Treatment of congenital heart diseases by Doppler ultrasound

2021-08-07 08:04 PM

Treatment guidelines for congenital heart disease with cyanosis, congenital heart disease without cyanosis, congenital heart disease in adults.

Ventilation

General perception

Congenital heart disease without cyanosis.

20% of congenital heart types.

85% of the cases are membranous ventricular septal defects, 15% of the funnel, other more rare parts: the receptacle, the trabecular muscle (with the possibility of many small holes).

The hole is a few mm, the left ventricular pressure difference - right ventricle does not change. If the stoma is larger but < 10 mm => right ventricular pressure increases. If the orifice is > 10 mm, it causes right ventricular hypertension, and the pulmonary artery leads to equalization of left ventricular pressure - right ventricle.
 
Ventricular septal defect diameter > aortic annulus diameter: left-right shunt decreases over time and then reverses the shunt due to increased pulmonary artery resistance and pulmonary artery stenosis secondary to often in the funnel (decreased and then reversed). causes Eisenmenger syndrome and eventually resembles T4F in the clinical setting).

Before 2 years of age, changes in pulmonary vascular resistance and funnel stenosis are common.

Ventricular septal defect after the age of 30 - 40 years is very rare because most small ventricular septal defects are closed over time.

Ultrasound: hole > 3 mm, on 2D can see other cases can use Doppler and color Doppler to detect.

Treatment

The appropriate age for surgery is 2 years old, in the case of infants undergoing surgery when there is pulmonary hypertension (≥ 70mmHg, surgery is about 9-12 months) or respiratory disorders (pulmonary artery dilatation and hypertension).

The ventricular septal defect can close on its own, possibly with Eisenmenger complications.

Small hole ventricular septal defect: no pulmonary hypertension => no need for surgery, surgery when symptomatic.
 
Small hole ventricular septal defect with pulmonary artery pressure ≥ 70 mmHg with heart failure that does not improve despite medical treatment => surgery is required. If HF therapy helps, check at 6 months of age, if pulmonary artery pressure ≥ 70 mmHg => surgery, if < 70 mmHg with reduced ventricular septal defect => appointment 6 months later to check again.

Ventricular septal defect with pulmonary artery pressure <70 mmHg with no heart failure or heart failure with stable medical treatment => ultrasound examination every 6 months.

Ventricular septal defect at the age of 4 - 5 with pulmonary artery pressure ≤ 55 mmHg with QP/QS ~ 1.5 -2 => surgery, if <1.5 => ultrasound every 6 months.

Adults with small ventricular septal defect with normal pulmonary artery pressure with QP/QS < 1.3 => medical treatment, if QP/QS ~ 1.3 - 1.5 => consider surgery, if QP/QS > 1.5 and pulmonary artery pressure ≤ 55 mmHg => need surgery.

Adults with a large ventricular septal defect with pulmonary artery pressure ≥70 mmHg but low QP/QS: surgery is not recommended.

Atrial communication

Congenital heart disease without cyanosis.

It accounts for about 15% of the congenital heart.

First hole atrial septal defect: near the AV junction, the size can be as large as the common atrium. Lesions are often associated with a ventricular septal defect or a common atrioventricular septum.

Atrial septal defect of the second foramen: near the foramen ovale (need to distinguish because common foramen ovale can be encountered in 10-35% of normal people). The size of the atrial septal defect is from 1 to 3 cm, the abnormality is often accompanied by an aneurysm of the atrial septum.

Atrial septal defect in the venous sinus: defect at the right atrium of the vena cava. The superior vena cava region is more common, the inferior vena cava region is rare. Accompanied by incomplete pulmonary vein abnormalities may be seen.

Atrial septal defect in the coronary sinus: the defect of the coronary sinus causes an atrial septal defect between the left atrium and the coronary sinus, the flow will be more important if the left superior vena cava drains into the coronary sinus.

2D ultrasound, Doppler will show location, size, classification, the direction of flow.

Pulsed Doppler image shows a continuous vortex flow with at least 3 peaks from mid systole to end-diastole.

The size of the right ventricle, right atrium, paradoxical interventricular septal movement suggests that the right chamber is increased in volume.

Measure pulmonary artery size, systolic, diastolic, and mean pulmonary artery pressure. Pay attention to the location of the pulmonary veins.

Internally treatment

Close the vent with an umbrella.

Surgical treatment

Surgery for all atrial septal defects with a QP/QS ≥ 1.5 with or without functional symptoms.

Surgery should be performed at the age of 3 - 5 years, the atrial septal defect can close on its own in 14 - 66%, therefore, surgery should not be performed at the age of < 1, except for heart failure or uncontrolled pulmonary hypertension.

Atrial septal defects discovered in old age can still be closed.

Atrial septal defect often complicates pulmonary hypertension at the age of 30 to 40 years, but surgery is possible when pulmonary vascular resistance is < 14 units/m2 and arterial blood O2 saturation ≥ 92% if the atrial septal defect is open. Severe 2 leaves can be operated despite pulmonary vascular resistance > 14 units/m2 (valve repair is recommended).

Do not operate when pulmonary artery pressure ~ aortic pressure, O2 saturation at rest < 92%.

Atrial septal defect in people 50 - 60 years old with pulmonary artery pressure ~ 50 mmHg still gives good results.

Patent ductus arteriosus

Congenital heart disease without cyanosis.

accounted for 10% of congenital heart.

15 hours after the birth of a full-term baby, the mesenchymal layer constricts leading to occlusion of the ductus arteriosus.

By the end of 8 weeks, the ductus arteriosus closed 90% of the cases with hemorrhagic rupture of the subendothelial layer and the formation of a fibrous occlusion of the lumen of the ductus arteriosus, with closure starting at the apex of the pulmonary artery. Sometimes pseudoaneurysms are seen in the neonatal period, which subsides spontaneously.

The ductus arteriosus is 2-15mm long, 5-15mm wide

Arterial duct ≤ 7mm: pulmonary artery pressure is slightly increased, when there is pulmonary artery pressure, the orifice size is usually > 7mm.

When there is a 2-way shunt or right-left shunt, surgery is contraindicated.

Internal treatment

Use Indomethacin.

Surgical treatment

The tube should be closed before complications of mechanical pulmonary hypertension change the direction of the shunt (Eisenmenger).

Newborns with heart failure complications need intensive medical treatment, if not controlled, catheter occlusion with cardiac catheterization or surgery.

With medical treatment to control heart failure, pulmonary artery pressure ≥ 70 mmHg requires repeat ultrasound at 5 months of age. If pulmonary artery pressure remains >70 mmHg, it should be closed before 6 months to avoid inversion of the shunt. If pulmonary arterial pressure decreases, re-examination at 12 months, and ductus arteriosus is closed at 1-2 years of age.

Heart failure or a child who is not growing up despite intensive medical treatment can close at any age, including neonates.

Pulmonary resistance > 10 units/m2 with no indication for closure.

Short, highly calcified ductus arteriosus requiring open-heart surgery.

Non-surgical ductal occlusion

Atrioventricular canal

Congenital heart disease without cyanosis.

Total atrioventricular canal: ventricular septal defect, atrial septal defect, and severe AV valve injury, mitral and tricuspid valves share a common AV valve opening and are usually 5 pieces.

Partial atrioventricular canal: 2nd hole type or unique atrial septal defect. The mitral valve usually has an interstitium that divides the valve into 3 pieces. The common anteroposterior atrioventricular valve is connected by a small piece of valve tissue thereby dividing the atrioventricular orifice into two separate orifices.

Supersonic:

Atrial septum, interventricular septum. Atrioventricular valve: see if the valve slit, the AV valve is attached to the interventricular septum (partial atrioventricular canal) or not (totally).

At with the first foramen septal defect. The first foramen septal defect is usually evident during systole.

The ventricular septal defect in the atrioventricular canal, due to its close proximity to the ligaments, may be obscured during diastole, thus making it visible during systole.

The common atrioventricular valve is usually five-piece or has a blade of fibrous tissue that connects the atrioventricular annulus to the leaflet, bridging posteriorly thereby dividing the atrioventricular orifice and the two sets of atrioventricular valves.

The mitral slit will point toward the interventricular septum of the receiving chamber, while the slit of the mitral valve will actually point toward the interventricular septum of the ejection chamber.

Muscle columns are located at 2 o'clock and 4 o'clock instead of 4 o'clock and 8 o'clock.

Complete atrioventricular canal: Poor prognosis, children usually die in the first 2 years, need surgery before 6 months of age, after 1 year of age, surgery is no longer indicated.

Partial atrioventricular canal: Better prognosis, surgery is required before mechanical pulmonary hypertension.

Pulmonary valve stenosis

Congenital heart disease without cyanosis.

10% congenital heart.

The pulmonary valve has 3 leaves: anterior, right posterior, and left posterior.

Valve stenosis: the leaflets are thick, stick together at the edge, so they form a tip that shoots out, at the apex into a small hole a few mm in diameter.

Supravalvular stenosis: rarer than valvular stenosis, may be located directly above the valve, maybe at the pulmonary trunk at the division of the proximal or distal branches.

Subvalvular stenosis: narrowing at the funnel or in the lumen of the right ventricle.

Pulmonary valve stenosis in children, older children, and adults: is considered mild when the difference in right ventricular/pulmonary artery pressure is ≤ 25mmHg; Moderate weight from 25 to 50 mmHg, the slower the intervention, the more severe the narrowing is due to the enlarged funnel area.

Newborns with severe pulmonary stenosis: Infusion of prostaglandin E1 helps the ductus arteriosus to open to get blood to the lungs, on a stable day, angioplasty or immediate surgery is indicated.

Young children (1 month - 12 months): severe pulmonary valve stenosis even though no symptoms need immediate balloon angioplasty or surgery, no intervention when mild stenosis, moderate stenosis needs to be considered on a case by case basis. For example, despite moderate stenosis, ventricular hypoplasia requires immediate surgery.

Pulmonary valve stenosis in older children and adults in severe form has little progress to severe, so there is little need for intervention, but if it is accompanied by right ventricular hypoplasia, it also requires early surgery.

In summary: surgery is indicated when: child < 2 years old if narrowing; 8-12 years old with right ventricular pressure ≥ 70 mmHg, right ventricular pressure < 70 mmHg with right ventricular end-diastolic pressure > 10 mmHg.

Coarctation of the aorta

Congenital heart disease without cyanosis.

7.5% of congenital heart types.

Simple

High blood pressure develops when the lumen of the aorta is <50% of normal.

Indications for surgery are cases with aortic diameter < 50% and decreased or absent inguinal vessels or elevated blood pressure ≥ 150 mmHg. The appropriate age for surgery is 2 - 5 years old.

Syndrome

Unadapted coarctation of the aorta causes premature heart failure. There is no difference between the upper and lower extremity blood pressure because the associated lesion is a shunt.

Accompanying ventricular septal defect leads to premature heart failure.

Mortality > 50% in infancy.

Summary

95% in the upper part of the descending aorta, right next to the left subclavian artery, people distinguish anterior ductus arteriosus, posterior ductus arteriosus.

Neonates with severe coarctation of the aorta (heart failure, metabolic changes, femoral vasoconstriction, shock) prostaglandin E1 infusion to help open the ductus arteriosus, surgery, or balloon angioplasty as early as 6-12 hours or immediately thereafter.

Surgery, the duration depends on symptoms of heart failure. Newborns with severe heart failure => intervention immediately after hemodynamic stabilization. For children with no symptoms of heart failure, systolic pressure difference over 40 mmHg, some authors advocate surgery at 3-6 months of age should not be older than 6 months.

Valsalva sinus aneurysm

Congenital heart disease without cyanosis.

0.15 - 0.35% congenital heart

Sinus bulges originate at the base of the heart and spread to the chambers of the heart.

The left coronary sinus does not originate from the ventricular septum, so aneurysms are rare.

2D-ultrasound detects aneurysms, combined with Doppler to determine rupture.

The sinus bulge will be large and disproportionate.

If no rupture, follow up periodically with ultrasound.

Surgery in the presence of complications or increased cardiac volume.

Indications for surgery are absolute when rupture.

The concomitant injury should be treated.

Atrioventricular - ventricular - great artery incompatibility

Congenital heart disease without cyanosis.

1% congenital heart.

Single form

The left atrium connects to the right ventricle through a tricuspid valve.

The right atrium connects the left ventricle through the mitral valve.

The aorta from the right ventricle, the pulmonary artery from the left ventricle.

Possible Ebstein-shaped tricuspid valve, abnormal mitral valve.

Form with a ventricular septal defect

In addition to incompatibility, there is also a ventricular septal defect.

Treatment: Shorten the pulmonary artery, to protect the pulmonary blood vessels, wait 18-24 months, then close the ventricular septal defect.

Form with ventricular septal defect and pulmonary stenosis

In addition to incompatibility, there is also a ventricular septal defect and pulmonary stenosis.

Under 2 years old: severe cyanosis with red blood cells > 6.5 million. Temporary repair by connecting the subclavian and pulmonary arteries.

Over 2 years of age: without associated damage, put in place left ventricular - aorta, right ventricular - pulmonary artery.

No aortic valve hole

Congenital heart disease without cyanosis.

1-2% congenital heart.

The left ventricle does not develop.

Diagnostic imaging by basic cross-sections.

No pulmonary valve hole

Congenital heart disease without cyanosis.

Heart tumor

Congenital heart disease without cyanosis.

75% are benign (myxome), 25% are malignant (sarcome).

Myxoma

75% in the left atrium, usually attached to the endocardium, broad base, pedunculated, tumor diameter from 1-15cm.

Mucinous neoplasms are often heterogeneous on 2D and do not have layers as thrombus, or are located in the anterior and medial portions of the left atrium while the thrombus is in the posterior wall of the left atrium.

Other benign primary heart tumors

Rhabdomyomas, fibroids, adipose tissue...

Sarcoma.

Thrombus.

It is often a complication of myocardial infarction, usually in the left and right ventricles. usually located at the apex of the left ventricle.

Spontaneous echogenicity (SC) can be seen.

Cyanotic congenital heart disease

Ultrasound: systematic investigation of all cyanotic congenital hearts is required:

Situs solitus or situs invensus.

Atrioventricular correlation.

Relationship between ventricles and great arteries.

Do a full overview to avoid injury.

T4F (tetralogy of Fallot)

Cyanotic congenital heart disease

9% congenital heart.

In T4F, it is common to have isolated pulmonary funnel stenosis, or surgical stenosis with valvular stenosis, very rarely isolated valvular stenosis.

The pulmonary trunk is often underdeveloped in the T4F pulmonary stenosis, often without dilatation behind the stenosis. Narrowing at the origin of the branch or branch is rare.

The ventricular septal defect is wide, the membranous part spreads to the funnel region.

Equestrian aorta, severe when ≥ 50%. There is always a mitral-aortic continuum (distinguishable from the right ventricular dilatation).

Combined lesions can be: right aortic arch, abnormal return of systemic venous return, left superior vena cava empties into the coronary sinus, and ductus arteriosus, the existence of foramen ovale (2/3) old T4F), coronary artery abnormalities.

Ideal surgery is 2 - 3 years old (between 6 months and 5 years old surgery is less risky)

Underdeveloped pulmonary artery < 50% (calculated by skin area); abnormal coronary artery path; Children < 5 kg with Hct > 70% with severe symptoms usually have temporary surgery.

Small pulmonary valve ring and

[pulmonary artery (right) + pulmonary artery (left)] / descending aorta < 1.5

with skin area < 0.48 m2, provisional surgery (measured in systole).

Neonates with severe functional symptoms may undergo surgery at 3 months of age.

Children with few functional symptoms: follow up every 6 months, wait for surgery at 24 months of age.

Transposition of the great arteries

Cyanotic congenital heart disease.

In addition to the common foramen ovale and the ductus arteriosus immediately after birth, the disease can be simple or complex (with ventricular septal defect, with pulmonary stenosis).

Simple

The heart is always on the left side and is of the Situs solitus type.

Atrioventricular compatibility.

A pulmonary artery from the left ventricle, there is a continuum between the pulmonary artery - the mitral valve.

The aorta is located anteriorly and may be left or right relative to the pulmonary artery, sometimes the two arteries are parallel.

Complicated

It is a single type with one or more lesions: ventricular septal defect, pulmonary stenosis, coarctation of the aorta...

ductus arteriosus: immediately after birth with 2-way shunt a few days after an aortopulmonary shunt.

Foramen ovale: 2-way shunt, from the lung to the aorta at the end of systole, from the aorta to the lungs at the end of diastole.

Treatment:

Medical: Infusion of Prostaglandin E1 to keep the ductus arteriosus open is effective for several days.

Most neonates with a simple macroarterial regurgitation die within a few days.

The type with a ventricular septal defect: progressing as severe as the simple form, it is best to operate as soon as possible, so surgery before 1 year of age usually after 2 weeks of age.

The retrograde with ventricular septal defect and aortic stenosis: a case-by-case review

Right ventricular two ways out

Cyanotic congenital heart disease.

0.5% congenital heart.

The two great arteries are both from the right ventricle, or the equestrian aorta, which is more than 50% of the right ventricle, the aortic valve is located approximately as high as the pulmonary valve.

A ventricular septal defect is a persistent lesion, usually periventricular.

Pulmonary artery stenosis is usually funneled stenosis or both funnel and valve stenosis, depending on the location of the ventricular septal defect and whether there is pulmonary stenosis or not, people are divided into 3 different types.

Combined lesions are usually: abnormal anastomosis of the pulmonary or vena cava, atrioventricular canal, congenital mitral stenosis, no foramen Ao.

Newborns need temporary repair.

More than 6 months: comprehensive surgery if: two ventricles are of near-normal size, atrioventricular valves are of near-normal size and function, pulmonary artery network is of normal size, no multiple partial ventricular septal defects muscle.

Heart of one ventricle

Cyanotic congenital heart disease.

1% congenital heart.

There is always a coordinated lesion.

Complex form does not survive more than 6 months in 50% of patients.

Temporal anatomy: To regulate pulmonary flow, host flow.

When the ventricular function is good, mean pulmonary artery pressure is < 20 mmHg, and pulmonary vascular resistance is normal, comprehensive surgery is performed.

Arterial trunk

Cyanotic congenital heart disease.

1% congenital heart.

There is only one body that comes from the bottom of the heart.

There are three abnormalities: abnormalities of the heart, abnormalities of the valves of the common trunk, and abnormalities of the arteries from the common trunk.

Cardiac abnormalities: ventricular septal defect, fibrous continuity between the common stem valve and the mitral or both mitral and tricuspid valves, less commonly TMP abnormalities, no tricuspid orifice, atrial canal ventricular, single ventricle.

Common stem valve abnormalities: varying from 1-6 valves, narrow, open...

The arteries originate from the common trunk: Abnormally in the origin of the coronary arteries, the Ao arch can have 1 or 2 arcs, located right or left of the trachea and esophagus axis. Horizontal ponds can be atrophied, fed under the body through the ductus arteriosus. From pulmonary artery abnormalities, people are divided into many common body types.

Symptomatic treatment of congestive heart failure, 85% mortality in the first year.

Surgery can be done in infants, 2-3 months old, usually 3 years old, 2nd surgery.

Ebstein

Cyanotic congenital heart disease.

0.6% congenital heart.

Lying in right ventricular obstructive disease.

Injury to the tricuspid valve due to residual valve tissue, adhesion of the septal and inferior posterior leaflets to the wall of the right ventricle, distal to the atrioventricular annulus, mild forms occur in the septal leaflets, severe forms both septal and posterior inferior leaflets.

Blood stasis in the right atrium and atrioventricular chambers of the ventricles.

Ultrasound: The septal leaflet of the tricuspid compared with the mitral valve major: difference > 15 mm in children and > 20 mm in adults.

Newborns are not able to have surgery, treated with O2, diuretic, Digoxin.

Older children and adults: surgery is indicated when: severe cyanosis, heart failure, blood stasis or arrhythmia.

No pulmonary valve orifice with a ventricular septal defect

Cyanotic congenital heart disease.

0.2% congenital heart.

There is no continuity between the right ventricle and the pulmonary artery, which resembles an abnormal pulmonary venous connection.

Pulmonary artery body 3/4 cases atrophy, 1/4 is not seen. The right and left pulmonary arteries may or may not communicate.

Perfusion of the lungs by the ductus arteriosus, collateral artery, bronchial artery, or pleura.

Treatment of cradle with Digoxin, diuretic, vasodilator.

Central pulmonary artery size ≥ 50% of normal value can be radically operated.

Abnormal return of pulmonary veins

Cyanotic congenital heart disease.

More than 2% of congenital heart.

Half

Usually, the left pulmonary vein enters the left superior vena cava and the coronary sinus; The right pulmonary vein drains into the right superior vena cava, inferior vena cava, and right atrium.

The right pulmonary vein is more common.

Some cases are associated with an atrial septal defect.

Complete

Two-thirds of the abnormal pulmonary vein anastomosis is a solitary lesion, and one-third is a combined lesion with ventricular septal defect, T4F, right ventricular dual outlet.

The pulmonary veins collect into a collection tube that is then connected to structures above the heart, at the heart, or below the heart.

The left atrium and left ventricle are usually small but not atrophied.

Return of systemic venous abnormalities

May occur alone, but is often associated with other cardiac abnormalities.

Abnormalities of the vena cava entering the right atrium

Often there are no clinical manifestations or manifestations of associated lesions.

The left superior vena cava drains into the coronary sinus: 0.3% of the congenital heart.

The inferior vena cava connects to the right atrium through the azygos vein.

Abnormal connection of part of the vena cava to the right atrium

The left superior vena cava drains into the right atrium.

The right superior vena cava drains into the left atrium.

The inferior vena cava drains into the left atrium.

Entire vena cava to the left atrium (rare):

Abnormalities of the valves of the venous sinuses:

Valve Eustachi (lower vena cava), valve Thebesius (coronary sinus).

These valves do not degenerate to form a membrane.

No tricuspid valve hole

Cyanotic congenital heart disease.

3% congenital heart.

The tricuspid valve does not develop.

The right atrium wall is thick, the right atrium does not increase in size, 10% have transposition of the great arteries, atrial appendage abnormalities.

The atrial septal defect has many types.

Ventricular septal defect, the left ventricle is the main chamber, the right ventricle is the auxiliary chamber.

Adjuvant surgery to stabilize pulmonary flow in infants 3 months of age.

Surgical repair at 2-4 years of age with conditions: sinus rhythm, normal vena cava, normal-sized NP with good contractility, mean pulmonary artery pressure < 15mmHg, normal pulmonary valve ring, function Good left ventricle, no mitral regurgitation, and no stenosis of the pulmonary artery are acceptable.

Congenital heart disease in adults

Atrial communication

The secondary atrial septal defect occurs in only 7% of congenital hearts in children but up to 40% of congenital hearts in adults. There are 4 types of atrial septal defect: Primary 15%; Secondary 75%; Sinus venous 10%; Coronary sinus.

Complications of the atrial septal defect without surgery: pulmonary hypertension, right heart failure, atrial fibrillation, and cerebrovascular accident.

Small-hole atrial septal defect with QP/QS ratio <1.5 does not require surgery.

Surgery should be performed before age 25 and systolic pulmonary artery pressure ≤ 40 mmHg. The indication for surgery is still based on pulmonary artery pressure, pulmonary vascular resistance, and QP/QS.

50-60 years old with an atrial septal defect with the same pressure: pulmonary artery systolic pressure ~ 50mmHg, surgery still gives good results.

Ventilation

Rarely in adults, ventricular septal defect requires early surgery to avoid Eisenmenger.

Complications of ventricular septal defect are pulmonary hypertension, left heart failure, infective endocarditis, aortic regurgitation.

Indications for ventricular septal surgery are based on pulmonary artery pressure and left heart failure symptoms.

Adults with a small ventricular septal defect with normal pulmonary artery pressure and a QP/QS <1.3 do not need surgery. If in the range 1.3 - 1.5 need to consider each case. If QP/QS > 1.5 and pulmonary artery pressure ≤ 50 mmHg, surgery is required.

Adults with a large ventricular septal defect with a pulmonary artery pressure ≥70 mmHg but a low QP/QS due to high pulmonary vascular resistance greater than 7 units/m2 should not be operated on.

Patent ductus arteriosus

The ductus arteriosus requires surgery before pulmonary hypertension can lead to Eisenmenger syndrome.

Complications of ductus arteriosus include left heart failure, pulmonary hypertension, infective endocarditis, and calcification of the ductus arteriosus starting from the aorta.

All patients with a patent ductus arteriosus required surgery, except for the small ductus arteriosus with no murmur with normal pulmonary artery pressure.

Pulmonary valve stenosis

96% of patients with pulmonary stenosis are alive by age 25.

Complications of nonoperative pulmonary valve stenosis are ventricular arrhythmias, infective endocarditis, right heart failure, progressive worsening of the stenosis.

Indications for surgery for pulmonary valve stenosis are based on the right ventricular-pulmonary pressure gradient and functional symptoms.

In adults, moderate to severe pulmonary valve stenosis is less likely to require intervention, but if it is accompanied by right ventricular hypoplasia, early surgery is also required.

Aortic valve stenosis

Left ventricular outflow tract obstruction may be aortic, superior, or sub valve stenosis; In adults, 50% of aortic stenosis is due to congenital bivalve aortic stenosis.

Complications of nonoperative aortic stenosis: sudden death, ventricular arrhythmia, left heart failure.

Indications for surgery are based on functional symptoms (angina, exertion dyspnea, syncope) and degree of stenosis.

Surgery is needed when severe aortic stenosis or left ventricular-aortic pressure gradient ≥70 mmHg.

Coarctation of the aorta

90% stenosis at the tip of the descending aorta, immediately adjacent to the left subclavian artery. People distinguish aortic stenosis: anterior and posterior ductus arteriosus.

Complications: heart failure, aortic valve disease, rupture or dissection of the aorta near the stenosis...

To avoid missing coarctation of the aorta, all hypertensive patients < 50 years of age should be examined for coarctation of the aorta.

Internal treatment with prostaglandin E1 infusion.

Treatment with balloon dilatation is still preferred in infants and children younger than 1 year of age.

Indications for surgery sooner or later depending on the symptoms of heart failure.

In children without symptoms of heart failure, coarctation of the aorta is significant when the systolic pressure gradient is > 40 mmHg. The appropriate time of surgery is from 3rd to 12th.

Eisenmenger's syndrome

Congenital heart with left-right shunt should not be operated early, usually before 12 months of age with mechanical changes in the pulmonary vasculature. When mechanical pulmonary hypertension is increased, even with surgical occlusion of the shunt, pulmonary artery pressure does not decrease after surgery, at this time the flow is bidirectional or completely reversed, leading to heart failure. About 5% of atrial septal defects may develop Eisenmenger syndrome in adulthood.

Patients with Eisenmenger syndrome have symptoms around the age of 40: tricuspid regurgitation due to the dilated right ventricular annulus, heart failure, atrial arrhythmias. Patients may die from acute hypoxia or ventricular arrhythmias.

The only treatment is a heart-lung transplant or a single-lung transplant with the closure of the stoma.