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PEDIATRICS CONFERENCE
PATHOLOGY
OF
CONGENITAL HEART DISEASE
Boonyathee Sorawit MD.
Mar 25th , 2014
Epidemiology
 Congenital heart disease occurs in 0.5–0.8% of live births.
 Incidence is higher in stillborns (3–4%), spontaneous abortuses
(10–25%) and premature infants (about 2% excluding patent
ductus arteriosus [PDA])
 About 2–3 in 1,000 newborn infants will be symptomatic with
heart disease in the 1st year of life.
 The diagnosis is established by 1 week of age in 40–50% of
patients with congenital heart disease and by 1 month of age in
50–60% of patients.
Etiology
 Caused by developmental abnormalities
 Genetic factors
 chromosomal abnormalities
 outflow tract defect may cause by abnormal development of neural crest-
derived cell (region of chromosome 22)
 Environmental factors, such as congenital infection or teratogens
 Multifactorial: genetic, environmental, and maternal factors
 The risk of recurrence of congenital heart disease increases if a
1st-degree relative (parent or sibling) is affected.
Chromosome Abnormalities
 Down Syndrome (40%) AVSD, VSD
 Trisomy 13 (80%) VSD, PDA
 Trisomy 18 (100%) VSD, PDA
 Turner’s Syndrome (35%) Coarctation of aorta
 Marfan’s Syndrome Aortic aneurysm, MVP
Teratogen
 Infection
 Rubella PDA, peripheral PS
 Drug
 Dilantin PS, AS
 Lithium Ebstein anomaly
 Alcohol VSD, ASD
 Retinoic acid Interrupted aortic arch, TGA, TOF
 Maternal disease
 Diabetes TGA, VSD, cardiomyopathy
 SLE Complete AV block
Frequencies of CHD
 VSD
 ASD
 Pulmonary stenosis
 PDA
 TOF
 AVSD
42 %
10 %
8 %
7 %
5 %
5 %
How to Approach Congenital Heart Disease
History Taking
 History of cardiology related
 Heart Failure
 Usually present in first year old
 Tachypnea, Prolong feeding, Poor weight gain, Diaphoresis, pale
(sympathetic activity increased), Exercise intolerance
 Cyanosis
 Hypoxic Spell
 Cyanosis, Tachypnea, Flaccid or loss of consciousness
 Arrhythmia
 Most common -> Supraventricular tachycardia
 Neurologic Symptoms
 Cerebral thrombosis, Brain Abscess
History Taking
 Maternal and Perinatal History
 History of drug used (Teratogen)
 Birth weight
 Family History
 Usually prevalence 8 in 1,000
 1st degree related increased in prevalence during 10 – 15 percent
Physical Examination
 General Appearance
 Finding dysmorphic feature
 Vital Signs
 Blood pressure -> Leg > Arm SBP 10-20 mmHg
 Pulse
 Weak Leg > Arm -> Coarctation of Aorta
 Diminish pulse -> Cardiogenic shock or low cardiac output
 Respiratory Rate (Upper limit)
 < 1 month 60 /min
 1 month – 1 year 50 /min
 1 year – 5 years 40 /min
 > 5 years 30 /mins
Physical Examination
 Jugular Venous Pressure
 Suspected right-side heart failure
 Cardiac Exam
 Inspection -> Precordial bulging (RV volume overload)
 Palpation
 Apex : 4th Lt intercostal space midclavicular line
 Thrill for grading murmur
 Auscultation
Physical Examination
 Pulmonary Exam
 Crepitation or Rales -> Pulmonary congestion
 Complication : Pneumonia -> Left to Right Shunt
 Liver and Spleen
 Right-side heart failure -> Hepatomegaly or splenomegaly
 Extremities
 Edema -> Systemic venous congestion -> Right-side heart failure
 Clubbing of finger -> Cyanotic heart
Chest X-Ray
 Cardiovascular Structure
 Location of Heart, Stomach and Liver
 Levocardia, Mesocardia, Dextrocardia (Left, Central, Right)
 Situs Solitus or Situs inversus
 Chamber and Heart Enlargement
 Cardiothoracic Ratio 50 – 60 %
 Identified what chamber enlargement
 Aorta
 Right or Left Aortic Arch
 Size of aorta
Chest X-ray
 Pulmonary Vasculature
 Increased pulmonary vasculature (Left to Right shunt)
 Decreased pulmonary vasculature (Right to Left shunt, PS, PA)
 Normal pulmonary vasculature (PS, AS)
 Extracardiac Structure
 Ribs and Vertebra
 Diaphragm
 Phrenic nerve paralysis, Diaphragmatic hernia
 Lung parenchyma
 Pneumonia or Atelectasis
Electrocardiography
 Ventricular Hypertrophy (Right or Left)
 Axis
Right Ventricular hypertrophy
• R wave >98th percentile in lead V1
• S wave >98th percentile in lead V6
• R wave in V1 + S wave in V6 >98th percentile
• R/S ratio >98 percentile in lead V1
• Right axis deviation
• qR pattern in V1
• Upright T wave in V1 (1 week old to 8 years old)
• RSR’ pattern in lead V1, where R’ >15 mm (<1
year old) or R’ >10 mm (>1 year old)
• Pure R wave in V1 >10 mm (newborn)
• RVH (by voltage criteria) with strain pattern
Left Ventricular Hypertrophy
• R wave >98th percentile in lead V6
• S wave >98th percentile in lead V1
• R wave in V6 + S wave in V1 >98th percentile
• Q wave >98th percentile in lead lll or V6
• R/S ratio >98th percentile in lead V6
• LVH (by voltage criteria) with strain pattern
Congenital heart disease
 Cyanotic / Acyanotic
 Three major categories:
 left-to-right shunt
 right-to-left shunt
 obstruction
 A shunt is an abnormal communication between chambers or
blood vessels
 Direction and magnitude of shunt depends on
 Size of defect
 Relative pulmonary and systemic pressures
 Relative pulmonary and systemic resistance
Congenital heart
disease
Cyanosis Acyanosis
Increase pulmonary
blood flow
Decrease pulmonary
blood flow
Normal pulmonary
blood flow
Increase pulmonary
blood flow
How to Approach Congenital Heart Disease
Acyanotic Congenital Heart Disease
Acyanotic Heart Disease
 Left to Right Shunt
 Increased pulmonary blood flow
 Clinical -> Heart Failure
 VSD, ASD, PDA, AVSD
 Obstructive Lesions
 Normal pulmonary blood flow
 Clinical -> Exercise intolerance
 PS, AS, Coarctation of aorta
Acyanosis
Increase pulmonary
blood flow
Normal pulmonary
blood flow
RVH LVHRVHLVH
ASD
PAPVR
PS
MS
VSD
PDA
AVSD
AS
CoA
Increased pulmonary blood flow
Acyanotic Congenital Heart Disease
Atrial Septal Defect (ASD)
 5 – 10 % of Total congenital heart diseases
 2-fold of Female than male
 Three types exist : primum, secundum and sinus venosus
 The most common is the secundum type (Patent Foramen
Ovale; PFO)
Type of ASD
The Cardiovascular System
Cochard, Larry R., PhD, Netter's Atlas of Human Embryology, Updated Edition, Chapter 4, 83-111
Pathophysiology
Clinical Presentation
 Usually incidental finding heart murmur without symptoms
 1% of first year -> Developed heart failure
 Clinical heart failure depended on shunt
 Physical examination
 Increased right ventricular impulse
 Systolic ejection murmur at left upper sternal border (Relative PS)
 Widely fixed split second heart sound -> prolong ejection time
Investigation for Diagnosis
 Plain film (CXR)
 can be normal is early stages +/- when the ASD is small
 signs of increased pulmonary flow (shunt vascularity)
 enlarged pulmonary vessels
 upper zone vascular prominence
 vessels visible to the periphery of the film
 eventual signs of pulmonary arterial hypertension
 chamber enlargement
 right atrium
 right ventricle
 note : left atrium is normal in size & aortic arch is small to normal
Investigation for Diagnosis
Chest X-ray
Investigation for Diagnosis
 Electrocardiography
 Right axis deviation
 Right atrial enlargement
 Right ventricular hypertrophy (rsR’ in V1 80-90 %)
Complications
 Right-side heart failure
 Right side of the heart to work harder (Volume overload)
 Arrhythmias
 Atrial enlargement -> Atrial Fibrillation
 Stroke
 Clot pass Right to Left to Systemic circulation
 Pulmonary Hypertension
Treatment
Ventricular Septal Defect (VSD)
 Most common of congenital heart disease (20 – 30 %)
 Type of VSD
 Subpulmonic VSD
 Perimembranous VSD
 Inlet VSD
 Muscular VSD
Pathophysiology
Clinical Presentation
 Depended on VSD size and Pulmonary vascular resistant
 Small VSD
 Usually asymptomatic
 Can detect murmur in 1st or 2nd weeks after birth
 Pansystolic murmur at Left lower parasternal border
 Moderate to Large VSD
 Developed heart failure in 1 – 2 months
 Murmur same as small VSD and loud P2
 Eisenmenger’s Complex
Eisenmenger’s Complex
 Consequence of a large preexisting left-to-right shunt
 Pulmonary artery pressures approach systemic levels and the
direction of the flow becomes bidirectional or right to left.
 The high pulmonary vascular resistance is usually established
in infancy (by age 2 years, except in ASD) and is sometimes
present from birth.
Eisenmenger’s Complex
Left to Right Shut
Increased pulmonary blood flow
(Shear stress / Circumferential stretch)
Endothelial dysfunction and vascular remodeling
(Smooth muscle cell proliferation, Increased in extracellular matrix, Intravascular thrombosis)
Increase in PVR
Inverted shunt : Right to Left
Cyanosis (Irreversible Process)
Investigation for Diagnosis
 Plain Film
 The chest radiograph can be normal with a small VSD.
 Larger VSDs may show cardiomegaly
 Left atrial enlargement
 Right and left ventricle can be enlarged
 A large VSD may also show features of pulmonary oedema, pleural
effusion or / and increased pulmonary vascular markings.
Investigation for Diagnosis
Chest X-ray
Investigation for Diagnosis
 Electrocardiography
 Small VSD : Normal EKG
 Moderate VSD : LVH (R in Lead V6 high and can be found deep Q)
 Large VSD : RVH and LVH
Treatment
 Spontaneous Closure (35 –
40 % within 2 years)
 Below 6 months that can’t
control heart failure or
recurrence pneumonia
 6 – 24 months should
surgery in pulmonary
hypertension case
Atrioventricular Septal Defect (AVSD)
 Endocardial cushion defect
 4 – 5 % of Total congenital heart disease
 40 % of Down Syndrome -> CHD
 40 % of CHD in Down Syndrome -> AVSD
 Type of AVSD
 Complete AVSD
 Primum ASD + Inlet VSD + Common AV valve
 Partial AVSD
 Primum ASD +/- Cleft of Mitral valve
 Transitional (Intermediate) AVSD
 Primum ASD + Small VSD but Separate Mitral and Tricuspid valve
Pathophysiology
Clinical Presentation
 Partial and Intermediate AVSD
 Can be developed heart failure in infant
 Cardiac murmur
 Systolic ejection murmur (Relative PS)
 Widely fixed split S2
 Pansystolic murmur at Apex (MS)
 Complete AVSD
 Within 4-8 weeks after birth can be developed heart failure
 Cardiac murmur same as above and Loud P2
Investigation for Diagnosis
 Plain Film
 Not specific can be found chamber enlargement
 Increased Pulmonary Vasculature
Investigation for Diagnosis
Chest X-ray
Investigation for Diagnosis
 Electrocardiography
 Left superior QRS axis (Lead I : positive and aVF : negative)
 Right ventricular hypertrophy or combine ventricular hypertrophy
Treatment
Patent ductus arteriosus (PDA)
 9 – 12 % of Total
congenital heart
disease
 2 fold of prevalence in
female than male
 80 % of Low birth
weight (1,200 gm)
Ductus arteriosus
 Lung expansion
 PVR↓ , PBF↑, PO2↑
 PGE2↓
 Ductus arteriosus constriction
 Functionally closed; immediately after birth (within 10-15hr)
 Anatomical closure; a week to 10 days
→ Ligamentum arteriosum
Pathophysiology
Clinical Presentation
 Small duct with no LV volume overload (normal LV) and normal
PAP (generally asymptomatic)
 Moderate PDA with predominant LV volume overload:
 large LV with normal or reduced function (may present with left
heart failure)
 Moderate PDA with predominant PAH:
 pressure-overloaded RV (may present with right heart failure)
 Large PDA:
 Eisenmenger physiology with differential hypoxaemia and
differential cyanosis (lower extremities cyanotic, sometimes left
arm, too)
Investigation for Diagnosis
 Plain Film
 may vary depending on
 isolated or associated with other cardiac anomalies
 direction of shunt flow (right to left or left to right).
 Can have cardiomegaly
 predominantly left atrial and left ventricular enlargement
Investigation for Diagnosis
Chest X-ray
Investigation for Diagnosis
 Electrocardiography
 Small PDA : normal
 Medium to Large PDA :
 LA enlargement (wide P wave > 2.5 mm)
 LV hypertrophy (R wave in lead V6 > 98th percentile)
 +/- ST elevation (decreased diastolic filling time -> myocardial ischemia)
Treatment
 Preterm with congestive heart failure
 Restrict fluid with corrected anemia (Keep Hct > 45%)
 Indomethacin should be used within 10 days
 Low birth weight : 0.2 mg/kg in first dose and 2nd – 3rd dose q 12-24 hrs
 < 2 days : 0.1 mg/kg
 2-7 days : 0.2 mg/kg
 > 7 days : 0.25 mg/kg
 Small PDA
 should perform intervention if more than 1 year old
 Medium to Large PDA with clinical heart failure
 should perform intervention as soon as possible
Treatment
Normal pulmonary blood flow
Acyanotic Congenital Heart Disease
Coarctation of aorta (CoA)
 6 – 8 % of Total congenital
heart disease
 2-fold of prevalence in male
than female
 35 % associated with Turner
syndrome
 Type of Coarctation of aorta
 Simple CoA -> +/- PDA
 Complex CoA -> Associated
with other congenital heart
disease
Pathophysiology
Clinical Presentation
 Depended on severity of stenosis and other congenital heart
disease
 critical CoA
 Shock, Hypotension, Congestive heart failure
 Ductal Dependent systemic blood flow
 Anemia, Poor perfusion, Tachypnea
 Weak pulse and BP Leg > Arm
 Differential cyanosis Leg < Arm (Except Large PDA)
 Adulthood may be present hypertension unknown caused
 Systolic ejection murmur at left upper sternal border
Investigation for Diagnosis
 Plain Film
 rib notching
 Compression of intercostal collateral arteries below rib
 3 sign
 Proximal to stenosis -> enlargement
 Distal to stenosis -> narrow
 pulmonary vascular marking normal
Chest X-ray
Investigation for Diagnosis
 Electrocardiography
 critical CoA
 Right ventricular hypertrophy
 Pumping of right ventricle -> ductus arteriosus -> descending aorta
Treatment
 Critical CoA
 Maintain Airway
 Prostaglandin E 1 for opening ductus arteriosus
 Coarctation repair
Treatment
Pulmonary Stenosis (PS)
 25 – 30 % of total congenital heart disease
 Type of pulmonary stenosis
 Pulmonary valve stenosis
 Infundibular stenosis
 Pulmonary artery stenosis
Clinical Presentation
 Subinfundibular/infundibular:
 asymptomatic or they may present with angina, dyspnoea, dizziness, or
syncope
 Valvular:
 Mild to moderate valvular PS (Usually asymptomatic)
 Moderate PS can progress at the valvular level (calcification) or at the subvalvular
level, due to reactive myocardial hypertrophy
 Severe stenosis
 dyspnoea and reduced exercise capacity, and have a worse prognosis
 Supravalvular:
 asymptomatic or have symptom of dyspnoea and reduced exercise
capacity
Investigation for Diagnosis
 Plain film
 Non specific
 normal heart size
 Right ventricular hypertrophy
 Dilated pulmonary trunk or a main pulmonary artery
 Electrocardiography
 Right axis deviation
 Right atrial enlargement
 Right ventricle hypertrophy
Chest X-ray
Treatment
 Mild PS
 Follow up and aware bacterial endocarditis prophylaxis
 Moderate to Severe PS
 Balloon pulmonary valvuloplasty
 Valvulotomy in case fail balloon valvuloplasty or dysplastic
pulmonic valve
 Critical PS
 Need ductus arteriosus should give prostaglandin E1 and corrected
anemia
Treatment
Aortic Stenosis (AS)
 3-8 % of total congenital heart disease
 4-fold of prevalence in male than female
 Type of aortic stenosis
 Aortic valve stenosis
 Subaortic stenosis
 Supravalvular aortic stenosis
Clinical Presentation
 90 % Asymptomatic
 10 % developed heart failure in first year
 2/3 occur in 1-2 months
 Tachypnea, low birth weight, hepatomegaly
 Critical AS
 Cardiogenic shcok, poor perfusion
 Ductal dependent systemic blood flow
 +/- Difference cyanosis (Due to PA pass to Aorta)
Investigation for Diagnosis
 Plain Film
 Usually normal cardiac size
 Critical AS -> Cardiomegaly
 Electrocardiography
 Critical AS
 Right atrial enlargement
 Right ventricle hypertrophy
 In adulthood
 Left ventricular hypertrophy
 +/- inverted T wave or ST depression in lead V5 and V6 due to myocardial
ischemia in severe stenosis
Treatment
 Mild AS
 No specific treatment -> F/U clinical and echocardiogram q 1-2 year
 Moderate AS (PPSG 50–75 mmHg) or EKG change or have
symptom
 Balloon valvuloplasty or valvulotomy
 Severe AS (PPSG > 75 mmHg)
 Suggest Balloon valvuloplasty or valvulotomy even though no
clinical symptom
 Critical AS
 Prostaglandin E1
 Inotropic drug
Treatment
Cyanotic Congenital Heart Disease
Acyanotic Heart Disease
 Decreased pulmonary blood flow
 Right ventricular outflow tract obstruction ->
right–to–left shunt
 Increased pulmonary blood flow
 Can be developed to congestive heart failure and cyanosis
Cyanosis
Decrease pulmonary
blood flow
Increase pulmonary
blood flow
RVH CVHRVHLVH
TOF
PA/VSD
Critical PS
DORV/PS
TGA/VSD/PS
TGA
TAPVR
DORV
HLHS
PA/IVS
Tricuspid
atresia
Truncus
arteriosus
Decreased pulmonary blood flow
Cyanotic Congenital Heart Disease
Tetralogy of Follot (TOF)
 Most common in cyanotic congenital heart disease
 9 % of total congenital heart disease
 4 Common of defect
 Ventricular septal defect
 Pulmonary stenosis
 Overriding of aorta
 Right ventricular hypertrophy
Pathophysiology
Clinical Presentation
 Early clinical presentation
 A heart murmur in infancy and progressive cyanosis (from right to
left shunting at the ventricular level secondary to RVOTO).
 Unoperated tetralogy carries a poor prognosis (.95% of patients
used to die before 40 years of age).
 Late clinical presentation
 Late survival after tetralogy repair is excellent, with a 35-year
survival of 85%.
Hypoxic Spell
Increased
Right to Left shunt
Hypoxia
Metabolic Acidosis
Hyperpnoea
Increased
systemic venous return
Infundibular
Spasm
Decreased Systemic
vascular resistance
Investigation for Diagnosis
 Plain Film
 Boot shape heart
 Apex shift with pulmonary trunk indentation
 Decreased pulmonary marking
 Electrocardiography
 Usually found Right ventricular hypertrophy
Chest X-Ray
Treatment
 Hypoxic Spells (Emergency condition)
 Knee-Chest Position
 Control Crying
 Oxygen supplement
 Morphine 0.1 mg/kg (Sedate and reduce infundibular spasm)
 IV Hydration and correct metabolic acidosis
 +/- drug for increased systemic vascular resistance
 Propranolol 1-4 mg/kg/day q 6-8 hrs
 Correct Anemia
 Surgery consideration
Treatment
Tricuspid Atresia (TA)
 3 % of total congenital heart disease
 Hemodynamic
 Blood can’t flow from right atrium to right ventricle
 Right Atrium -> Left atrium (Via ASD) -> Left ventricle)
Clinical presentation
 Developed cyanosis in 1 month
 Large VSD and no pulmonary stenosis
 Heart failure due to decreased pulmonary vascular resistance
 Progressive cyanosis due to restrictive VSD
 Progressive pulmonary stenosis -> Hypoxic Spell
 Cardiac murmur
 Pansystolic murmur at left lower sternal border (restrictive VSD)
 Systolic ejection murmur (pulmonary stenosis)
 Single second heart sound
Investigation for Diagnosis
 Plain Film
 Normal or minimal cardiomegaly
 Decreased pulmonary vascular marking
 Electrocardiography
 Left superior QRS axis
 R wave amplitude in lead V1 low (due to hypoplastic right ventricle)
 +/- Left ventricular hypertrophy
Treatment
 Newborn with severe cyanosis
 Give PGE1 dose 0.05–0.1 mcg/kg/min for maintain ductus arteriosus
 Consider modified Blalock-Taussig shunt
 If restrictive ASD -> balloon atrial septostomy
 Large VSD and no pulmonary stenosis
 For prevent to develop heart failure -> pulmonary artery banding
Timing for Operation
 6 – 12 months
 Bidirectional Glenn shunt
 Superior vena cava -> pulmonary artery
 3 – 4 year
 Fontan operation or total cavopulmonary shunt
 Inferior vena cava -> pulmonary artery
Treatment
Increased pulmonary blood flow
Cyanotic Congenital Heart Disease
Transposition of Great Arteries (TGA)
 5 % of total congenital heart disease
 Ventriculo-arterial discordance:
 Left ventricle to pulmonary artery
 Right ventricle to aorta
Pathophysiology
Clinical Presentation
 Pure TGA with small PFO and PDA
 Cyanosis after birth, metabolic acidosis
 Weak murmur, single second heart sound
 TGA with VSD
 Minimal cyanosis
 Developed heart failure in 2-6 months
 TGA with VSD with PS
 Similar tetralogy of Fallot
 Cyanosis but no clinical heart failure
Investigation for Diagnosis
 Plain film
 Cardiomegaly with a cardiac contours classically described as
appearing like an egg on a string.
 Narrowing of the superior mediastinum as result of the aortic and
pulmonary arterial configuration.
 Electrocardiography
 Right axis deviation
 Right ventricular hypertrophy
 TGA with VSD -> combined ventricular hypertrophy
Chest X-ray
Treatment
 Severe Cyanosis
 PGE1: dose 0.05– 0.1 mcg/kg/min for maintain ductus arteriosus
 Correct metabolic acidosis and electrolytes
 If persistent cyanosis
 Atrial balloon septostomy then consider arterial switch operation that
should perform within 2 weeks
 If more than 2 weeks -> Two-stage arterial switch operation
 Pulmonary banding -> For Left ventricle hypertrophy
 Arterial switch after 7-10 days of pulmonary banding
Treatment
 TGA with VSD and PS -> Palliative operation
 modified Blalock–Taussig shunt then considered definite surgery
if body weight 10-15 kgs for Ratelli Operation
 Closed VSD and used conduit bridging between right ventricle and
pulmonary artery
Treatment
Truncus Arteriosus
 < 1 % of total congenital heart disease
 Common route between pulmonary artery and aorta
Type of Truncus arteriosus
Clinical Presentation
 Minimal Cyanosis
 Can be developed heart failure in 1 month
 Cardiac murmur : pansystolic murmur at left lower sternal
bonder
 Single second heart sound
Investigation for Diagnosis
 Plain Film
 Cardiomegaly
 Increased pulmonary marking
 Electrocardiography
 Left atrial enlargement
 Combined ventricular hypertrophy
Treatment
 Control heart failure during waiting for operation
 Consider for Total repair (Should perform before 6 months)
 Closed VSD
 Connected conduit between right ventricle to pulmonary artery
 If can’t do total repair
 Pulmonary artery banding for prevent pulmonary vascular
obstructive disease
Treatment
Total Anomalous Pulmonary Venous Return
(TAPVR)
 1.5 % of total congenital heart disease
 Usually pulmonary vein must drainage into Left atrium but
blood returning from the lungs drains back to the right side of
the heart.
Pathophysiology
Type of TAPVR
Clinical Presentation
 56 % Symptom presented in first month
 Minimal cyanosis
 Sign of Heart failure
 Systolic ejection murmur at left upper sternal border (Relative PS)
Investigation for Diagnosis
 Plain film
 The right heart is prominent
 because of the increased flow volume
 The supracardiac variant (type I) can classically depict a snowman
appearance on a frontal chest radiograph, also known as figure of 8
heart or cottage loaf heart .
Chest X-Ray
Investigation for Diagnosis
 Electrocardiography
 Right axis deviation
 Right atrium enlargement
 Right ventricular hypertrophy
Treatment
 Should perform Total repair as soon as possible
Double-outlet right ventricular (DORV)
 Two Great Arteries (Aorta and Pulmonary Artery) both originate
from the right ventricle
 Blood from the left ventricle passes across a VSD into the RV to
reach the great arteries
Double-outlet right ventricular (DORV)
Hypoplastic left heart syndrome (HLHS)
 Left side of the heart is very poorly formed
 Cannot support the main circulation.
 Left ventricle and aorta are abnormally small (hypoplastic)
Treatment
Thank you for your kind attention.
Reference
 Moss and Adams’ heart disease in infants, children, and
adolescents, 6 th ed. In: Allen HD, Gutgesell HP, Clark EB,
Driscoll DJ, eds. Philadelphia: Lippincott Williams & Wilkins,
2001:618-35.
 The science and practice of pediatric cardiology, 2nd ed. In:
Garson A Jr, Bricker JT, Fisher DJ, Neish SR, eds. Baltimore:
Williams & Wilkins, 1998:1141–79.
 Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am
1999;46:355–68.
 Comprehensive surgical management of congenital heart
disease. In: Jonas RA, ed. London: Arnold, 2004:386-401.
Reference
 Waldman JD, Wernly JA. Cyanotic congenital heart disease with
decreased pulmonary blood flow in children. Pediatr Clin North
Am 1999;46:385-404.
 Victorica BE. Cyanotic newborns. In: Gessner IH, Victorica BE,
eds. Pediatric cardiology: a problem oriented approach.
Philadelphia, W.B. Saunders 1993:97-109.
 Pediatric cardiac surgery. In: Mavroudis C, Backer CL, eds.
Philadelphia, Mosby 2003:383-97.
Picture Reference
 http://radiopaedia.org
 http://www.rch.org.au/cardiology/heart_defects/

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Congenital Heart Disease

  • 1. PEDIATRICS CONFERENCE PATHOLOGY OF CONGENITAL HEART DISEASE Boonyathee Sorawit MD. Mar 25th , 2014
  • 2. Epidemiology  Congenital heart disease occurs in 0.5–0.8% of live births.  Incidence is higher in stillborns (3–4%), spontaneous abortuses (10–25%) and premature infants (about 2% excluding patent ductus arteriosus [PDA])  About 2–3 in 1,000 newborn infants will be symptomatic with heart disease in the 1st year of life.  The diagnosis is established by 1 week of age in 40–50% of patients with congenital heart disease and by 1 month of age in 50–60% of patients.
  • 3. Etiology  Caused by developmental abnormalities  Genetic factors  chromosomal abnormalities  outflow tract defect may cause by abnormal development of neural crest- derived cell (region of chromosome 22)  Environmental factors, such as congenital infection or teratogens  Multifactorial: genetic, environmental, and maternal factors  The risk of recurrence of congenital heart disease increases if a 1st-degree relative (parent or sibling) is affected.
  • 4. Chromosome Abnormalities  Down Syndrome (40%) AVSD, VSD  Trisomy 13 (80%) VSD, PDA  Trisomy 18 (100%) VSD, PDA  Turner’s Syndrome (35%) Coarctation of aorta  Marfan’s Syndrome Aortic aneurysm, MVP
  • 5. Teratogen  Infection  Rubella PDA, peripheral PS  Drug  Dilantin PS, AS  Lithium Ebstein anomaly  Alcohol VSD, ASD  Retinoic acid Interrupted aortic arch, TGA, TOF  Maternal disease  Diabetes TGA, VSD, cardiomyopathy  SLE Complete AV block
  • 6. Frequencies of CHD  VSD  ASD  Pulmonary stenosis  PDA  TOF  AVSD 42 % 10 % 8 % 7 % 5 % 5 %
  • 7. How to Approach Congenital Heart Disease
  • 8. History Taking  History of cardiology related  Heart Failure  Usually present in first year old  Tachypnea, Prolong feeding, Poor weight gain, Diaphoresis, pale (sympathetic activity increased), Exercise intolerance  Cyanosis  Hypoxic Spell  Cyanosis, Tachypnea, Flaccid or loss of consciousness  Arrhythmia  Most common -> Supraventricular tachycardia  Neurologic Symptoms  Cerebral thrombosis, Brain Abscess
  • 9. History Taking  Maternal and Perinatal History  History of drug used (Teratogen)  Birth weight  Family History  Usually prevalence 8 in 1,000  1st degree related increased in prevalence during 10 – 15 percent
  • 10. Physical Examination  General Appearance  Finding dysmorphic feature  Vital Signs  Blood pressure -> Leg > Arm SBP 10-20 mmHg  Pulse  Weak Leg > Arm -> Coarctation of Aorta  Diminish pulse -> Cardiogenic shock or low cardiac output  Respiratory Rate (Upper limit)  < 1 month 60 /min  1 month – 1 year 50 /min  1 year – 5 years 40 /min  > 5 years 30 /mins
  • 11. Physical Examination  Jugular Venous Pressure  Suspected right-side heart failure  Cardiac Exam  Inspection -> Precordial bulging (RV volume overload)  Palpation  Apex : 4th Lt intercostal space midclavicular line  Thrill for grading murmur  Auscultation
  • 12. Physical Examination  Pulmonary Exam  Crepitation or Rales -> Pulmonary congestion  Complication : Pneumonia -> Left to Right Shunt  Liver and Spleen  Right-side heart failure -> Hepatomegaly or splenomegaly  Extremities  Edema -> Systemic venous congestion -> Right-side heart failure  Clubbing of finger -> Cyanotic heart
  • 13. Chest X-Ray  Cardiovascular Structure  Location of Heart, Stomach and Liver  Levocardia, Mesocardia, Dextrocardia (Left, Central, Right)  Situs Solitus or Situs inversus  Chamber and Heart Enlargement  Cardiothoracic Ratio 50 – 60 %  Identified what chamber enlargement  Aorta  Right or Left Aortic Arch  Size of aorta
  • 14. Chest X-ray  Pulmonary Vasculature  Increased pulmonary vasculature (Left to Right shunt)  Decreased pulmonary vasculature (Right to Left shunt, PS, PA)  Normal pulmonary vasculature (PS, AS)  Extracardiac Structure  Ribs and Vertebra  Diaphragm  Phrenic nerve paralysis, Diaphragmatic hernia  Lung parenchyma  Pneumonia or Atelectasis
  • 15. Electrocardiography  Ventricular Hypertrophy (Right or Left)  Axis Right Ventricular hypertrophy • R wave >98th percentile in lead V1 • S wave >98th percentile in lead V6 • R wave in V1 + S wave in V6 >98th percentile • R/S ratio >98 percentile in lead V1 • Right axis deviation • qR pattern in V1 • Upright T wave in V1 (1 week old to 8 years old) • RSR’ pattern in lead V1, where R’ >15 mm (<1 year old) or R’ >10 mm (>1 year old) • Pure R wave in V1 >10 mm (newborn) • RVH (by voltage criteria) with strain pattern Left Ventricular Hypertrophy • R wave >98th percentile in lead V6 • S wave >98th percentile in lead V1 • R wave in V6 + S wave in V1 >98th percentile • Q wave >98th percentile in lead lll or V6 • R/S ratio >98th percentile in lead V6 • LVH (by voltage criteria) with strain pattern
  • 16. Congenital heart disease  Cyanotic / Acyanotic  Three major categories:  left-to-right shunt  right-to-left shunt  obstruction  A shunt is an abnormal communication between chambers or blood vessels  Direction and magnitude of shunt depends on  Size of defect  Relative pulmonary and systemic pressures  Relative pulmonary and systemic resistance
  • 17. Congenital heart disease Cyanosis Acyanosis Increase pulmonary blood flow Decrease pulmonary blood flow Normal pulmonary blood flow Increase pulmonary blood flow How to Approach Congenital Heart Disease
  • 19. Acyanotic Heart Disease  Left to Right Shunt  Increased pulmonary blood flow  Clinical -> Heart Failure  VSD, ASD, PDA, AVSD  Obstructive Lesions  Normal pulmonary blood flow  Clinical -> Exercise intolerance  PS, AS, Coarctation of aorta
  • 20. Acyanosis Increase pulmonary blood flow Normal pulmonary blood flow RVH LVHRVHLVH ASD PAPVR PS MS VSD PDA AVSD AS CoA
  • 21. Increased pulmonary blood flow Acyanotic Congenital Heart Disease
  • 22. Atrial Septal Defect (ASD)  5 – 10 % of Total congenital heart diseases  2-fold of Female than male  Three types exist : primum, secundum and sinus venosus  The most common is the secundum type (Patent Foramen Ovale; PFO)
  • 23. Type of ASD The Cardiovascular System Cochard, Larry R., PhD, Netter's Atlas of Human Embryology, Updated Edition, Chapter 4, 83-111
  • 25. Clinical Presentation  Usually incidental finding heart murmur without symptoms  1% of first year -> Developed heart failure  Clinical heart failure depended on shunt  Physical examination  Increased right ventricular impulse  Systolic ejection murmur at left upper sternal border (Relative PS)  Widely fixed split second heart sound -> prolong ejection time
  • 26. Investigation for Diagnosis  Plain film (CXR)  can be normal is early stages +/- when the ASD is small  signs of increased pulmonary flow (shunt vascularity)  enlarged pulmonary vessels  upper zone vascular prominence  vessels visible to the periphery of the film  eventual signs of pulmonary arterial hypertension  chamber enlargement  right atrium  right ventricle  note : left atrium is normal in size & aortic arch is small to normal
  • 29. Investigation for Diagnosis  Electrocardiography  Right axis deviation  Right atrial enlargement  Right ventricular hypertrophy (rsR’ in V1 80-90 %)
  • 30. Complications  Right-side heart failure  Right side of the heart to work harder (Volume overload)  Arrhythmias  Atrial enlargement -> Atrial Fibrillation  Stroke  Clot pass Right to Left to Systemic circulation  Pulmonary Hypertension
  • 32. Ventricular Septal Defect (VSD)  Most common of congenital heart disease (20 – 30 %)  Type of VSD  Subpulmonic VSD  Perimembranous VSD  Inlet VSD  Muscular VSD
  • 34. Clinical Presentation  Depended on VSD size and Pulmonary vascular resistant  Small VSD  Usually asymptomatic  Can detect murmur in 1st or 2nd weeks after birth  Pansystolic murmur at Left lower parasternal border  Moderate to Large VSD  Developed heart failure in 1 – 2 months  Murmur same as small VSD and loud P2  Eisenmenger’s Complex
  • 35. Eisenmenger’s Complex  Consequence of a large preexisting left-to-right shunt  Pulmonary artery pressures approach systemic levels and the direction of the flow becomes bidirectional or right to left.  The high pulmonary vascular resistance is usually established in infancy (by age 2 years, except in ASD) and is sometimes present from birth.
  • 36. Eisenmenger’s Complex Left to Right Shut Increased pulmonary blood flow (Shear stress / Circumferential stretch) Endothelial dysfunction and vascular remodeling (Smooth muscle cell proliferation, Increased in extracellular matrix, Intravascular thrombosis) Increase in PVR Inverted shunt : Right to Left Cyanosis (Irreversible Process)
  • 37. Investigation for Diagnosis  Plain Film  The chest radiograph can be normal with a small VSD.  Larger VSDs may show cardiomegaly  Left atrial enlargement  Right and left ventricle can be enlarged  A large VSD may also show features of pulmonary oedema, pleural effusion or / and increased pulmonary vascular markings.
  • 40. Investigation for Diagnosis  Electrocardiography  Small VSD : Normal EKG  Moderate VSD : LVH (R in Lead V6 high and can be found deep Q)  Large VSD : RVH and LVH
  • 41. Treatment  Spontaneous Closure (35 – 40 % within 2 years)  Below 6 months that can’t control heart failure or recurrence pneumonia  6 – 24 months should surgery in pulmonary hypertension case
  • 42. Atrioventricular Septal Defect (AVSD)  Endocardial cushion defect  4 – 5 % of Total congenital heart disease  40 % of Down Syndrome -> CHD  40 % of CHD in Down Syndrome -> AVSD  Type of AVSD  Complete AVSD  Primum ASD + Inlet VSD + Common AV valve  Partial AVSD  Primum ASD +/- Cleft of Mitral valve  Transitional (Intermediate) AVSD  Primum ASD + Small VSD but Separate Mitral and Tricuspid valve
  • 44. Clinical Presentation  Partial and Intermediate AVSD  Can be developed heart failure in infant  Cardiac murmur  Systolic ejection murmur (Relative PS)  Widely fixed split S2  Pansystolic murmur at Apex (MS)  Complete AVSD  Within 4-8 weeks after birth can be developed heart failure  Cardiac murmur same as above and Loud P2
  • 45. Investigation for Diagnosis  Plain Film  Not specific can be found chamber enlargement  Increased Pulmonary Vasculature
  • 48. Investigation for Diagnosis  Electrocardiography  Left superior QRS axis (Lead I : positive and aVF : negative)  Right ventricular hypertrophy or combine ventricular hypertrophy
  • 50. Patent ductus arteriosus (PDA)  9 – 12 % of Total congenital heart disease  2 fold of prevalence in female than male  80 % of Low birth weight (1,200 gm)
  • 51. Ductus arteriosus  Lung expansion  PVR↓ , PBF↑, PO2↑  PGE2↓  Ductus arteriosus constriction  Functionally closed; immediately after birth (within 10-15hr)  Anatomical closure; a week to 10 days → Ligamentum arteriosum
  • 53. Clinical Presentation  Small duct with no LV volume overload (normal LV) and normal PAP (generally asymptomatic)  Moderate PDA with predominant LV volume overload:  large LV with normal or reduced function (may present with left heart failure)  Moderate PDA with predominant PAH:  pressure-overloaded RV (may present with right heart failure)  Large PDA:  Eisenmenger physiology with differential hypoxaemia and differential cyanosis (lower extremities cyanotic, sometimes left arm, too)
  • 54. Investigation for Diagnosis  Plain Film  may vary depending on  isolated or associated with other cardiac anomalies  direction of shunt flow (right to left or left to right).  Can have cardiomegaly  predominantly left atrial and left ventricular enlargement
  • 57. Investigation for Diagnosis  Electrocardiography  Small PDA : normal  Medium to Large PDA :  LA enlargement (wide P wave > 2.5 mm)  LV hypertrophy (R wave in lead V6 > 98th percentile)  +/- ST elevation (decreased diastolic filling time -> myocardial ischemia)
  • 58. Treatment  Preterm with congestive heart failure  Restrict fluid with corrected anemia (Keep Hct > 45%)  Indomethacin should be used within 10 days  Low birth weight : 0.2 mg/kg in first dose and 2nd – 3rd dose q 12-24 hrs  < 2 days : 0.1 mg/kg  2-7 days : 0.2 mg/kg  > 7 days : 0.25 mg/kg  Small PDA  should perform intervention if more than 1 year old  Medium to Large PDA with clinical heart failure  should perform intervention as soon as possible
  • 60. Normal pulmonary blood flow Acyanotic Congenital Heart Disease
  • 61. Coarctation of aorta (CoA)  6 – 8 % of Total congenital heart disease  2-fold of prevalence in male than female  35 % associated with Turner syndrome  Type of Coarctation of aorta  Simple CoA -> +/- PDA  Complex CoA -> Associated with other congenital heart disease
  • 63. Clinical Presentation  Depended on severity of stenosis and other congenital heart disease  critical CoA  Shock, Hypotension, Congestive heart failure  Ductal Dependent systemic blood flow  Anemia, Poor perfusion, Tachypnea  Weak pulse and BP Leg > Arm  Differential cyanosis Leg < Arm (Except Large PDA)  Adulthood may be present hypertension unknown caused  Systolic ejection murmur at left upper sternal border
  • 64. Investigation for Diagnosis  Plain Film  rib notching  Compression of intercostal collateral arteries below rib  3 sign  Proximal to stenosis -> enlargement  Distal to stenosis -> narrow  pulmonary vascular marking normal
  • 66. Investigation for Diagnosis  Electrocardiography  critical CoA  Right ventricular hypertrophy  Pumping of right ventricle -> ductus arteriosus -> descending aorta
  • 67. Treatment  Critical CoA  Maintain Airway  Prostaglandin E 1 for opening ductus arteriosus  Coarctation repair
  • 69. Pulmonary Stenosis (PS)  25 – 30 % of total congenital heart disease  Type of pulmonary stenosis  Pulmonary valve stenosis  Infundibular stenosis  Pulmonary artery stenosis
  • 70. Clinical Presentation  Subinfundibular/infundibular:  asymptomatic or they may present with angina, dyspnoea, dizziness, or syncope  Valvular:  Mild to moderate valvular PS (Usually asymptomatic)  Moderate PS can progress at the valvular level (calcification) or at the subvalvular level, due to reactive myocardial hypertrophy  Severe stenosis  dyspnoea and reduced exercise capacity, and have a worse prognosis  Supravalvular:  asymptomatic or have symptom of dyspnoea and reduced exercise capacity
  • 71. Investigation for Diagnosis  Plain film  Non specific  normal heart size  Right ventricular hypertrophy  Dilated pulmonary trunk or a main pulmonary artery  Electrocardiography  Right axis deviation  Right atrial enlargement  Right ventricle hypertrophy
  • 73. Treatment  Mild PS  Follow up and aware bacterial endocarditis prophylaxis  Moderate to Severe PS  Balloon pulmonary valvuloplasty  Valvulotomy in case fail balloon valvuloplasty or dysplastic pulmonic valve  Critical PS  Need ductus arteriosus should give prostaglandin E1 and corrected anemia
  • 75. Aortic Stenosis (AS)  3-8 % of total congenital heart disease  4-fold of prevalence in male than female  Type of aortic stenosis  Aortic valve stenosis  Subaortic stenosis  Supravalvular aortic stenosis
  • 76. Clinical Presentation  90 % Asymptomatic  10 % developed heart failure in first year  2/3 occur in 1-2 months  Tachypnea, low birth weight, hepatomegaly  Critical AS  Cardiogenic shcok, poor perfusion  Ductal dependent systemic blood flow  +/- Difference cyanosis (Due to PA pass to Aorta)
  • 77. Investigation for Diagnosis  Plain Film  Usually normal cardiac size  Critical AS -> Cardiomegaly  Electrocardiography  Critical AS  Right atrial enlargement  Right ventricle hypertrophy  In adulthood  Left ventricular hypertrophy  +/- inverted T wave or ST depression in lead V5 and V6 due to myocardial ischemia in severe stenosis
  • 78. Treatment  Mild AS  No specific treatment -> F/U clinical and echocardiogram q 1-2 year  Moderate AS (PPSG 50–75 mmHg) or EKG change or have symptom  Balloon valvuloplasty or valvulotomy  Severe AS (PPSG > 75 mmHg)  Suggest Balloon valvuloplasty or valvulotomy even though no clinical symptom  Critical AS  Prostaglandin E1  Inotropic drug
  • 81. Acyanotic Heart Disease  Decreased pulmonary blood flow  Right ventricular outflow tract obstruction -> right–to–left shunt  Increased pulmonary blood flow  Can be developed to congestive heart failure and cyanosis
  • 82. Cyanosis Decrease pulmonary blood flow Increase pulmonary blood flow RVH CVHRVHLVH TOF PA/VSD Critical PS DORV/PS TGA/VSD/PS TGA TAPVR DORV HLHS PA/IVS Tricuspid atresia Truncus arteriosus
  • 83. Decreased pulmonary blood flow Cyanotic Congenital Heart Disease
  • 84. Tetralogy of Follot (TOF)  Most common in cyanotic congenital heart disease  9 % of total congenital heart disease  4 Common of defect  Ventricular septal defect  Pulmonary stenosis  Overriding of aorta  Right ventricular hypertrophy
  • 86. Clinical Presentation  Early clinical presentation  A heart murmur in infancy and progressive cyanosis (from right to left shunting at the ventricular level secondary to RVOTO).  Unoperated tetralogy carries a poor prognosis (.95% of patients used to die before 40 years of age).  Late clinical presentation  Late survival after tetralogy repair is excellent, with a 35-year survival of 85%.
  • 87. Hypoxic Spell Increased Right to Left shunt Hypoxia Metabolic Acidosis Hyperpnoea Increased systemic venous return Infundibular Spasm Decreased Systemic vascular resistance
  • 88. Investigation for Diagnosis  Plain Film  Boot shape heart  Apex shift with pulmonary trunk indentation  Decreased pulmonary marking  Electrocardiography  Usually found Right ventricular hypertrophy
  • 90. Treatment  Hypoxic Spells (Emergency condition)  Knee-Chest Position  Control Crying  Oxygen supplement  Morphine 0.1 mg/kg (Sedate and reduce infundibular spasm)  IV Hydration and correct metabolic acidosis  +/- drug for increased systemic vascular resistance  Propranolol 1-4 mg/kg/day q 6-8 hrs  Correct Anemia  Surgery consideration
  • 92. Tricuspid Atresia (TA)  3 % of total congenital heart disease  Hemodynamic  Blood can’t flow from right atrium to right ventricle  Right Atrium -> Left atrium (Via ASD) -> Left ventricle)
  • 93. Clinical presentation  Developed cyanosis in 1 month  Large VSD and no pulmonary stenosis  Heart failure due to decreased pulmonary vascular resistance  Progressive cyanosis due to restrictive VSD  Progressive pulmonary stenosis -> Hypoxic Spell  Cardiac murmur  Pansystolic murmur at left lower sternal border (restrictive VSD)  Systolic ejection murmur (pulmonary stenosis)  Single second heart sound
  • 94. Investigation for Diagnosis  Plain Film  Normal or minimal cardiomegaly  Decreased pulmonary vascular marking  Electrocardiography  Left superior QRS axis  R wave amplitude in lead V1 low (due to hypoplastic right ventricle)  +/- Left ventricular hypertrophy
  • 95. Treatment  Newborn with severe cyanosis  Give PGE1 dose 0.05–0.1 mcg/kg/min for maintain ductus arteriosus  Consider modified Blalock-Taussig shunt  If restrictive ASD -> balloon atrial septostomy  Large VSD and no pulmonary stenosis  For prevent to develop heart failure -> pulmonary artery banding
  • 96. Timing for Operation  6 – 12 months  Bidirectional Glenn shunt  Superior vena cava -> pulmonary artery  3 – 4 year  Fontan operation or total cavopulmonary shunt  Inferior vena cava -> pulmonary artery
  • 98. Increased pulmonary blood flow Cyanotic Congenital Heart Disease
  • 99. Transposition of Great Arteries (TGA)  5 % of total congenital heart disease  Ventriculo-arterial discordance:  Left ventricle to pulmonary artery  Right ventricle to aorta
  • 101. Clinical Presentation  Pure TGA with small PFO and PDA  Cyanosis after birth, metabolic acidosis  Weak murmur, single second heart sound  TGA with VSD  Minimal cyanosis  Developed heart failure in 2-6 months  TGA with VSD with PS  Similar tetralogy of Fallot  Cyanosis but no clinical heart failure
  • 102. Investigation for Diagnosis  Plain film  Cardiomegaly with a cardiac contours classically described as appearing like an egg on a string.  Narrowing of the superior mediastinum as result of the aortic and pulmonary arterial configuration.  Electrocardiography  Right axis deviation  Right ventricular hypertrophy  TGA with VSD -> combined ventricular hypertrophy
  • 104. Treatment  Severe Cyanosis  PGE1: dose 0.05– 0.1 mcg/kg/min for maintain ductus arteriosus  Correct metabolic acidosis and electrolytes  If persistent cyanosis  Atrial balloon septostomy then consider arterial switch operation that should perform within 2 weeks  If more than 2 weeks -> Two-stage arterial switch operation  Pulmonary banding -> For Left ventricle hypertrophy  Arterial switch after 7-10 days of pulmonary banding
  • 105. Treatment  TGA with VSD and PS -> Palliative operation  modified Blalock–Taussig shunt then considered definite surgery if body weight 10-15 kgs for Ratelli Operation  Closed VSD and used conduit bridging between right ventricle and pulmonary artery
  • 107. Truncus Arteriosus  < 1 % of total congenital heart disease  Common route between pulmonary artery and aorta
  • 108. Type of Truncus arteriosus
  • 109. Clinical Presentation  Minimal Cyanosis  Can be developed heart failure in 1 month  Cardiac murmur : pansystolic murmur at left lower sternal bonder  Single second heart sound
  • 110. Investigation for Diagnosis  Plain Film  Cardiomegaly  Increased pulmonary marking  Electrocardiography  Left atrial enlargement  Combined ventricular hypertrophy
  • 111. Treatment  Control heart failure during waiting for operation  Consider for Total repair (Should perform before 6 months)  Closed VSD  Connected conduit between right ventricle to pulmonary artery  If can’t do total repair  Pulmonary artery banding for prevent pulmonary vascular obstructive disease
  • 113. Total Anomalous Pulmonary Venous Return (TAPVR)  1.5 % of total congenital heart disease  Usually pulmonary vein must drainage into Left atrium but blood returning from the lungs drains back to the right side of the heart.
  • 116. Clinical Presentation  56 % Symptom presented in first month  Minimal cyanosis  Sign of Heart failure  Systolic ejection murmur at left upper sternal border (Relative PS)
  • 117. Investigation for Diagnosis  Plain film  The right heart is prominent  because of the increased flow volume  The supracardiac variant (type I) can classically depict a snowman appearance on a frontal chest radiograph, also known as figure of 8 heart or cottage loaf heart .
  • 119. Investigation for Diagnosis  Electrocardiography  Right axis deviation  Right atrium enlargement  Right ventricular hypertrophy
  • 120. Treatment  Should perform Total repair as soon as possible
  • 121. Double-outlet right ventricular (DORV)  Two Great Arteries (Aorta and Pulmonary Artery) both originate from the right ventricle  Blood from the left ventricle passes across a VSD into the RV to reach the great arteries
  • 123. Hypoplastic left heart syndrome (HLHS)  Left side of the heart is very poorly formed  Cannot support the main circulation.  Left ventricle and aorta are abnormally small (hypoplastic)
  • 125. Thank you for your kind attention.
  • 126. Reference  Moss and Adams’ heart disease in infants, children, and adolescents, 6 th ed. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ, eds. Philadelphia: Lippincott Williams & Wilkins, 2001:618-35.  The science and practice of pediatric cardiology, 2nd ed. In: Garson A Jr, Bricker JT, Fisher DJ, Neish SR, eds. Baltimore: Williams & Wilkins, 1998:1141–79.  Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am 1999;46:355–68.  Comprehensive surgical management of congenital heart disease. In: Jonas RA, ed. London: Arnold, 2004:386-401.
  • 127. Reference  Waldman JD, Wernly JA. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am 1999;46:385-404.  Victorica BE. Cyanotic newborns. In: Gessner IH, Victorica BE, eds. Pediatric cardiology: a problem oriented approach. Philadelphia, W.B. Saunders 1993:97-109.  Pediatric cardiac surgery. In: Mavroudis C, Backer CL, eds. Philadelphia, Mosby 2003:383-97.
  • 128. Picture Reference  http://radiopaedia.org  http://www.rch.org.au/cardiology/heart_defects/