultrasound: Allows catheter to be seen as well as shunt, assessing shunt flow as well as pulmonary artery pressure.

Evolution:

Heart failure: Depends on shunt flow and pulmonary artery pressure. Heart failure followed by rapid total inert heart failure with cardio therapy.

Infectious endocarditis on the duct wall: severe, difficult to treat. An enlargement of the tube can make surgery difficult.

Aneurysm: risk of rupture.

Clinical form:

And the ductus arteriosus suckers: blow systole with a strong T2 in the pulmonary valve drive. Diagnosis may be difficult to differentiate from ventricular septal defect. In the presence of hypertension, dilated aorta as well as pulmonary vasodilation often cause premature heart failure and require early surgical intervention.

The ductus arteriosus has strong pulmonary hypertension: difficulty breathing, possibly purple (especially lower extremities), or lung infection, severe heart failure, chest deformity, physical retardation.

Hearing the heart is no longer continuous blowing, very strong T2 sometimes has diastolic blowing due to a functional pulmonary valve opening.

A cardiac radiograph is large, the pulmonary artery is bulging while the pulmonary periphery is bright at increasing pulmonary arterioles resistance and small shunt.

The ECG increased the combined left ventricular diastolic burden to increase the right ventricular systolic burden (due to large shunt flow or large pulmonary resistance).

Cardiac catheterization, in addition to measuring the pulmonary artery pressure, also allows identifying the mechanism of increased pressure (due to increased flow or pulmonary resistance).

Combination form: aortic stenosis, ventricular septal, aortic stenosis.

Valsalva sinus aneurysm

The Valsalva bulge sac is often latent and found by chance. However, there is a risk of rupture of an aneurysm and infectious endocarditis.

Broken: Often times a strenuous movement causes severe chest pain, extreme dizziness and difficulty breathing. Usually, the aneurysm ruptures into the right ventricle and rarely ruptures into the right atrium. Intense continuous murmur cardiac examination is located in the lower-left sternum and peripheral markers of aortic regurgitation. X-rays and electrocardiograms show signs of both ventricular overloads. Right cardiac catheterization shows large left and right shunt in the atrioventricular or ventricular layer as well as indicates the degree of pulmonary hypertension. Doppler ultrasound also allows the diagnosis of rupture of the Valsalva aneurysm. Progressive death rapidly within weeks or months of heart failure.

The rupture of an aneurysm into the pericardium can cause pericardial bleeding, rupture into the left atrium, creating a continuous blow without a shunt, rupture into the left ventricle causing aortic valve opening.

Differential diagnosis with coronary artery probe, master waste detector.

Other complications: An aneurysm may also rupture. When the sac enlarges towards the ventricular septum, conduction disturbances may occur.

Congenital heart disease with right and left flow

The Quartet of Fallot

Is a combined birth defect including the following 4 conditions?

Pulmonary artery stenosis.

Ventricular communication.

The aorta deviates to the right.

Thick right ventricle concentric.

Clinical purple finger-foot drumstick, children when tired or squatting, shortness of breath, fainting. Hear has rough strong systolic blowing in the 3rd thymus intercostal space due to pulmonary artery stenosis. The T2 sound decreases or is lost.

Heart radiographs are normal but are angular, with a concave mid-left arc, small pulmonary arteries, and a bright lung field.

Electrocardiogram has right ventricular thickening and right atrium.

Ultrasound showed the equestrian aorta, intermembrane septal defect, right ventricular thickening, as well as examination of the pulmonary valve and its trunk.

Angiography allows for the diagnosis of abnormalities in this birth defect.

 

Epstein disease

A rare congenital abnormality of the tricuspid valve. The septal leaves and posterior leaves stick together and only separate in the lower 1/3. They form an abnormally low lower valve hole. thus, creating atrial ventricular disease.

The atrial part of the ventricle contracts at the same time as the atrium, thus hindering the filling of the atrium. The tricuspid valve opening is narrow or open. As a result, circulatory stasis in the upstream of the tricuspid valve, if there is an atrial septal combination, there will be left-right shunt, if the atrial septum is normal, there is peripheral venous stasis (enlarged liver, cervical vein floating, ascites).

Listening to the heart, there are heart sounds separating due to the complete right branch block, the T1 component of the tricuspid valve sounds metallic. Common systolic murmur caused by tricuspid valve regurgitation is characterized by shifting to the left compared to normal, causing mitral regurgitation.

X-ray with large heart balloon can mistake pericardial effusion. ECG with right atrial deviation and right branch block imaging.

M-mode ultrasound showed that the tricuspid valve closed 0.04-0.12s slowly compared to the mitral valve, the two-plane ultrasound showed low tricuspid adhesion.

Angiogram revealed atrioventricular atrial condition as well as right heart chamber pressure measurement.

Progressive change, average life 13 years. If passing the teenage period can live 30-40 years. Cause of death due to stroke, right heart failure, arrhythmia.

Eisenmenger complex

First described in 1897 as different from the quartet of Fallot. Late cyanosis, including the following malformations:

Inter ventricular high.

The aorta comes from the ventricular septal area that can be a horse.

Right ventricular thickening.

Pulmonary artery dilatation.

ECG: Usually 2 ventricular thick. X-ray: The baby has a large heart and dark hilum.

Currently, all congenital heart diseases are classified with left-right but reversed shunt

The right-left shunt is called Eisenmenger syndrome.

Congenital heart disease without a hole

Aortic stenosis

Clinical manifestations: the moderate intensity of systolic left thymus, no mIU fibrillation but clearly posterior spread, increased blood pressure and strong pulse in the upper extremities, low blood pressure and weak pulse in lower extremities.

Electrocardiogram, X-ray: Strong or moderate left ventricular thickening depends on aortic stenosis near or far from aortic valve hole, moderately large left ventricle with upper left collar aortic dilatation accompanied by rib erosion (dilated intercostal arteries).

Doppler ultrasound: See the position and size of the stenosis, the flow increases speed through the narrow place and mark the effects of the aortic stenosis (left ventricular dilatation ...).

Cardiac catheterization, angiography: Left ventricular pressure increases, pressure in the aortic systole increases in narrow upper but normal in the inguinal artery. Angiography shows the narrow position, decrease in lateral circulation of the intercostal artery, the inner breast artery.

In addition to aortic stenosis, there is congenital sub-aortic stenosis due to the existence of an inferior membrane blocking left ventricular ejection.

Pulmonary valve stenosis

It accounts for 7% of congenital heart disease including stenosis, sub-valve stenosis, funnels stenosis, and coordination.

Clinical manifestations: Strong systolic blow in the pulmonary valve drive with cataracts, mild or absent T2, late cyanosis.

Electrocardiogram, X-ray: Selective right ventricular thickening, pulmonary artery body dilatation and swelling, RV enlargement, decreased blood vessels in both lung fields (bright lung).

Ultrasound-Doppler: Shows the stenosis, sub-valve, funnel or coordination, thick right ventricle, high blood speed through the pulmonary valve increased, the difference through the stenosis increased.

Cardiac catheterization, angiography: Seeing the pressure difference between the right ventricle and the pulmonary artery increased (right ventricular pressure increased while low in the pulmonary artery). Angiogram revealed narrow, contrast pass through the stenosis.

Treatment

Atrial septal defect

Most atrial fissure does not lead to heart failure. The pressure difference between the two atrial chambers is not high so that the atrial opening is also free from infectious endocarditis complications. Therefore, there is usually no need to use prophylactic antibiotics for tooth extraction, dental treatment, or surgical procedures.

Medical:

Only a relative role is when there is no indication for surgery such as:

Treatment of pulmonary superinfection.

Treatment of arrhythmias in the presence of atrial fibrillation, paroxysmal supraventricular tachycardia.

Treatment of heart failure.

Treatment of prevention of embolism.

Closing the atrial catheter with a catheter inserting an instrument called a double parachute into the catheter is a procedure performed by cardiologists.

Surgery:

With external circulation surgery, close the atrial septal hole by stitching directly or with a synthetic patch. The atrium can close on its own.

Indications for surgery: The age of surgery has the author so that each one at school age is 3-5 years old, or it can be done at the age of 15-20, but in general, the atrial surgery is usually not urgent as Ventricular ventilation due to pulmonary hypertension complications usually occurs later. There is research for newly discovered elderly also should perform surgery. Indications are usually consistent when:

Pulmonary blood flow index / total arterial blood flow index.

Unchanged shunt (O2 concentration of arterial saturation (92% or DMP <15 Woods units / m2 body).

No surgery when: Doppler pulmonary artery pressure is close to systemic vascular pressure, very little and bidirectional flow, O2 saturation below 92% and less during exercise. (Ho Chi Minh City Heart Institute).

Ventricular communication (TLT)

In general medicine, it is necessary to treat complications of TLT such as heart failure, pulmonary superinfection, infectious endocarditis. Newborn surgery usually has a 10-20% higher mortality compared to older children 2%. The septal septum can also close on its own. In severe cases, it is easy to switch to Eisenmenger syndrome with surgical difficulty.

Ventricle with small vents, small shunt:

There is no surgical indication because the patient is well adapted. Only prevent endocardial infection. However, small hole TLT accompanied by aortic valve opening (Laubry - Pezzi syndrome), though mild, should also be removed early.

Large ventricular septal, large left-right shunt (group II ventricular septal):

Occupy 50%. Internal medicine helps treat these two complications when the patient does not have an indication for surgery. Treatment is mainly surgical with external circulation, using an artificial patch. The risk of death ranges from 1-2% in the mild body and <10% in the severe form. Common surgical complications are caused by damage to His bundle causing an atrioventricular block or branch block.

Indications: Should be questioned from 2 -10 years old, pulmonary artery pressure index / total arterial pressure (0.75 with heart failure need immediate surgery. If heart failure is stable during medical treatment

In addition to the two clinical blood types above, two other special groups are less common:

Group III with severe pulmonary hypertension, close to Eisenmenger syndrome, but the left-right shunt is still very weak. There is no indication for surgery.

Group IV with stenosis or funnel DMP (ventricular septal defect) is close to the quadrilateral Fallot, but the left-right shunt remains, though very weak. Surgery is needed if RV thickening is clear.

And the ductus arteriosus (CODM)

All patients with diabetes, if unable to close on their own, need surgery because there are many risks if not resolved such as infectious endocarditis, heart failure, pulmonary hypertension, ductal calcification.

Medical:

In infants and nursing mothers can use indomethacin (Indocin) 25mgx 1-2 tablets/day 1 month to close the ductus arteriosus due to the drug inhibiting the spasmolytic effect of Prostacyclin. There are authors using Aspirin also results. Without results

Surgery:

With a ligation or catheter surgery if the hole is less than 5mm in diameter and the child (8kg. Surgical application for 1-2 years old but maybe older but not changed shunt. report 1-2% due to heart failure, Osler.

According to Kirklin, all symptomatic AGMs or children who are not growing despite active medical treatment can have surgery at any age.

The ductus arteriosus with a large shunt and mild pulmonary hypertension:

Surgical treatment: Forced cutting. The risk of death is higher than in the group above.

Longer ductus arteriosus with a small shunt and severe increased pulmonary pressure.

Treatment: Surgical indications need to be discussed.

If the pulmonary pressure resistance is more than 10 units / m2, surgery is no longer indicated. If the pulmonary vascular resistance is lower but the ductus arteriosus is short and calcified, open-heart surgery to avoid rupture of the artery when clamping in closed surgery.

 The Quartet of Fallot

Internal Medicine: Treatment is only temporary, alleviating the symptoms prepared for surgery. Reduce RV ejection with-blockers such as propranolol 40 mg x 1 v orally. Give anticoagulants or antiplatelet agents such as Aspirin 0.25g / day. Infants with insufficient pulmonary circulation can pass prostaglandin E1 to keep the ductus arteriosus open. All children with a high globular capacity need an extra iron tablet. Anti-paroxysmal hypoxia: for low knee-chest, O2, Morphine 1ctg 0.01-0.1 mg / kg TB, sodium bicarbonate infusion.

Surgery: Can have temporary surgery or repair surgery. The ideal age for corrective or radical surgery (patching the ventricular hole with graft and repairing enlargement of DMP) is 2 years. To slow-growing old will have many complications after surgery.

Left ventricular condition and pulmonary artery size were important factors in determining this technique.

About temporary surgery or the common technique is Blalock - Taussig (link lower left arm and left arm DMP) and Blalock - Taussig modified (connected by Gore-Tex tube). Do not perform Blalock - Taussig surgery in children under 6 months old or having diameter (3 mm) because they are prone to embolism.

Surgery is required early when symptoms of muscle function increase or red blood cell volume is about 65%.

Mortality is about <5% for Blalock and 10% for radical treatment. Skill

Blalock only gives good results in about 50% of cases within 10 years.

Pulmonary artery stenosis

 Internal Medicine: Less effective. Considered lightly when the difference in right ventricular pressure / PP (25 mmHg, severe when the difference is between 25 and 50 mmHg. Open surgery at present, only percutaneous balloon aspiration is indicated, but of high value, if the age is young This is a selective method, with almost no mortality.

Surgery: Only applied when the transcutaneous valve is not available. When the right ventricular pressure is more than 50 mmHg higher than the pulmonary artery pressure. Surgery to create a mitral pulmonary valve with or without repair of the pulmonary funnel. When there is heart failure, the mortality rate is over 14%.

Aortic stenosis

Surgery: Surgery should be raised as soon as the patient can tolerate it. The ideal age is from 10 to 15 years old, and later there is a high risk of co-atherosclerosis. The risk of death is less than 5%. Surgery by connecting or connecting with an artificial tube.

Complexes and Syndrome EISENMENGER

Internal Medicine: Treatment of complications of heart failure: O2, heart support, diuretic ...

Surgery: Surgery is not indicated except for heart replacement.