CHD Flashcards
Occur during the 1 st 8 weeks of fetal development.
during the 1 st 8 weeks of fetal development.
q Majority have unknown cause.
critical CHD
critical CHD may present with symptoms of
cyanosis, CHF, poor pedal pulses, or a failed newborn CHD pulse oximeter prior to discharge.
q It may be challenging to distinguish cardiac disease from
pulmonary disease or sepsis in a newborn with cyanosis and/or respiratory distress.
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.
SPECIFIC TYPES OF CYANOSIS
Di fferential Cyanosis (foot more blue)
PDA
Reversed differential Cyanosis (hands more blue)
d TGA + PDA + PHTN
Intermittent Cyanosis
Ebestein’s anomalie
The transformation from fetal to neonatal circulation involves two major changes:
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.
ASD
AUtosomal dominant if familiar
Maybe sporadic
Osteium second ASD
Holt oram sundrome
Trisomy 18
Klienfilter
ATRIAL SEPTAL DEFECT (ASD)
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).
ATRIAL SEPTAL DEFECT (ASD
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).
مهم
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)
ATRIAL SEPTAL DEFECT (ASD)
v Definitive therapy for an atrial septal defect (ASD) has historically
been limited to surgical closure.
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).
VENTRICULAR SEPTAL DEFECT (VSD)
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.
Eisenmenger’s complex of right to left shunting may occur as the elevated right-sided pressures (pulmonary hypertension) exceed left-sided pressures
1-cyanosis
2-polycythemia’
3-paradoxical embolism
VENTRICULAR SEPTAL DEFECT (VSD)
Types
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.
VSD
Harsh holosystolic murmur left LSB
Small defect can produce high pitched or squeaky noise
Large VSD
Cariomegaly loud ءsingle second hreat sound characteristics
Uper left sternal border
Large VSD ECG
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.
VENTRICULAR SEPTAL DEFECT (VSD)
v Spontaneous closure
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.
Prolapse of an aortic valve cusp is an
infection is an indication for
indication for surgery even if the VSD is surgical repair.
small.
Therapies used to manage symptomatic congestive heart failure (CHF) in children with moderate or large ventricular septal defects (VSDs) may include the following:
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).
AVSD
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.
AVSD exam
Down
Failure to thrive
Harrison sulcus = croninc tachy
Eisenmenger
VSD PDA
PATENT DUCTUS ARTERIOSUS (PDA)
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 .
Coarctation of the Aorta
Coarctation of the aorta may occur as an isolated
defect or in association with various other lesions,
most commonly bicuspid aortic valve and VSD.
Coarctation of the Aorta
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).
Coarctation of the Aorta
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.
Coarctation of the Aorta
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.
Coarctation of the Aorta
Symptoms of aortic coarctation
The following physical findings may be noted:
may include the following:
The diagnosis of coarctation generally can be made on the basis of physical examination. Blood pressure differential and pulse delay are pathognomonic.
CoA
Coa =collateral =continuos murmur in back
CoA murmur
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).
PE
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).
X-ray of CoA
1-rib notch #clollatral
2-aoric knob then acutal coractation the substenotic dilatic descendind aorta
Medical tt of CoA
Neonatate
- intubation
- PGE1
- correction of acidosis
- inotropic support CHF
=less sever +bynod neonatal period
1-digoxin diuretic
Postpon surgery untill hemodynamic stable
TETROLOGY OF FALLOT (TOF)
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%.
Cyanoyic spell / tet spell
TOF
Single S2
Single s2 = overriding aorta = TOF
TETROLOGY OF FALLOT (TOF)
§ Boot shape heart § Apex shift upward with
pulmonary trunk indentation § Decreased pulmonary vascular
marking.
Usually Right ventricular hypertrophy.
TETROLOGY OF FALLOT (TOF)
q Hypoxic Spells (Emergency condition):
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.
TRANSPOSITION OF GREAT ARTERIES (TGA)
Ventriculo- arterial discordance:
Ø Left ventricle to pulmonary artery
Ø Right ventricle to aorta.
مهم انواع TGA صورة
TRANSPOSITION OF GREAT ARTERIES (TGA)
q Severe Cyanosis:
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:
TGA surgery
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).
TRUNCUS ARTERIOSUS
CHF>cyanosis
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.
TRICUSPID ATRESIA
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).
TRICUSPID ATRESIA
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.
TRICUSPID ATRESIA
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
TRICUSPID ATRESIA
3 steps op
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.
TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)
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.
TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)
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.
TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVC)
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.
EBSTEIN ANOMALY
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.
EBSTEIN ANOMALY
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
HYPOPLASTIC LEFT HEART SYNDROME (HLHS)
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.
Normal Sex development and diferentiation
• 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
