Critical Congenital Heart Disease Sasathorn Tharapoom Ubolwan Chansingthong 5 th year medical student Advisor Yasinee Apiraknapanon M.D. Department of Pediatric, Naresuan University Hospital
Critical Congenital Heart Disease
Sasathorn Tharapoom
Ubolwan Chansingthong 5th year medical student
Advisor
Yasinee Apiraknapanon M.D.
Department of Pediatric, Naresuan University Hospital
Embryology and Physiology
Fetal Circulation
Flow Chart of Fetal Circulation
Neonatal Circulation
1. Placenta Removal 2. Breathing
Lung Expansion
Pulmonary Artery Dilatation
PVR
Ductus Venosus Closure
Flow to RA
RA Pressure
PBF
LA Pressure
Close Foramen Ovale
SVR
Flow to LA
PDA Flow
PaO2
Circulatory Adjustment After Birth
1. Placenta Removal 2. Breathing
Lung Expansion
Pulmonary Artery dilatation
PVR PBF
SVR
PDA flow
PaO2
Closure of Ductus Arteriosus
PGE
Lt. to Rt. Rt. to Lt. Shunt
Ductal tissue contraction
Close Ductus Arteriosus
Human Circulation
Fetal vs. Infant Circulation
• Fetal
• Low pressure system
• Right to left shunting
• Lungs non-functional
• Increased pulmonary
resistance
• Decreased systemic
resistance
• Infant
• High pressure system
• Left to right blood flow
• Lungs functional
• Decreased pulmonary
resistance
• Increased systemic
resistance
Approach to Critical Congenital Heart Disease (CCHD)
Classification of CCHD
• Ductal dependent for pulmonary blood flow
• Ductal dependent for systemic blood flow
• Ductal dependent for blood mixed
• Total anomalous pulmonary venous return with obstruction
Clinical Presentation in CCHD
• Can be asymptomatic in newborn period
• Tachypnea o Many other etiologies
• Absent or weak femoral pulses o Only occurs after the PDA has closed
• Murmur o Occur in up to 77% of newborns, often not pathologic
o Some CCHD lesions do not have murmurs
o Some forms of CCHD do not have murmur until pulmonary vascular resistance falls
• Cyanosis from hypoxia
• Shock
Clinical Presentation in CCHD
Clinical
• Ductal dependent for
pulmonary blood flow
• Ductal dependent for blood
mixed
• Total anomalous pulmonary venous return with obstruction
• Cyanosis from hypoxia • + Tachypnea, heart murmur
• Ductal dependent for systemic
blood flow
• Shock
• Absent or weak femoral pulses
(PDA closed)
• + Tachypnea, cyanosis, heart murmur
Cyanosis • Visual recognition of hypoxia – not easily detected
• Dependent on hemoglobin levels
• Central cyanosis : deoxygenated Hb ≥ 5𝑔/𝑑𝑙
• Peripheral cyanosis : poor perfusion
• Differential cyanosis : preductal SpO2 > postductal
SpO2
• Reversed-differential cyanosis : postductal SpO2 >
preductal SpO2
Cyanosis in Newborn • Heart : CCHD
• Lungs
o Parenchyma : MAS, RDS, pneumonia, atelectasis etc.
o Airway : choanal atresia, Tracheal stenosis, etc.
o Extrinsic lung compression : pneumothorax, pleural effusion,
diaphragmatic hernia, etc.
o Hypoventilation : CNS/neuromuscular disease, sedation,
sepsis etc.
• PPHN
• Abnormal hemoglobin : methemoglobinemia
• Metabolic : hypothermia, hypoglycemia, neonatal
sepsis (poor tissue perfusion)
Initial Evaluation and Management
• ABC : airway, breathing and circulation
• Oxygen supplement : keep SpO2 75-85%
• PGE1
• Pre and postductal SpO2
o Differential cyanosis (preductal SpO2 > postductal SpO2) :
PPHN, severe coarctation of aorta with PDA
o Reverse differential cyanosis (postductal SpO2 > preductal
SpO2) : D-TGA with pulmonary hypertension, D-TGA with
coarctation of aorta
• Hyperoxia test ในผปวยทเขยว
Acute Mx.
• ABC
Hx, PE
• CCHD : heart murmur, cardiomegaly hepatomegaly
• PPHN : respiratory distress, labile cyanosis
Inv.
• CXR
• Hct, blood glucose, ABG
• Hyperoxia test, hyperventilation, pre-post duct PaO2
EKG •Suspected CCHD
PGE1
•Can’t exclude ductal dependent lesion
•Consult & supportive treatment
Test Method Result (ABG) Suggest
Hyperoxia Exposed to 100%
FiO2 for 5-10 min
• PaO2 increases to
>100mmHg
• PaO2 increases to
< 20mmHg
• Pulmonary
parenchymal
disease
• PPHN/CCHD
Hyperoxia-
Hyperventilation
Mechanical
ventilator with FiO2
100%
RR 100-150 bpm
• PaO2 increases to
>100mmHg
without
hyperventilation
• PaO2 increases at
a critical PCO2
often to <25mmHg
• No increase in
PaO2 despite
hyperventilation
• Pulmonary
parenchymal
disease
• PPHN
• CCHD
Test Method Result (ABG) Suggest
Simultaneous
preductal-
postductal PO2
Compare PO2 of right
arm/shoulder to that lower
abdomen/extremities
• Preductal
PO2 ≥15 +
postductal PO2
• Rt. to Lt.
shunt at
PDA
Central Cyanosis
Hyperoxia test
Not Improved
Hyperoxia-Hyperventilation test
Improved
Improved Not Improved
PPHN CCHD Echo
Pulse Oximetry for CCHD (CCHD Screening)
• Pulse oximetry can identify hypoxemia that is not
visible clinically
• Hypoxemia occurs in many forms of CHD
• Can also identify differences in pre and post ductal
saturations
• Pulse oximetry is quick, easy, and safe
Primary Targets for Screening
• Hypoplastic Left Heart Syndrome
• Pulmonary Atresia
• Tetrology of Fallot
• Total Anomalous Pulmonary Venous Return
• Transposition of Great Arteries
• Tricuspid Atresia
• Truncus Arteriosus
Routine Pulse Oximetry Screening
PROS CONS
• Earlier detection of
CHD, earlier treatment
• Decrease in mortality
• Detection of other
conditions (PPHN, PNX,
PNA, early sepsis)
• Low risk of harm, non-
invasive
• Cost?
• False reassurance if
negative screen
• False Positives
• Parental and staff
anxiety
• Elevated use of
healthcare
• Cost?
Ductal Dependent “Pulmonary Blood
Flow”
Ductal Dependent Pulmonary Blood Flow
• Obstruction to pulmonary blood flow
• most common causes : right-sided obstructive lesion
• Need PDA for pulmonary blood flow
• Presentation o Progressive cyanosis
o PE : single S2 + murmur
o Chest X-ray : decreased PVM
Ductal Dependent Pulmonary Blood Flow
• Common lesions
o Tetralogy of Fallot
o Double outlet right ventricle with pulmonary stenosis
o Single ventricle with pulmonary stenosis
o Pulmonary atresia with intact ventricular septum
o Pulmonary atresia with VSD
o Tricuspid atresia with pulmonary stenosis
Management
• PGE1 0.05-1 mcg/kg/min : increase PBF
• Stabilizes infant : until surgical intervention
• Assisted ventilation and increased environmental
oxygen
• SpO2 75-85% are adequate
PGE1 • Dose 0.05-1mcg/kg/min
o Starting dose of PGE1 is 0.05 mcg/kg/min IV
o If no improvement, ↑ dose to 0.1mcg/kg/min
o usual maintenances dose of PGE1 is 0.025 mcg/kg/min
• Complications of PGE1: o Apnea : direct effect of PGE1 on the CNS
• Need assisted ventilation immediately after PGE1 has been started
o Hypotension : peripheral vasodilation, decreased cardiac output
• continuous measurement of arterial blood pressure
o Fever
o Irritability, abnormal EEG and seizures
o Diarrhea
o hypertrophy of gastric mucosa : longer term effects
Tetralogy of Fallot (TOF)
TOF
• 5-10% of Congenital heart disease
• Most common cyanotic heart disease
• Conotruncal maldevelopment
• Anterior displacement of conus septum
LV
RV
Conus Cordis RVOT, LVOT
1
1
1
0.4 0.6
0.6
0.6
1
Qp : Qs
0.6 : 1
Left heart
Decreased volume
Normal pressure Right heart
Normal volume
Increased pressure
TOF o RVOT obstruction
o RV pressure ~ LV pressure
o Rt. to Lt. shunt (through VSD) cyanosis
• Severity depends on : RVOT obstruction, collateral
vessel
Signs & Symptoms
• Central Cyanosis
• Dyspnea on exertion
• Squatting position
• Clubbing fingers
• Severe cyanosis : tachypnea, single S2 (severe PS)
• SEM at mid LUSB (turbulent flow ผาน RVOT)
• No VSD murmur (pressure RV ~LV)
• Hypoxic spells : severe cyanosis, hyperpnoea, PS murmur เบาสนลง
• Acyanotic TOF (pink TOF) : heart murmur
Vicious Cycle of Hypoxic Spell
(Systemic vascular resistance)
Investigation
• Chest X-ray
• EKG
• Echocardiogram
• Cardiac catheterization
CXR
• Pulmonary
vascular marking
• Boot shaped heart
(RVH)
Right axis deviation,
RVH : Tall R wave with upright T wave in right chest lead,
Deep S wave in left chest lead
Echocardiogram • VSD
• Overriding aorta
• RVOT obstruction : infundibular stenosis/valvular PS
• Turbulent flow
• peak flow velocity pressure gradient
• Main pulmonary artery and bifurcation
characteristics
• Coronary artery abnormality
• Others abnormality : ASD, PDA, collateral vessels
Cardiac Catheterization • For surgery
• Distal pulmonary artery, collateral vessels, abnormal
coronary artery
• Hemodynamic
o Ascending aorta : desaturation
Treatment • Medical treatment : hypoxic spell
• Surgery repair anatomical defect : indication
o Decreased exercise tolerance
o Increased hypoxic spells
o Polycythemia
Hypoxic Spells Management 1. Calm down
2. Knee chest position ( systemic vascular resistance , systemic venous return)
3. Oxygen supplement
4. MO 0.1 mg/kg ( systemic venous return)
5. IV Fluid
(correct dehydration)
6. NaHCO3 1-2 mEq/kg ( acidosis when spell >10-15 min)
7. Propranolol 0.1mg/kg + NSS up to 10 ml IV in 5-10min ( infundibular spasm)
8. Correct anemia 9. Phenylephrine HCl
10. Propranolol 1-2MKday o 3-4 times/day IV (prevent spell)
Surgical Management • Severe cyanosis (Hct >65%) : palliative surgery
(systemic to pulmonary shunt)
o At age 6 month echocardiogram, cardiac catheterization ประเมนขนาด PA
o ถา PAขนาดใหญพอ total correction/เลกท า shunt เพม
• Moderate cyanosis : พจารณาผาตดตามขนาด PA
• Mild cyanosis : total correction (สามารถท าไดเนองจาก PA มขนาดใหญพอ) o VSD closure
o ขยายทางเดนเลอดจาก RV Lungs
Systemic to Pulmonary Shunt
Pulmonary Atresia with VSD
Pulmonary Atresia with VSD
• Extremely severe form of TOF
• Incomplete septation conus cordis VSD
• Abnormal septation of truncus arteriosus atretic
pulmonic valve, abnormal pulmonary trunk
• 70% PBF ผาน PDA, 30% PBF จาก systemic collateral
artery
Lung bud
Paired dorsal a.
Intersegmental a.
Pul. vas. plx. 6th aortic arch
Pulmonary trunk
RPA,LPA
PA
Bronchial a. Dorsal a. collateral
Pulmonary Trunk Abnormalities
PV annulus
Hypoplastic
pulmonary trunk
Systemic Collateral Artery
PDA
Signs & Symptoms • Cyanosis and dyspnea in a few hours/days after
birth
• O2sat 90-92% (nonsevere hypoxia)
• Single S2
• No murmur
• SEM/continuous murmur of PDA at LUSB
Investigation • EKG : right axis deviation, RVH
• CXR : decreased PBF, concave/flat pulmonary trunk
• Echocardiogram :
o PDA, collateral a.
o VSD
o Overriding aorta
o ไมพบ pulmonary outflow จาก RV
o Atretic/hypoplastic pulmonary trunk
o Cardiac catheterization : collateral artery
Treatment • Severe cyanosis
o PGE1 0.05-1 mcg/kg/min IV 0.01-0.03 mcg/kg/min
o Systemic to pulmonary shunt/PDA stenting
• Surgery o Palliative surgery 2-3 ปกอนท า total correction
o Total correction : at age 3-5 year
• VSD closure
• Ligate systemic to pulmonary shunt
Double Outlet Right Ventricle (DORV) with
Pulmonary Stenosis
DORV • Cyanotic heart disease
• 0.8-1% of congenital heart disease
• Aorta and pulmonary artery arise from RV
• No arteries arise from LV
DORV • Conotruncal maldevelopment
• Incomplete septation of conus cordis
• the aortic and mitral valves are separated by a
smooth muscular conus
• No leftward shift of LVOT structure
Subaortic
VSD Subpulmonic
VSD
Doubly
committed
VSD
Remoted
VSD
Subaortic
VSD
คลาย Large VSD (Left to right shunt มาก) เขยวไมมาก
O2sat 90-96%
Heart failure + PHT
คลาย d-TGA with VSD เขยวมาก
Heart failure + PHT
Subpulmonic
VSD
Doubly
committed
VSD
Remoted
VSD
คลาย Large VSD
เขยวมากกวา subaortic นอยกวา subpulmonic Heart failure + PHT
PBF
PS
Signs & Symptoms DORV/PS • Hypoxia cyanosis dyspnea
• Severity depends on : severity of PS, relationship
with VSD
• PE :
o Single S2
o SEM at LUSB (PS murmur) may transmit to back
Investigations • CXR
o Normal heart size
o Decreased pulmonary vascular marking as in TOF
• EKG
o Right axis deviation
o RVH ± RAE (in severe PS)
• Echocardiogram
o Relationship of VSD and great arteries
o No LV outflow
o VSD
o Turbulent flow ผาน subvalvular, pulmonary valve
Treatment • Treat hypoxic spell
• Surgery
o Small PA, severe PS, severe cyanosis
• Palliative Blallock-Taussig shunt correction surgery
o Subaortic VSD/doubly committed VSD
• ปด VSD ดวย patch/graft
• แกไข PS (เหมอน TOF)
o Subpulmonic VSD/remote VSD
• ปด VSD แบบ intraventricular tunnel
• Homograft valve conduit ระหวาง RVOT, MPA
o DORV with PS with restrictive VSD
• ขยาย VSD intraventricular tunnel repair
Tricuspid Atresia with Pulmonary Stenosis
Tricuspid Atresia with Pulmonary Stenosis
• SVC/IVC RA TR obstruction need
associated lesion RV
• Associated lesion
o PDA
o PFO
o ASD
o VSD
o Others : TGA, PS
.
Normal related Great arteries
d-TGA
PA PS No PS
Sings & Symptoms • Severity depends on PBF (ถามากจะเขยวนอย)
• Cyanosis and dyspnea in 1st month after birth
• Hepatomegaly
• Heart sound :
o Single S2
o SEM at LUSB (PS/PDA/relative PS)
o SEM mid LSB (restrictive VSD)
Investigation
• Chest X-ray
o Prominent right atrial border
o Decreased pulmonary vasculature
• EKG
• Echocardiogram o RAE, PFO/ASD, atrial septum โปงไปทางซาย
o Hypoplastic RV
o Tricuspid valve ไมมรเปดลงไป RV
EKG
Left superior QRS axis
RAE, LVH
Treatment • Supportive treatment
• Treat hypoxic spell
• PGE1
• เลอดไปปอดนอย (PA/PS), เขยวมาก : systemic to pulmonary
shunt
• Age 6 month
o Cardiac catheterization : PVR, PAP evaluation
o Bidirectional superior cavopulmonary connection (Bidirectional Glenn shunt) : SVC PA
• Age 3 year : Fontan type operation : SVC, IVC PA
Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)
PA/IVS
• 1% of CHD
• Well-formed RV,
tricuspid valve
• Inflammatory process at pulmonic valve
fibrosis
• Hypoplastic RV
• Need PDA, PFO
• Tripatite : TR รวมบอย
• Severity : PDA, PVR
2. Outlet
1. Inlet
3. Trabecular
DA
RVO obstruction
RV systolic
pressure
แรงดนยอนกลบจาก RV ไปCoronary sinusoid
pressure injury effect
to coronary a.
coronary a.:
Intimal, medial
thickening
Proximal coronary
a. stenosis
Myocardium
Sings & Symptoms • Cyanosis and dyspnea in a few hours/days after
birth
• Normal lung sound
• Hepatomegaly (flow ผาน PFO ไมสะดวก RA pressure สง)
• Heart sound :
o Single S2
o Most no murmur
o Systolic murmur (flow ผาน PDA ทก าลงจะปด)
o PSM/SEM of tricuspid regurgitation นอย
CXR
• Normal heart size
• Decreased PVM
• Concave
Pulmonary trunk
• Prominent RA
border
Left axis deviation, RAE, LVH
Echocardiogram • RAE
• Hypoplastic RV
• Atrial septum โปงไปทาง LA
• Right to left shunt ผาน PFO
• TR
• Coronary sinusoid
• Ventriculocoronary artery connection in RV
• PA, small MPS/RPA/LPA, PDA
• Right to left shunt ผาน PDA
Treatment • PGE1 0.05-1 mcg/kg/min
• Definitive repair : เลอกตามลกษณะ RV
o Biventricular repair
o Univentricular repair
o One and a half ventricle repair
o Transplantation
• Catheter intervention o Tripatite, tricuspid valve, RV ขนาดใหญพอ
Single Ventricle with Pulmonary Stenosis
Single Ventricle
• 1% of CHD
• ความผดปกตของการเคลอนยาย AV canal
• Double inlet ventricle
Signs & Symptoms • Hypoxia cyanosis dyspnea
• Severity depends on : severity of PS
• PE :
o Single S2
o SEM at LUSB (PS murmur)
Investigation • CXR
o Normal heart size
o Normal/decreased pulmonary vasculature
• EKG
o Superior axis deviation (common AV valve)
o Ventricular hypertrophy
o Abnormal Q wave
• ECHO
o Ventricular morphology
o AV valve
o Associated cardiac anomaly : e.g. PS, PA
Right superior axis
Deep S in V1, V2, V4
Deep Q in V5, V6
Treatment • PGE1
• Surgery o Systemic to pulmonary artery shunt
o Bidirectional Glenn operation at 6-8 month
o Fontan type operation at 3-5 year
Ductal Dependent “Systemic Blood Flow”
Ductal Dependent Systemic Blood Flow
• Critically obstructed systemic circulation
• Neonate who present with signs of poor systemic
circulation need to be rapidly evaluation and
categorized as having CHD or other diagnosis such
as sepsis or metabolic disease
• Neonate with congenital obstructive left heart
syndrome or left ventricular outflow tract will usually
present in the first few weeks of life as their ductal
dependent systemic circulation become compromised with the closure of the PDA
Ductal Dependent Systemic Blood Flow
• Finding in neonates with cardiogenic shock
o Tachycardia
o Hypotension
o Diaphoresis
o Poor perfusion (dusky, mottled)
o Oliguria
o Acidosis
Management • Maintain an airway (they can quickly tire and
apnea)
• Place the patient on oxygen and cardio-pulmonary
monitoring (be sure to have a bag valve mask and
oxygen already flowing)
• Vital signs should include pre & post ductal pulse
oximetry and blood pressure
• Immediate two IV/IO access points
Management • When concerned about cardiogenic shock only 10
mL/kg of crystalloid should be initially be given
• EKG and portable CXR should be done as soon as
possible
• Bedside initial lab : glucose, CBC, basic metabolic
panel, blood culture and ABG
• Hyperoxia test
Management • Neonate who thought to have a ductal dependent
lesion is to immediately start an infusion of PGE1 to
re-open/maintain the ductus 0.05-0.1 mcg/kg/min
IV route
o Major side effect is apnea
• If not impossible to immediately rule out septic
shock, antibiotics should be given as soon as
possible. With plan LP as soon as the pt. is stabilized
• Consult pediatric cardiologist and echocardiogram
Differential Diagnosis
• Aortic stenosis
• Coarctation of the aorta
• Interruption of the aortic arch
• Hypoplastic left heart syndrome (HLHS)
Aortic Stenosis
Critical Aortic stenosis in NB
เลอดไป aorta นอย LA pressure สง
Pulmonary congestion
เลอดบางสวนผาน foramen ovale
ม ductus arteriosus เลอดจาก Pulmonary artery จงไป aorta ได
ทารกจงม cardiac output เพยงพอ ไมเหนอย
Rt to Lt shunting
Clinical Manifestation
• Critical AS in neonate
o Tachypnea
o Irritable
o Poor feeding
o Cold extremity
Physical Examination
• Systolic thrill at RUSB, suprasternal notch, carotid
artery both side
• SEM at LUPSB, RUPSB radiate to neck
• Valvular AS : systolic ejection click จาก stenotic aortic
valve
• Supravalvular AS : BP Right arm > Left arm and lower
extremities (Coanda effect)
• Subvalvular AS : hypertrophic valve cusp Aortic regurgitation diastolic blowing murmur at LUPSB
Investigation
• ECG:
o Non severe AS : normal EKG or LVH
o Severe AS : LVH and strain pattern
• Chest X-ray
o Cardiomegaly
• Echocardiogram
• Cardiac catheterization
Treatment • Valvular AS :
o Asymptomatic Mild Moderate AS : observe
• if doppler echo peak pressure gradient ≥ 70 mmHg or
• peak velocity > 4.2 m/s
o Severe valvular AS
• Start with balloon aortic valvuloplasty
• Surgical if cannot or fail cardiac cath
Cardiac
catheterization
and balloon aortic valvuloplasty
Indication for Balloon aortic valvuloplasty
1. critical AS in neonate
2. peak gradient > 60 mmHg
3. Symptoms (angina,
syncope, and/or dyspnea on
exertion) and a transvalvular peak-
to-peak aortic valve gradient at
catheterization greater than 50
mmHg
Treatment • Supravalvular AS and subvalvular AS : only surgery
• Valvular AS :
o Normal size of aortic annulus : open aortic valvulotomy
under inflow occlusion or under cardiopulmonary bypass
o Small aortic annulus : Ross-Konno or Ross’s operation
• Supravalvular AS :
o Dilate ascending aorta under cardiopulmonary bypass
• Subvalvular AS : o Myectomy
Coarctation of The Aorta
Development of Coarctation
Development of Coarctation
• Abnormal development of left 4th and 6th aortic arches
• No real impact prior to birth due to presence of PDA unless there is fetal closure
Development of Coarctation
Ductus tissue theory Hemodynamic theory
• Due to a migration of ductus smooth muscle cells into the periductal aorta with subsequent constriction and narrowing of the aortic lumen
• Evident when ductus closes
• Reduced intrauterine
blood flow causes
underdevelopment
of aortic arch
• Results from reduced
volume of blood flow
through the fetal
aortic arch and isthmus
Pathophysiology
Fetus LV pressure=RV
pressure
Due to ductus arteriosus
Neonate ductus arteriosus
closed
LV pressure increase
LV pressure overload
proximal artery HT
Upper ext. hypertension
Lower ext. hypotension
Ductus closure
LV pressure overload
: immediately
Pulmonary
congestion
low output : distal to
coarctation
Severe HF with
oliguria
Clinical Manifestation
• Asymptomatic
o Persistent patent ductus arteriosus (PDA)
o Not severe coarctation
• Pale, irritable, diaphoretic, and dyspneic with absent femoral pulses
• Heart failure and/or shock when the PDA closes
o Severe coarctation
Physical Examination
• Absent or delayed femoral pulse (when compared
with the brachial pulse).
• Murmur
o May associated with other cardiac defects
• Differential cyanosis
o Severe coarctation of the aorta
o Large PDA with a right-to-left shunt into the descending
thoracic aorta
• Hepatomegaly
Investigation
• ECG:
o Isolated coarctation normal EKG or LVH
o Coarctation with VSD and PDA RVH or combined
ventricular hypertrophy
• Chest X-ray
o Cardiomegaly
o Figure of 3
o E sign or reverse 3 sign (Barium swallow)
• Echocardiogram
• Cardiac catheterization
Treatment • Medical treatment
o Clinical heart failure : inotropic drug, diuretic drug before surgery
• Surgical treatment o Asymptomatic isolated coarctation if hypertension
• Coarctation repair
• Balloon angiography
• Coarctation stenting
o Coarctation with VSD
• small VSD : coarctation repair alone
• Large VSD : coarctation repair with pulmonary artery banding
• Staged repair
Interrupted of The Aortic Arch
LV Prox. IAA
RV PA PDA Distal IAA
O2 sat upper ext. > lower ext.
PDA closure =>
Decrease
pulmonary vascular
resistant
Increase pulmonary
blood flow BP upper>lower ext.
Large VSD RV pressure = LV pressure
PDA closure
Decrease pulmonary
vascular resistant
Increase pulmonary
blood flow
Heart failure and
Pulmonary congestion
Lower extremities - Low output
- Acidosis
- Oliguria
Clinical Manifestation
• When PDA was closed interrupted aortic arch may
present with
o Dyspnea
o Tachypnea
o Cold extremities
Physical Examination
• Tachypnea
• Dyspnea
• Tachycardia
• Weak or absent femoral pulses
• Differential cyanosis
• Crepitation both lung
• S2P loud
• SEM grade 2-3 at mid left sternal border
• Hepatomegaly
Investigation
• EKG
o RVH (upright T wave and tall R wave)
• Chest X-ray
o Cardiomegaly
o Increased pulmonary vascular marking
o Pulmonary venous congestion
• Echocardiogram
• Cardiac catheterization
Treatment • Medical treatment
o Clinical heart failure : inotropic drug, diuretic drug before surgery
• Surgical treatment
Hypoplastic Left Heart Syndrome (HLHS)
LA PFO RA
RV PA PDA aorta
Mixed blood
PDA closure
Decrease pulmonary
vascular resistant
Increase pulmonary
blood flow
Decrease systemic
blood flow
Low output&Shock
Clinical Manifestation – With ASD
• Typically have a "honeymoon" period (adequate
systemic perfusion)
• As the PDA begins to close, infants become
symptomatic
o Systemic perfusion : diminished peripheral pulses
o Pulmonary blood flow hypotension, acidosis, and
respiratory distress
o Symptoms can rapidly progress from cyanosis, increased
respiratory distress, and poor feeding to heart failure and
cardiogenic shock
Clinical Manifestation – Without ASD
• Severe cyanosis and respiratory distress
• Quickly develop cardiogenic shock and die
Physical Examination • Tachypnea
• Dyspnea
• Tachycardia
• Weak or absent peripheral pulses
• Hypotension
• Blood pressure upper = lower ext.
• Mild cyanosis
• Crepitation both lungs
• Active precordium
• Single and loud second heart sound
• SEM grade1-2 at left sternal border
• Hepatomegaly
Investigation
• EKG
o RVH (m/c) or LVH (rare)
• Chest X-Ray
o Cardiomegaly
o Pulmonary venous congestion
• Echocardiogram
• Cardiac catheterization
Treatment • Medical treatment
o Clinical heart failure : inotropic drug, diuretic drug before surgery
o Avoid oxygen if no indication (can cause pulmonary
vasodilation)
• Surgical treatment
o The three steps consist of the following procedures:
• Norwood procedure
• Bi-directional Glenn operation
• Fontan operation
Ductal Dependent “Mixed Blood Flow”
D-TGA
D-TGA
RT Systemic Lung LT
Parallel circulation
Aorta PA
PDA
ASD
VSD
I-TGA
RA LA
Corrected TGA
Aorta PA LV RV
Systemic Lung
Decrease pulmonary
vascular resistant
Decrease PA pressure
Aorta PDA PA
Lung
Oxygenated blood LA
If PDA closure
Progressive cyanosis
LA PFO RA RV
aorta systemic
VSD
Ventricular systole Rt Lt
Ventricular diastole Lt Rt
Bidirectional flow by VSD
Clinical Manifestation
• Cyanosis
• Tachypnea
• Signs of congestive heart failure
Physical Examination
• Simple TGA
o Tachypnea with cyanosis
o Single S2
o SEM at LUPSB
• TGA with VSD
o Tachypnea with cyanosis
o Single S2
o SEM at LUPSB
o Crepitation both lungs (heart failure)
Investigation
• EKG : RVH
• Chest X-ray
o Cardiomegaly
o Pulmonary venous congestion : increase pulmonary
vasculature
o Egg on a string
Investigation
• Echocardiogram
• Cardiac catheterization
Treatment • Medical treatment
o If cyanosis after administer PGE1
o Balloon atrial septostomy (BAS)
o Clinical heart failure
• Inotropic drug
• Diuretic drug
Treatment
• Surgical treatment
o All patient
o Atrial switch operation
(ASO)
Total Anomalous Pulmonary Venous Return (TAPVR)
Lung bud
Intraparenchymal pulmonary v. เชอมกบ
common pulmonary v. (ยนจาก LA)
• Splanchnic plexus lung bud
• Venous drainage : Umbilico -
vitelline & cardinal venous
system
Pulmonary vascular bed
• Pulmonary plexus & splanchnic
plexus obliterate • Pulmonary venous drainage ตอกบ
common pulmonary vein
Common pulmonary vein รวมกบ LA
Pulmonary vein เทเขา posterior wall ของ LA
TAPVR
Cor triatrium
Cardiac type Cardiac type Supracardiac
Infracardiac
Mixed
Signs & Symptoms • TAPVR without pulmonary venous obstruction
o แรกคลอดเหมอนปกต แต SpO2 90%
o 3-6 month : เขยว หายใจเรวแรง น าหนกขนนอย ตดเชอทางเดนหายใจบอยๆ
o Mild cyanosis, undernourished, tachypnea, dyspnea
o Tachycardia bulging precordium with palpable right
ventricular impulse
o Widely fixed split S2 , louder S2P, SEM gr 2-3/6 at LUSB, S3
gallop + diastolic rumbling murmur at LLSB
o hepatomegary
Signs & Symptoms • TAPVR with pulmonary venous obstruction
o เขยว หายใจเหนอยเมอแรกคลอด
o Moderate - severe cyanosis, tachypnea with retraction
o Lungs: crepitation
o Loud S2P ± murmur (Systolic murmur at LSB)
o Hepatomegaly
CXR
• TAPVR without
pulmonary venous obstruction o Cardiomegaly
o Increased PVM
EKG • TAPVR without pulmonary venous obstruction
o RVH : rSR’/ tall R in right chest lead
o RAE
• TAPVR with pulmonary venous obstruction
o RVH : T upright in V4R, V1/ tall R in right chest lead
Echocardiogram • RAE
• RVH
• Atrial septum โปงไปทาง LA
• ไมพบ pulmonary vein เทเขา LA แตเทเขา SVC/IVC/Coronary
sinus
• Right to left flow ผาน PFO
• Pulmonary venous confluence ผานเขา systemic vein
Treatment • Medical treatment : ควบคม heart failure
o TAPVR without PVO : digitalis, diuretic
o TAPVR with PVO, pulmonary edema : ETT on ventilator ให
O2, PEEP
• Surgical treatment o เชอม pulmonary vein & LA โดยให anastomosis กวางทสด
o TAPVR with PVO : ควรผาอยางรบดวน
o TAPVR without PVO : ผาเมอผปวยพรอม ทงไวนานจะ heart fail รนแรงขน
Reference
Reference
Reference