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Congenital Heart Disease Prof. Prof. Pavlyshyn H.A. Pavlyshyn H.A.

Congenital Heart Disease Prof. Pavlyshyn H.A. Congenital heart disease (CHD) – general points is a defect in the structure of the heart andheart great

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Congenital Heart Disease

Prof. Pavlyshyn H.A.Prof. Pavlyshyn H.A.

Congenital heart disease (CHD) – general points

is a defect in the structure of the heart and great vessels which is present at birth

• CHD are the main cause of defect-related deaths • Incidence is 8-9/1000 live births• More common in premature infants• May be associated with a significant musculoskeletal defect(e.g. diaphragmatic hernia, exomphalos, tracheo-oesophageal fistula, imperforate anus)

Congenital heart disease (CHD)

• Causes are multifactorial and include maternal illness (diabetes mellitus, phenylketonuria, and systemic lupus erythematosus), maternal infections (Rubella), drugs (lithium, thalidomide), known teratogens, harmful habits (alcohol, hydantoin) and associations with chromosomal abnormality or other recognized patterns of malformation or syndrome;

Trisomy 18 100% have CHD

Evaluation of the newborn

• Cyanosis = blue color of skin and mucous membranes

caused by reduced oxygen content

• oxygen content of blood depends upon:

Hgb level

oxygen saturation

blood flow

• cyanosis usually noted when Sat’s <86%

• cyanosis more easily seen in polycythemia

• cyanosis more difficult to see in anemia

• Acrocyanosis vs. central cyanosis

Recognition of Cyanosis

Central cyanosis

• noted in the trunk, tongue, mucous membranes

• due to reduced oxygen saturation

Peripheral cyanosis

• noted in the hands and feet, around mouth

• due to reduced local blood flow

Recognition of Cyanosis

Differential cyanosis

1. pink upper, blue lower

CoA (Coarctation of the aorta), IAA (Interrupted aortic arch), Pulm Htn

2. blue upper, pink lower Transposition of the great vessels

d-TGA with pulm Htn dextro-Transposition of the great arteries

*indicates serious underlying cardiac or lung disease*

Recognition of Cyanosis

Chest Radiograph may be helpfulChest Radiograph may be helpful

The following lesions present with reduced pulmonary vascular markings

• • Tetralogy of Fallot (TOF)• • Pulmonary atresia • • Tricuspid atresia • • Critical pulmonary stenosis

The following lesions present with increased pulmonary vascular markings on chest X-ray

• • VSD, Transposition of the great arteries (TGA)• • Truncus arteriosus• • Total anomalous pulmonary venous drainage (TAPVD)

A 15-year-old girl with short stature, neck webbing, and sexual infantilism is found to have coarctation of the aorta.

A chromosomal analysis likely would demonstrate which of the following?

• a. Mutation at chromosome 15q21.1a. Mutation at chromosome 15q21.1

• b. Trisomy 21b. Trisomy 21

• c. XO karyotypec. XO karyotype

• d. Defect at chromosome 4p16d. Defect at chromosome 4p16

• e. Normal chromosome analysise. Normal chromosome analysis

Acyanotic Congenital Heart Disease↑ Pulmanary blood flow

Left-to-Right Shunt LesionsLeft-to-Right Shunt Lesions

• Patent Ductus Arteriosus (PDA)Patent Ductus Arteriosus (PDA)

• Atrial Septal Defect (ASD)Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)Ventricular Septal Defect (VSD)

• Atrioventricular Septal Defect (AV Canal)Atrioventricular Septal Defect (AV Canal)

Patent Ductus Arteriosus• PDAPDA – Persistence of the normal fetal vessel that joins the – Persistence of the normal fetal vessel that joins the

PA to the Aorta.PA to the Aorta.• Normally closes in the 1Normally closes in the 1stst wk of life. wk of life.

• Female:Male ratio of 2:1Female:Male ratio of 2:1

• Often associated w/ Often associated w/ coarctation & VSD.coarctation & VSD.

to R arm& head To L arm

MHMC PDA ligation

Patent Ductus Arteriosus

HemodynamicsHemodynamics

• As a result of higher As a result of higher aortic pressure, blood aortic pressure, blood shunts L to R through shunts L to R through the ductus from Aorta the ductus from Aorta to PA.to PA.

Patent Ductus Arteriosus

Clinical Signs & SymptomsClinical Signs & Symptoms

• tachycardiatachycardia• respiratory problems - respiratory problems -

shortness of breathshortness of breath• Poor growthPoor growth• Differential cyanosis - Differential cyanosis -

cyanosis of the lower extremities cyanosis of the lower extremities but not of the upper body.but not of the upper body.

Patent Ductus Arteriosus

Clinical Signs & SymptomsClinical Signs & Symptoms• Characteristic systolic-

diastolic murmur at the at the base of the heart with base of the heart with maximum in the PAmaximum in the PA

• It localized to the 2nd left It localized to the 2nd left intercostal space or radiate intercostal space or radiate down the left sternal border or down the left sternal border or to the left clavicle.to the left clavicle.

Patent Ductus Arteriosus

• ClassicClassic continuous continuous machine-like murmurmachine-like murmur

• It begins soon after onset of the 1st sound, It begins soon after onset of the 1st sound, reaches maximal intensity at the end of reaches maximal intensity at the end of systole, and wanes in late diastole. systole, and wanes in late diastole.

• prominent apical impulseprominent apical impulse enlarged heart, enlarged heart,

• Left subclavicular thrillLeft subclavicular thrill• Bounding pulseBounding pulse• WWidened pulse pressureidened pulse pressure

Clinical Signs & SymptomsClinical Signs & Symptoms

• pulmonary vascularity is increased;• • enlargement of left sided heart

Chest X-ray

Patent Ductus Arteriosus

TreatmentTreatment• Indomethacin - can be used in premature Indomethacin - can be used in premature

infants (0.2infants (0.2 mg/kg mg/kg))• Ibuprofen -10-5-5 mg\kgIbuprofen -10-5-5 mg\kg• PDA requires surgical or catheter closure.PDA requires surgical or catheter closure.• Closure is required treatment heart failure & Closure is required treatment heart failure &

to prevent pulmonary vascular disease.to prevent pulmonary vascular disease.

• Mortality is < 1%Mortality is < 1%

How can you characterize the PDA?

– Cyanotic CHD with R-to-L shuntCyanotic CHD with R-to-L shunt– Cyanotic CHD with mixed blood flowCyanotic CHD with mixed blood flow– Acyanotic CHD with R-to-L shuntAcyanotic CHD with R-to-L shunt– Acyanotic CHD with L-to-R shuntAcyanotic CHD with L-to-R shunt– Cyanotic CHD with Cyanotic CHD with ↓ Pulmonary blood flow↓ Pulmonary blood flow

Ventricular Septal Defect

• VSD VSD – is an abnormal opening in the – is an abnormal opening in the ventricular septum, which allows free ventricular septum, which allows free communication between the Rt & Lt communication between the Rt & Lt ventricles. ventricles.

• Accounts for 25% of CHD.Accounts for 25% of CHD.

Ventricular Septal Defect

• During systole some of the blood from the During systole some of the blood from the LV leaks into the RVLV leaks into the RV, , passes through the lungs and reenters the LV via the pulmonary veins passes through the lungs and reenters the LV via the pulmonary veins and LA.and LA.

• Such circuitous route of blood causes Such circuitous route of blood causes volume overload on the LVvolume overload on the LV. . • The LV normally has a much higher systolic pressure (~100 mm Hg) The LV normally has a much higher systolic pressure (~100 mm Hg)

than the RV (~85 mm Hg) and through VSD blood leaks into the RV than the RV (~85 mm Hg) and through VSD blood leaks into the RV and elevates RV pressure and volume, causing and elevates RV pressure and volume, causing Pulm HTNPulm HTN. .

• These changes lead to elevated RV & pulmonary pressures & volume These changes lead to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.hypertrophy of the LA & LV.

Ventricular Septal Defect

Clinical Signs & SymptomsClinical Signs & Symptoms

•• Small - moderate VSD, 3-6mm, are usually Small - moderate VSD, 3-6mm, are usually

asymptomatic.asymptomatic.

Small defects located predominantly in the muscular Small defects located predominantly in the muscular septum with slight hemodynamic impairment septum with slight hemodynamic impairment ((Tolochinov-RogeTolochinov-Roge disease) disease)

•• Moderate – large VSD, almost always have Moderate – large VSD, almost always have

symptoms and will require surgical repair.symptoms and will require surgical repair.

Ventricular Septal DefectClinical Signs & SymptomsClinical Signs & Symptoms

• If the defect is large and pulmonary vascular If the defect is large and pulmonary vascular resistance is not significantly elevated (L-t-R-shunt) resistance is not significantly elevated (L-t-R-shunt)

– – growth failure, CHF, repeated lower respiratory growth failure, CHF, repeated lower respiratory tract infectionstract infections (begin at 1-2month); (begin at 1-2month);

• If the defect is large and pulmonary vascular If the defect is large and pulmonary vascular resistance is very high (resistance is very high (Eisenmenger’s reactionEisenmenger’s reaction) – ) – shortness of breath, dyspnea on exertion, chest pain, shortness of breath, dyspnea on exertion, chest pain, cyanosis; cyanosis;

Listen at the back for radiation of murmurs

• Pansystolic/holosystolic murmurPansystolic/holosystolic murmur - loud, harsh, blowing - loud, harsh, blowing heard best over the LLSB, frequently is accompanied by thrill heard best over the LLSB, frequently is accompanied by thrill (depending upon the size of the defect) +/-(depending upon the size of the defect) +/-• mmore prominent with small VSD, may be absent with a very large ore prominent with small VSD, may be absent with a very large VSD.VSD.

Ventricular Septal Defect

Chest X-rayChest X-ray::• increased pulmonary increased pulmonary vascularity,vascularity,• enlargement of the enlargement of the LA and LV;LA and LV;

Ventricular Septal Defect

CXR: progressive dilatation of heartCXR: progressive dilatation of heart..

• cardiomegalycardiomegaly

• increased increased pulmonary pulmonary vascularity,vascularity,

• enlargement enlargement

of the of the LA and LVLA and LV;;

І

ІІ

ІІІ

AVR

AVL

AVF

V1

V2

V3

V4

V5

V6

ECG: overload of LV and RV

LA, LV or biventricular hypertrophy. RV hypertrophy predominates when pulmonary vascular resistance is high.

Ventricular Septal Defect

TreatmentTreatment

•• Small VSD - no surgical intervention, noSmall VSD - no surgical intervention, no physical restrictions;physical restrictions;• Bacterial endocarditis prophylaxis is indicated.Bacterial endocarditis prophylaxis is indicated.

•• Symptomatic VSD - Medical treatment Symptomatic VSD - Medical treatment initially with afterload reducers & diuretics. initially with afterload reducers & diuretics.

Atrial Septal Defect - ASD

• isis a form of CHD that enables blood flow between the left and a form of CHD that enables blood flow between the left and right atria via the interatrial septumright atria via the interatrial septum (it is possible for blood to (it is possible for blood to

travel from the left side to the right side of the hearttravel from the left side to the right side of the heart).). • Seen in 10% of all CHD.Seen in 10% of all CHD.

There are 3 major types:There are 3 major types:• Secundum ASDSecundum ASD

• Primum ASDPrimum ASD – – low in the septumlow in the septum

•• Sinus VenosusSinus Venosus ASDASD

Sinus Sinus VenosusVenosus

ASD with left-to-right shunt

In normal, the chambers of the left side of the heart are higher pressure than of the right side;

ASD with left-to-right shunt

• In the case of a large ASD (>9mm), may result in left-to-right shunt, blood will shunt from the LA to the RA.

• This extra blood may cause a volume overload of both the right atrium and the right ventricle.

• Ultimately the RV must push out more blood than the LV due to the L-to-R shunt. This condition can result in eventually RV-failure (dilatation and decreased systolic function) and Pulm Htn.

Atrial septal defect with left-to-right shunt

• When the pressure in the RA rises to the level in the LA, the left-to-right shunt will diminish or cease.

• When the pressure in the RA to be higher than the pressure in the LA and will reverse the shunt → right-to-left shunt will exist (this phenomenon is known as Eisenmenger’s syndrome).

Atrial Septal Defect

Clinical Signs & SymptomsClinical Signs & Symptoms

•• Most are asymptomatic, but may have easy Most are asymptomatic, but may have easy fatigability, mild growth failure, frequent fatigability, mild growth failure, frequent lower respiratory tract infection.lower respiratory tract infection.

•• Cyanosis does not occur unless Pulm HTN or Cyanosis does not occur unless Pulm HTN or R-to-L shunt is present.R-to-L shunt is present.

Feel the precordium for hyperactivityand for thrills

Physical findingPhysical finding

•• mild left mild left precordial bulgeprecordial bulge (hyperactive precordium);(hyperactive precordium);

• RV heave RV heave ((RV systolic liftRV systolic lift is palpable at the LSB);is palpable at the LSB);

Atrial Septal Defect

• Loud 1st heart soundLoud 1st heart sound, , sometimes pulmonic ejection click;sometimes pulmonic ejection click;• The The 2nd heart sound2nd heart sound is is widely split and fixedwidely split and fixed in all in all phases respiration;phases respiration;

Atrial Septal Defect

Listen carefully

Systolic ejection murmurSystolic ejection murmur – its medium pitched, seldom – its medium pitched, seldom accompanied by a thrill, and best heard at the LSB (left middle and accompanied by a thrill, and best heard at the LSB (left middle and upper sternal border); upper sternal border);

Short, rumbling mid-diastolic murmurShort, rumbling mid-diastolic murmur produced by the produced by the increased volume of blood flow across the tricuspid valve is often increased volume of blood flow across the tricuspid valve is often audible at the LLSB (lower left sternal border) .audible at the LLSB (lower left sternal border) .

Atrial Septal Defect

Diagnosis Diagnosis •• X-ray chest: pulmonary vascularity is increasedX-ray chest: pulmonary vascularity is increased

• ECG: ECG: right-axis deviation;right-axis deviation;

• Echo-CG: Echo-CG: RV is enlarged, defect is visualized;RV is enlarged, defect is visualized;

І

ІІ

ІІІ

AVR

AVL

AVF

V1

V2

V3

V4

V5

V6

ECG:ECG:

right-axis deviation, right-axis deviation, hypertrophy RV, RAhypertrophy RV, RA

Atrial Septal Defect

Treatment:Treatment:• Surgical or catheterization laboratory closure is Surgical or catheterization laboratory closure is

generally recommended for secundum ASD.generally recommended for secundum ASD.

•• Closure is performed electively between ages 2 & 5 yrs Closure is performed electively between ages 2 & 5 yrs to avoid late complications.to avoid late complications.

•• •• Mortality is < 1%.Mortality is < 1%.

• Coarctation of the AortaCoarctation of the Aorta

• Pulmonary StenosisPulmonary Stenosis

• Aortic StenosisAortic Stenosis

Acyanotic Congenital Heart Disease Obstruction to blood flow

from ventricles

Coarctation of the Aorta

• CoarctationCoarctation- is narrowing of the aorta at - is narrowing of the aorta at varying points anywhere from the varying points anywhere from the transverse arch to the iliac bifurcation.transverse arch to the iliac bifurcation.

• Male: Female ratio 3:1.Male: Female ratio 3:1.

• Accounts for 7 % of all CHD.Accounts for 7 % of all CHD.

Coarctation of the Aorta

HemodynamicsHemodynamics

• Obstruction of left ventricular outflow Obstruction of left ventricular outflow LV afterload increases LV afterload increases pressure pressure hypertrophy of the LV.hypertrophy of the LV.

Coarctation of the Aorta

Clinical Signs & SymptomsClinical Signs & Symptoms

• Higher BP in the upper extremities as Higher BP in the upper extremities as compared to the lower extremities.compared to the lower extremities.

• 90% have systolic hypertension of the 90% have systolic hypertension of the upper extremities.upper extremities.

Feel the pulsesespecially brachial and femoral

Clinical Signs & Clinical Signs & SymptomsSymptoms

• Classic signs of coarctation are diminution or absence of femoral pulses..

• Pulse discrepancy Pulse discrepancy between rt & lt arms.between rt & lt arms.

Coarctation of the Aorta

Coarctation of the AortaClinical Signs & SymptomsClinical Signs & Symptoms

• Sings of low cardiac output, poor peripheral perfusion - Sings of low cardiac output, poor peripheral perfusion - LELE hypoperfusion, acidosis, HF and shock.hypoperfusion, acidosis, HF and shock.

• Decreased and delayed pulses in lower extremities.Decreased and delayed pulses in lower extremities.

• Systolic ejection murmur @ LSB.Systolic ejection murmur @ LSB.

• Cardiomegaly, rib notching on X-rayCardiomegaly, rib notching on X-ray..

Coarctation of the Aorta

rib notching

Coarctation of the Aorta

TreatmentTreatment• With severe coarctation maintaining the ductus With severe coarctation maintaining the ductus

with prostaglandin E is essential.with prostaglandin E is essential.

• Surgical intervention, to prevent LV dysfunction.Surgical intervention, to prevent LV dysfunction.

• Angioplasty is used by some centers.Angioplasty is used by some centers.

• Balloon angioplasty is the procedure of choice.Balloon angioplasty is the procedure of choice.

Pulmonary Stenosis

• Pulmonary StenosisPulmonary Stenosis is obstruction in the region is obstruction in the region of either the pulmonary valve or the subpulmonary of either the pulmonary valve or the subpulmonary ventricular outflow tract.ventricular outflow tract.

• Accounts for 7-10% of all CHD.Accounts for 7-10% of all CHD.

• Most cases are Most cases are isolated lesionsisolated lesions

Pulmonary Stenosis

HemodynamicsHemodynamics

• RV pressure hypertrophy RV pressure hypertrophy RV failure. RV failure.• RV pressures maybe > systemic pressure.RV pressures maybe > systemic pressure.• Post-stenotic dilation of main PA.Post-stenotic dilation of main PA.• W/intact septum & severe stenosis W/intact septum & severe stenosis R-L R-L

shunt through FO shunt through FO cyanosis. cyanosis.• Cyanosis is indicative of Critical PS.Cyanosis is indicative of Critical PS.

Pulmonary StenosisClinical Signs & SymptomsClinical Signs & Symptoms

• Depends on the severity of obstruction.Depends on the severity of obstruction.• Asymptomatic w/ mild PS < 30mmHg.Asymptomatic w/ mild PS < 30mmHg.• Mod-severe: 30-60mmHg, > 60mmHgMod-severe: 30-60mmHg, > 60mmHg• Prominent jugular a-wave Prominent jugular a-wave • RV lift, RV heaveRV lift, RV heave• Split 2Split 2ndnd hrt sound hrt sound• Ejection click, followed by systolic murmur.Ejection click, followed by systolic murmur.• Heart failure & cyanosis not relieved by inhaled Heart failure & cyanosis not relieved by inhaled

oxygen seen in severe cases.oxygen seen in severe cases.

Pulmonary Stenosis

TreatmentTreatment

• Balloon valvuloplasty, treatment of choice.Balloon valvuloplasty, treatment of choice.

• Surgical valvotomy is also a consideration.Surgical valvotomy is also a consideration.

Cyanotic Congenital Heart Disease

•Severe CyanosisSevere Cyanosis PaO PaO22 ≤ 40 mmHg ≤ 40 mmHg

• Obstruction to RV outflow causes intracardiac R-to-L shuntingObstruction to RV outflow causes intracardiac R-to-L shunting

• Complex anatomic defects cause an admixture of pulmonary and Complex anatomic defects cause an admixture of pulmonary and systemic venous return in the heartsystemic venous return in the heart

• Decreased pulmonary blood flowDecreased pulmonary blood flow

– Tricuspid atresia, intact ventricular septumTricuspid atresia, intact ventricular septum

– Critical pulmonary stenosisCritical pulmonary stenosis

– Ebstein’s anomalyEbstein’s anomaly

– Tetralogy of Fallot*Tetralogy of Fallot*

• Chest X-RayChest X-Ray

Decreased pulmonary vascular markingsDecreased pulmonary vascular markings

“ “Boot-shaped heart” in Tetralogy of FallotBoot-shaped heart” in Tetralogy of Fallot

Right sided obstruction

1. Obstruction of RV outflow (Pulmonary stenosis);

2. VSD;

3. Dextroposition of the aorta with override of the ventricular septum;

4. RV hypertrophy

Tetralogy of Fallot

Assessment Findings with Tetralogy of Fallot

Symptoms are variable depending of degree of obstructionSymptoms are variable depending of degree of obstruction

• Cyanosis – is variable (isn’t present at the birth, occurs later in Cyanosis – is variable (isn’t present at the birth, occurs later in the 1the 1stst yr of life) yr of life)

• Digital clubbing and hyperpnea at rest are directly related to the Digital clubbing and hyperpnea at rest are directly related to the degree of cyanosisdegree of cyanosis

• TachycardiaTachycardia• Mental retardationMental retardation• Retarded growth and developmentRetarded growth and development• RV heaveRV heave• Systolic ejection murmur Systolic ejection murmur is heard along the left sternal borderis heard along the left sternal border

Assessment Findings with Tetralogy of Fallot

• Paroxymal dyspneaParoxymal dyspnea• Severe dyspnea on exertionSevere dyspnea on exertion

• Squatting position for the relief of dyspnea caused Squatting position for the relief of dyspnea caused physical effort, physical effort,

• “ “Blue” spells, “tet” spells, paroxysmal Blue” spells, “tet” spells, paroxysmal hypercyanotic attacks – infant becomes hyperpnea, hypercyanotic attacks – infant becomes hyperpnea, restless, cyanosis increases, gasping respirations, restless, cyanosis increases, gasping respirations, syncope syncope

Hypercyanotic Spells/Blue Spells/Tet Spells

Clinical Manifestations

,Most often occurs in morning after feedings ٭defecation, or crying

Acute cyanosis ٭

Hyperpnea ٭

Inconsolable crying ٭

Hypoxia which leads to acidosis ٭

Chest X-RayChest X-Ray

• Decreased Decreased pulmonary vascular pulmonary vascular markingmarking

• “ “Boot-shaped Boot-shaped heart”heart”

Treatment of the Child with TOF

• Decrease cardiac workloadDecrease cardiac workload

• Prevention of intercurrent infectionPrevention of intercurrent infection

• Prevention of hemoconcentrationPrevention of hemoconcentration

• Surgical repair – palliative or corrective Surgical repair – palliative or corrective surgerysurgery

Balloon Dilatation of Pulmonic Valve

d-Transposition of the Great Arteries

• PathophysiologyPathophysiology– Cyanosis due to failure of delivery of pulmonary Cyanosis due to failure of delivery of pulmonary

venous blood to the systemic circulationvenous blood to the systemic circulation– Two parallel circulations with no mixingTwo parallel circulations with no mixing– Open atrial septum (fossa ovalis) allows some left-to-Open atrial septum (fossa ovalis) allows some left-to-

right shunt, enhanced by a left-to-right ductus right shunt, enhanced by a left-to-right ductus arteriosus shuntarteriosus shunt

– Presence of ventricular septal defect facilitates mixingPresence of ventricular septal defect facilitates mixing

d-Transposition of the Great Arteries

Transposition of the Great Arteries

• Aorta from right ventricle, pulmonary Aorta from right ventricle, pulmonary artery from left ventricle.artery from left ventricle.

• Cyanosis from birth, hypoxic spells Cyanosis from birth, hypoxic spells sometimes present.sometimes present.

• Heart failure often present.Heart failure often present.

• Cardiac enlargement and diminished Cardiac enlargement and diminished pulmonary artery segment on x-ray.pulmonary artery segment on x-ray.

Transposition of the Great Arteries

• Anatomic communication must exist Anatomic communication must exist between pulmonary and systemic between pulmonary and systemic circulation, VSD, ASD, or PDA.circulation, VSD, ASD, or PDA.

• Untreated, the vast majority of these infants Untreated, the vast majority of these infants would not survive the neonatal period.would not survive the neonatal period.

Transposition of the Great Arteries Clinical Manifestations

• Cyanosis, tachypnea Cyanosis, tachypnea are most often recognized within are most often recognized within the 1st hrs or days of life.the 1st hrs or days of life.

• Hypoxemia Hypoxemia is usually moderate to severe, depending on is usually moderate to severe, depending on the degree of atrial level shunting and whether the ductus the degree of atrial level shunting and whether the ductus is partially open or totally closed. is partially open or totally closed.

• Physical findings, other than cyanosis, may be remarkably Physical findings, other than cyanosis, may be remarkably nonspecific. nonspecific.

• Murmurs may be absentMurmurs may be absent, or a soft systolic ejection , or a soft systolic ejection murmur may be noted at the midleft sternal border. murmur may be noted at the midleft sternal border.

• Chest filmChest film– Oval-shaped heartOval-shaped heart– Narrow mediastinumNarrow mediastinum– Normal or increased pulmonary vascular markingsNormal or increased pulmonary vascular markings

d-Transposition of the Great Arteries

D-Transposition of the Great Arteries

This condition is a medical emergency, and only early diagnosis and appropriate intervention can avert the

development of prolonged severe hypoxemia and acidosis, which lead to death

Treatment

When transposition is suspected, an infusion of prostaglandin E1 should be initiated immediately to maintain patency of the ductus arteriosus and improve oxygenation. Endotracheal intubation

Infants who remain severely hypoxic or acidotic despite prostaglandin infusion should undergo Rashkind balloon atrial septostomy A Rashkind atrial septostomy is also usually performed in all patients in whom any significant delay in surgery is necessary.

Preventing Birth Defects

• Stop smoking Stop smoking • Avoid drinking alcohol while pregnant Avoid drinking alcohol while pregnant • Take a daily vitamin containing folic acid Take a daily vitamin containing folic acid • Check with your doctor to make sure any medication Check with your doctor to make sure any medication

(over-the-counter or prescription) is safe to take during (over-the-counter or prescription) is safe to take during pregnancy pregnancy

• Stop use of any illegal or "street" drugsStop use of any illegal or "street" drugs

Thank you for attention!