CHD

Answer 1

Occur during the 1 st 8 weeks of fetal development.
during the 1 st 8 weeks of fetal development.
q Majority have unknown cause.

Question 2

critical CHD

Answer 2

critical CHD may present with symptoms of

cyanosis, CHF, poor pedal pulses, or a failed newborn CHD pulse oximeter prior to discharge.

Question 3

q It may be challenging to distinguish cardiac disease from

pulmonary disease or sepsis in a newborn with cyanosis and/or respiratory distress.

Answer 3

Chest radiography and assessment of
response to oxygen and/or positive
pressure ventilation as clinically indicated
help to elucidate the cause.
q The pulse oximetry screen for critical CHD
in newborns was approved as a part of the
routine universal screening program in
2011.

Question 4

SPECIFIC TYPES OF CYANOSIS

Answer 4

Di fferential Cyanosis (foot more blue)
PDA

Reversed differential Cyanosis (hands more blue)
d TGA + PDA + PHTN

Intermittent Cyanosis

Ebestein’s anomalie

Question 5

The transformation from fetal to neonatal circulation involves two major changes:

Answer 5

The transformation from fetal to neonatal circulation involves two major changes:
1. A marked increase in
systemic resistance caused by loss of the low-resistance placenta.
2. A marked decrease in
pulmonary resistance caused by pulmonary artery dilation with the neonate’s first breaths.

Question 6

ASD

Answer 6

AUtosomal dominant if familiar

Maybe sporadic

Question 7

Osteium second ASD

Answer 7

Holt oram sundrome
Trisomy 18
Klienfilter

Question 8

ATRIAL SEPTAL DEFECT (ASD)

Answer 8

v Usually asymptomatic and incidental finding heart
murmur is the usual presenting symptom. v1% develop heart failure in first year of life. vClinical heart failure depended on magnitude of the left
to right shunt. v Physical examination:
Ø Increased right ventricular impulse. Ø Systolic ejection murmur at left upper sternal
border (Relative PS). Ø Widely fixed Split second heart sound (prolong
ejection time).

Question 9

ATRIAL SEPTAL DEFECT (ASD

Answer 9

Electrocardiographs (ECGs) from a child with a secundum atrial septal defect (ASD). Note the right-axis deviation and rSR’ pattern in lead V1.
heart with v Electrocardiography (ECG) most commonly demonstrates
right-axis deviation, right ventricular hypertrophy, and an
prominent vascularity
rSR’ or rsR’ pattern in the right precordial leads. v The QRS duration is usually normal. v However, the ECG may be normal, especially in infants and in
young children with small defects.

v Electrocardiogram (ECG)
from a child with a primum
atrial septal defect (ASD): Ø left-axis deviation with a
counterclockwise vector of depolarization (small q waves in leads I and aVL).
Ø Right ventricular
hypertrophy. Ø Volume overload (rSR'
pattern and upright T wave
in lead V1).

Question 10

مهم

Answer 10

With the exception of ostium secundum types; atrial septal defects are structural defects that do not spontaneously close and needs surgical closure
Medical therapy is of no benefit in children with asymptomatic atrial septal defects (ASDs)

Question 11

ATRIAL SEPTAL DEFECT (ASD)
v Definitive therapy for an atrial septal defect (ASD) has historically
been limited to surgical closure.

Answer 11

ATRIAL SEPTAL DEFECT (ASD)
v Definitive therapy for an atrial septal defect (ASD) has historically
been limited to surgical closure.
v However, with the advent of transcatheter techniques, many children
undergo successful treatment in the cardiac catheterization laboratory. v Surgery is ideally performed in children aged 2- 4 years and has a very
low mortality rate (However, surgery may be performed earlier than this
if the child has evidence of CHF).

Question 12

VENTRICULAR SEPTAL DEFECT (VSD)

Answer 12

An isolated VSD occurs in approximately
2-6 of every 1000 live births and accounts for
more than 20% of all congenital heart
diseases. v After bicuspid aortic valves, VSDs are the
most commonly encountered congenital
heart defect. v The symptoms and physical findings
associated with VSD depend on the size of
the defect and magnitude of the left to right
shunt, which, in turn, depends on the relative
resistances of the systemic and pulmonary
circulation. v Echocardiography is the diagnostic imaging
modality of choice.

Question 13

Eisenmenger’s complex of right to left shunting may occur as the elevated right-sided pressures (pulmonary hypertension) exceed left-sided pressures

Answer 13

1-cyanosis
2-polycythemia’
3-paradoxical embolism

Question 14

VENTRICULAR SEPTAL DEFECT (VSD)

Types

Answer 14

Subarterial VSDs can be
classified as abnormalities of
ectomesenchymal tissue
migration. 2. Perimembranous VSDs can
be classified as abnormalities
of intracardiac blood flow. 3. Muscular VSDs can be
classified as abnormalities in
cell death. 4. Inlet VSDs can be classified
as abnormalities of the
extracellular matrix and
defects in the endocardial
cushion.

Question 15

VSD

Answer 15

Harsh holosystolic murmur left LSB

Small defect can produce high pitched or squeaky noise

Question 16

Large VSD

Answer 16

Cariomegaly loud ءsingle second hreat sound characteristics
Uper left sternal border

Question 17

Large VSD ECG

Answer 17

Signs of biventricular
hypertrophy and widespread high
voltages of QRS, especially in the
precordial leads. 
Ø These large biphasic QRS complexes
from V2–V5 (symmetric R/S in the
mid-precordial leads) are called
Katz-Wachtel sign.

Question 18

VENTRICULAR SEPTAL DEFECT (VSD)

v Spontaneous closure

Answer 18

v Spontaneous closure  frequently occurs in
children, usually by age 2 years.
vClosure is uncommon after age 4 years. vClosure is most frequently observed in
muscular defects (80%), followed by
perimembranous defects (35-40%).
vOutlet VSDs have a low incidence of
spontaneous closure, and inlet VSDs do
not close.

Answer 19

Prolapse of an aortic valve cusp is an
infection is an indication for
indication for surgery even if the VSD is surgical repair.
small.

Question 20

Therapies used to manage symptomatic
congestive heart failure (CHF) in
children with moderate or large
ventricular septal defects (VSDs) may
include the following:

Answer 20

Therapies used to manage symptomatic
congestive heart failure (CHF) in
children with moderate or large
ventricular septal defects (VSDs) may
include the following:
Ø Increased caloric density of
feedings to ensure adequate weight
gain. Ø Diuretics (eg, furosemide) to relieve
pulmonary congestion. Ø Angiotensin-converting enzyme
(ACE) inhibitors (eg, captopril and
enalapril). Ø Digoxin (5-10 µg/kg/d).

Question 21

AVSD

Answer 21

v Irreversible pulmonary vascular disease may be present by age 2 years or, in
rare cases, earlier.
v The pulmonary vascular disease may occur earlier in infants with Down
syndrome.

Question 22

AVSD exam

Answer 22

Down
Failure to thrive
Harrison sulcus = croninc tachy

Question 23

Eisenmenger

Answer 23

VSD PDA

Question 24

PATENT DUCTUS ARTERIOSUS (PDA)

Answer 24

Depending on the size of the patent ductus arteriosus (PDA), the
gestational age of the neonate, and the pulmonary vascular resistance
(PVR), a premature neonate may develop life-threatening pulmonary
overcirculation in the first few days of life. q Conversely, an adult with a small patent ductus arteriosus (PDA) may
present with a newly discovered murmur well after adolescence. q Patients can present at any age. q The typical child with a patent ductus arteriosus (PDA) is
asymptomatic. q If the left-to-right shunt is large, precordial activity is increased. q The apical impulse is laterally displaced; a thrill may be present in the
suprasternal notch or in the left infraclavicular region .

Question 25

Coarctation of the Aorta

Answer 25

Coarctation of the aorta may occur as an isolated defect or in association with various other lesions, most commonly bicuspid aortic valve and VSD.

Question 26

Coarctation of the Aorta

Answer 26

``` The classic coarctation of the aorta is located in the thoracic aorta distal to the origin of the left subclavian artery at about the level of the ductal structure. v However, rarely, a coarcted segment is present in the lower thoracic or abdominal aorta (in such instances, the coarcted segment may be long and fusiform with irregular lumen; many consider these to be inflammatory or autoimmune in origin, and they may be variants of Takayasu arteritis). ```

Question 27

Coarctation of the Aorta

Answer 27

Coarctation of the Aorta v The most commonly associated clinically significant defects include PDA, VSD, and aortic stenosis. v The earlier the infant presents, the more likely a significant associated defect is present. v Bicuspid aortic valve may be seen in nearly two thirds of infants with coarctation of the aorta, whereas only 30% of those who present in childhood have such an anomaly. v Mitral valve anomalies, although less common than those of the aortic valve, are also associated with coarctation of the aorta. v Sometimes, coarctation of the aorta is a complicating feature of a more complex cyanotic heart defect, such as TGA, Taussig-Bing anomaly, double-inlet left ventricle, Tricuspid atresia with transposition of the great arteries, and HLHS. v Aortic coarctation is extremely rare in patients with severe right ventricular outflow tract obstructions such as TOF and PA/VSD. v Some patients with coarctation of the aorta may have cerebral aneurysms, predisposing them to cerebrovascular accidents with severe hypertension later in life. Coarctation of the aorta is the most common cardiac defect associated with Turner syndrome.

Question 28

Coarctation of the Aorta

Answer 28

Sometimes, coarctation of the aorta is a complicating feature of a more complex cyanotic heart defect, such as TGA, Taussig-Bing anomaly, double-inlet left ventricle, Tricuspid atresia with transposition of the great arteries, and HLHS.

Question 29

Coarctation of the Aorta Symptoms of aortic coarctation The following physical findings may be noted: may include the following:

Answer 29

``` The diagnosis of coarctation generally can be made on the basis of physical examination. Blood pressure differential and pulse delay are pathognomonic. ```

Question 30

CoA

Answer 30

Coa =collateral =continuos murmur in back

Question 31

CoA murmur

Answer 31

Characteristic murmurs and sounds on auscultation (eg, continuous or late systolic murmur posteriorly over the thoracic spine, bilateral collateral arterial murmurs, aortiic ejection sound, short midsystolic murmur, or early diastolic may be seen. murmur of aortic regurgitation).

Question 32

PE

Answer 32

Extracardiac vascular anomalies (eg, aberrant subclavian artery, berry aneurysms of the circle of Willis, development of large upper-to-lower collateral arteries, or hemangiomas). v Extracardiac nonvascular anomalies (eg, head and neck, musculoskeletal, gastrointestinal, genitourinary, or respiratory). Extracardiac vascular anomalies (eg, aberrant subclavian artery, berry aneurysms of the circle of Willis, development of large upper-to-lower collateral arteries, or hemangiomas). v Extracardiac nonvascular anomalies (eg, head and neck, musculoskeletal, gastrointestinal, genitourinary, or respiratory).

Question 33

X-ray of CoA

Answer 33

1-rib notch #clollatral | 2-aoric knob then acutal coractation the substenotic dilatic descendind aorta

Question 34

Medical tt of CoA

Answer 34

Neonatate - intubation - PGE1 - correction of acidosis - inotropic support CHF =less sever +bynod neonatal period 1-digoxin diuretic Postpon surgery untill hemodynamic stable

Question 35

TETROLOGY OF FALLOT (TOF)

Answer 35

Most common cyanotic CHD. q 9 % of total congenital heart disease. q 4 Common defects: Ø Ventricular septal defect Ø Pulmonary stenosis Ø Overriding of aorta Ø Right ventricular hypertrophy. q Early clinical presentation: Ø A heart murmur in infancy and progressive cyanosis (from right to left shunting at the ventricular level secondary to RVOTO). Ø Unoperated tetralogy carries a poor prognosis ( 95% of patients used to die before 40 years of age). q Late clinical presentation: Ø Late survival after tetralogy repair is excellent, with a 35-year of survival 85%.

Question 36

Cyanoyic spell / tet spell

Answer 36

TOF

Question 37

Single S2

Answer 37

Single s2 = overriding aorta = TOF

Question 38

TETROLOGY OF FALLOT (TOF)

Answer 38

§ Boot shape heart § Apex shift upward with pulmonary trunk indentation § Decreased pulmonary vascular marking. Usually Right ventricular hypertrophy.

Question 39

TETROLOGY OF FALLOT (TOF) | q Hypoxic Spells (Emergency condition):

Answer 39

Hypoxic Spells (Emergency condition): Ø Knee-Chest Position Ø Control Crying Ø Oxygen supplement Ø Morphine 0.1 mg/kg (Sedate and reduce infundibular spasm) Ø IV Hydration and correct metabolic acidosis Ø +/- drug for increased systemic vascular resistance Ø Propranolol 1-4 mg/kg/day q 6-8 hrs Ø Correct Anemia Ø Surgery consideration.

Question 40

TRANSPOSITION OF GREAT ARTERIES (TGA)

Answer 40

Ventriculo- arterial discordance: Ø Left ventricle to pulmonary artery Ø Right ventricle to aorta.

Question 41

مهم انواع TGA صورة

Question 42

TRANSPOSITION OF GREAT ARTERIES (TGA) | q Severe Cyanosis:

Answer 42

``` Severe Cyanosis: Ø PGE1: dose 0.05– 0.1 mcg/kg/min for maintain ductus arteriosus. Ø Correct metabolic acidosis and electrolytes. Ø If persistent cyanosis: ü Pulmonary banding -> For Left ventricle training. ü Arterial switch after 7-10 days of pulmonary banding. § Atrial balloon septostomy then consider arterial switch operation that should perform within 2 weeks. § If more than 2 weeks -> Two- stage arterial switch operation: ```

Question 43

TGA surgery

Answer 43

arterial switch TGA with VSD and PS: Ø modified Blalock–Taussig shunt then considered definite surgery. Ø if body weight 10-15 kgs Ratelli Operation (Closed VSD and used conduit bridging between right ventricle and pulmonary artery).

Question 44

TRUNCUS ARTERIOSUS | CHF>cyanosis

Answer 44

``` Patients with truncus arteriosus rarely present with cyanosis but it may occur in very young neonates in whom pulmonary vascular resistance remains elevated. q Symptoms and signs of congestive heart failure are more common findings than cyanosis in patients presenting early in life. With progressively increasing pulmonary blood flow and, consequently, myocardial work, the initial symptoms of congestive heart failure (eg, poor feeding, diaphoresis, mild lethargy) become more evident as failure to thrive ensues. ```

Question 45

TRICUSPID ATRESIA

Answer 45

``` Tricuspid atresia (TA) is a cyanotic congenital heart defect characterized by the complete agenesis of the tricuspid valve (TV). q There are several subtypes of this disease with varied clinical presentations based on the degree of pulmonary blood flow. q It carries a very high mortality rate if there is no intervention during the first year of life. q Tricuspid atresia is the third most common cyanotic congenital heart disease with a prevalence of around 1.2 per 10000 live births (0.3 – 3.7 % in patients with congenital heart disease). ```

Question 46

TRICUSPID ATRESIA

Answer 46

q cyanosis is the most common clinical feature of this lesion. q The degree of cyanosis depends on the degree of pulmonary blood flow. q Infants with associated diminished pulmonary blood flow or infants who depend on a patent ductus arteriosus manifest pronounced cyanosis that worsens as the ductus begins to close. q Patients with relatively normal or increased pulmonary blood flow manifest little cyanosis but more pronounced congestive heart failure. q Digital clubbing is common in infants older than 3 months. q Jugular venous pulsations and distention are common.

Question 47

TRICUSPID ATRESIA

Answer 47

electrocardiogram usually shows left axis deviation (very unlike the RVH seen in normal newborns) and right atrial hypertrophy and left-ventricular hypertrophy. Chest x-ray may show increased or decreased pulmonary blood flow depending on the shunt and a normal or mildly increased heart siz

Question 48

TRICUSPID ATRESIA | 3 steps op

Answer 48

``` Modified BlalockTaussig shunt: Ø to maintain pulmonay blood flow by placing a Gore-Tex conduit between the subclavian artery and the pulmonary artery. q Cavopulmonary anastomosis: Ø (bidirectional Glenn) to provide stable pulmonary flow. q Fontan procedure: Ø redirect inferior vena cava and hepatic vein flow into the pulmonary circulation. ```

Question 49

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)

Answer 49

TAPVC defines as the anomaly in which the pulmonary veins have no connection with the left atrium. q Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium. q PFO or ASD is present essentially in those who survive after birth.

Question 50

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)

Answer 50

Type I: Supracardiac connections : § 45% of TAPVC patients. § The common venous confluence joins SVC. q Type II: Cardiac TAPVC : § It accounts for 25% of cases. § CVC drains into coronary sinus or directly into RA. q Type III: Infracardiac TAPVC: § Approximately 21% of cases. § CVC drains into hepatic vein, ductus venosus, portal vein or IVC. § The common pulmonary vein penetrates the diaphragm through the esophageal hiatus. q Type IV: Mixed Type: § It accounts for <10% of cases.

Question 51

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)

Answer 51

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC) v Infants presenting with obstructed TAPVC represent surgical emergency. v They need intensive resuscitation before going for definitive surgery. v Non-obstructed TAPVC patient are relatively stable and can be taken for elective corrective surgery within few days of diagnosis irrespective of patients age and weight.

Question 52

EBSTEIN ANOMALY

Answer 52

``` Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. ```

Question 53

EBSTEIN ANOMALY

Answer 53

Treatment options include: Ø Diuretics (eg, furosemide) Ø medical therapy. Ø Cardiac glycosides (eg, digoxin) Ø radiofrequency ablation. Ø Angiotensin-converting enzyme inhibitors (eg, enalapril). Ø and surgical procedures. v Radiofrequency ablation of the accessory pathways is an alternative to medication for treatment of

Question 54

HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

Answer 54

HYPOPLASTIC LEFT HEART SYNDROME (HLHS) v The term hypoplastic left heart syndrome (HLHS), initially proposed by Noonan and Nadas, describes a spectrum of cardiac abnormalities characterized by marked hypoplasia of the left ventricle and ascending aorta. v The aortic and mitral valves are atretic, hypoplastic, or stenotic. v A patent foramen ovale or an atrial septal defect is usually present. vCoarctation of the aorta is also commonly present. vThe ventricular septum is usually intact. vA large patent ductus arteriosus supplies blood to the systemic circulation. vSystemic arterial desaturation may be present because of complete mixing of pulmonary and systemic venous blood in the right atrium.

Question 55

Normal Sex development and diferentiation

Answer 55

• Four major steps which consttute normal sexual diferentaton ① Fertlizaton and determinaton of genetc sex ② Formaton of organs common to both sexes ③ Gonadal diferentaton ④ Diferentaton of the internal ducts and external genitalia