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8/3/2019 Cyanotic Heart Lesions
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Cyanotic Heart Lesions
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
Dr. Kalpana MallaMBBS MD (Pediatrics)
Manipal Teaching Hospital
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Cyanotic Heart Lesions
The 5 Ts
Tetralogy of Fallot
Transposition of the Great Arteries Truncus Arteriosus (Persistent)
Tricuspid Atresia
Total Anomalous Pulmonary Venous Return(TAPVR)
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Cyanotic Heart Lesions
Hypoplastic left heart syndrome (HLH)
Pulmonary atresia (PA) / critical PS Double outlet right ventricle (DORV)
Ebstein anomaly
Single ventricle
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RpL
RpL with pulmonary stenosis
TOF
Tricuspid atresia Ebsteins anomaly
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Evaluation possible congenital heart
Exam: rate, rhythm, impulse, murmur, pulses
(brachial and femoral)
Oxygen saturation - Hyperoxia test ABG
Chest X- ray
Echocardiogram
Cardiac catheterization
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Tetralogy of Fallot (TOF):
Most common (75% )cyanotic CHD in >2yrs
~ 10% of all CHD
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Tetralogy of Fallot TOF = consists
Ventricular septal defect
Rt ventricular outflow obstruction infundibular or
infundibular + pulmonary valve stenosis
Aorta position is shifted to the right and over-rides the VSD
Hypertrophy of the right ventricle
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Essential components:
VSD
Pulmonary stenosis
Other components :overriding of Aorta
RVH
Pentalogy of Fallot: all above + ASD
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Hemodynamics:
Large, non-restrictive VSD, perimembranous type,
extending upto right ventricular outlet allows
equalisation of pressures in two ventricles VSD is
silent
Pulmonary stenosis Shunting of blood from RpL
ventriclemixing of oxygenated & deoxygenated
blood in left ventricle circulated to whole body
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Severity depends upon degree of pulmonary stenosis
Pulmonary stenosis causes concentric rt ventricularhypertrophy without cardiac enlargement & rt vent
pressure
Flow from Rt vent to pul artery across pul stenosis produce ejection systolic murmur
I
f obstruction small, R
L shunt minimal or absent(pink or acyanotic TOF)
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P2 Delayed & reduced in intensity due to rt
vent outflow obstruction reduced PA pressure
S2
single and A2 audible Severity of cyanosis directly proportional to
severity of pul stenosis but intensity of systolic
murmur inversely related to severity of
pulmonic stenosis
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Clinical features:
May become symptomatic any time after birth usually 2nd half of 1st yr
Anoxic spells (synonyms- hypoxic,hypercyanotic, blue, tet ) paroxysmal attackof dyspnea
Common symptoms dyspnea on
exertion,exercise intolerance Cyanosis
H/O squatting during dyspneaic episodes
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Anoxic spells
Occur predominantly after waking up or
following exertion
Most commonly start around 4 to 6 months ofage and are charcterized by
1.Sudden crying
2.Sudden onset or deepening of cyanosis3.Sudden onset of dyspnea
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Anoxic spells
4. Alterations of consciousness
5. Convulsions
6.Decrease in intensity of systolic murmurFrequency varies from once in a few days
to numerous attacks every day
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Mild outflow obstruction: cyanosis in later part of 1st year
Severe outflow obstruction: cyanosis immediately afterbirth (as ductus starts to close)
CCF unusual in children with TOF except in:
Severe anemia
Valvular regurgitation
Infective endocarditisSystemic hypertension
Coincidental myocardial diseases
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Physical examination:
Cyanosis
Clubbing
Polycythemia
Normal sized heart
Mild parastrnal impulse
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Auscultation
S1 normal
S2 Single, (A2 heard,P2 soft &delayed
Murmur
Shunt absent
Flow - Loud short pulmonary ejection systolic
murmur grade 3-5/6 at 3rd
ICS in left side
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Diagnosis: Blood: polycythemia CXR:
1. Upturned apex (RVH)- Small boot shaped
heart2. Oligemic lung fields
3. Absence or concavity of pulmonary arterysegment gives the shape described as cor-
en sabot
4. Right aortic arch ~25 -30%
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Diagnosis:
ECG:
RAD, RVH with tall peaked p waves
Echo: overriding aorta,RVH,outflow
obstruction
Cardiac catheterisation
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Complications:
Cerebral thrombosis
Brain abscess
Bleeding diathesis
I
nfective endocarditis
CCF-in acyanotic or pink TOF
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Complications:
CNS - Embolism to CNS - sluggish circulation
from polycythemia
Hemiplegia - infarction in CNS during anoxicspell
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Management:
Management of Tet spells:- knee-chest position
- humidified O2 inhalation
- morphine 0.1 mg/kg s/c/ iv
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Management of Tet spells:
- IV fluids
- Correct metabolic acidosis- Na-bicarbonate- Propanolol 0.1mg/kg/iv during spell (0.5-1
mg/kg PO 6 hrly
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Management:
Vasopressors- methoxamine IM or IV drip
penylephrine Correct anemia
Consider surgery
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General measures:
- Correction of iron deficiency anemia
- Adequate hydration
- Antibiotics for infection
- Prophylaxis with propranolol
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Surgery
Palliative surgery:
- Blalock-Taussig shunt subclavian artery to
pulm artery- Potts shunt-descending aorta to PA
- Waterson operation ascending aorta to
Rt pulm artery-Modified Blalock-Taussig shunt
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Corrective surgery: open heart surgery for
closer of VSD
- resecting the infundibular obstruction PSSurgery can be performed at any age
Success 85-90%
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Complications of surgery
Complete heart block
RBBB
Residual VSD & Pulm stenosis Pulm regurgitation
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Tricuspid atresia
Cong absence of
Tricuspid valve
Rt ven hypoplastic
Absent inflow portion
2% of CHD
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Hemodynamics
No communication between Rt atrium rt
ventricle (hypoplastic)
Blood from Rt atrium
lt atrium throughpatent foramen ovale or ASD.mixing of
oxygenated + deoxygenated blood to lt
ventricle aorta
Lt vent rt vent there is VSD pul artery ( lt
.ventricle maintains both systemic &
pulmonary circulation saturation of blood is
identicle in pulm artery and aorta
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Clinical features
Depends on state of pulmonary flow
90 % are with diminished blood flow
Features :As TOF
Differentiating points :
1.Cyanosis from birth
2.More sicker than TOF
3.Lt ventricular type of apical impulse
4.Enlarged liver with presystolic pulsations
5.ECG- LAD,LVH
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Diagnosis
Blood: polycythemia CXR:1.Oligemic lung fields
2.Left ventricular configuration3.Prominent SVC shadow ECG:
Rt & Lt atrial hypertrophy, LAD,LVH
Echo: large single ventricular cavity
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Tricuspid Atresia
Repair not usually performed in neonatal
period- over a series of procedures
Systemic to PA shunt SVC to PA shunt (followed by ligation of first
shunt) Glenn Shunt
IVC to PA shunt completion Fontan
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Ebsteins Anomaly
Rare CCHD
Post and septal leaflet of TV displaced downwards
The upper part of the right ventricle is part of theright atrium - atrialized rt ventricle
Rt ventricle is too small and Rt. atrium is too large.
Leaflets malformed and fused obstruction offlow to rt ventricle
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Ebsteins Anomaly
Often Associated with other heart lesions
ASD
Pulmonary Stenosis Pulmonary Atresia
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Hemodynamics :
Abnormal leaflets obstruction to forward
flow & regurgitation from Rt ven to Rt
atrium atrium dilates Patent FO / ASD
allows R L shunt( cyanosis) Lt atrium
(enlarged) Lt ventricle (enlarged &
hypertrophied)
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Clinical picture
Cyanosis
Effort intolerance
Fatigue Paroxysmal attack of tachycardia
Clubbing
Lt ventricular apical impulse Systolic thrill may be palpable LSB
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Auscultation
S1- normal
S2 widely split but variable
Rt ventricular 3rd
soundor rt atrial 4th
soundaudible triple/quadruple sound usually
heard
Murmur-midsystolic ejection or pansystolic
Short tricuspid delayed diastolic M
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Investigations
CXR cardiomegaly square shaped
Lung oligemic
ECG- p pulmonale pmitrale,RBBBWolff Parkinson white type conducton defect
maybe seen
ECHO- displaced tricuspid valve
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Treatment
Surgical obliteration of atrialised portion of
rt.ventricle and repairof tricuspid valve
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Fallots physiology
Presence of large VSD with PS
Useful for bedside identification of group of
condition with similar clinical findings
Defects with Fallots physiology:
1. Complete TGA with VSD & PS
2. DORV with PS & large VSD (subaortic)3. Tricuspid atresia with diminished pul flow
4.Single ventricle with PS
5. corrected TGA with VSD & PS
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Transposition of the Great Arteries
Most common cyanotic condition thatrequires hospitalization in first 2 weeks oflife
Aorta arises from RV
Pulmonary artery originates in the leftventricle
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Oxygenated pulmonary venous blood
recirculates in lungs and systemic venous
blood recirculates in systemic circulation
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Transposition of the Great Vessels
A PDA,ASD,VSD, is necessary for these infants to
survive until they can have corrective surgery
More common in infants of diabetic mothers
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Classification
1. Complete variety
2. Physiologically corrected type
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Complete variety
Rt atrium Rt ventricle aorta
Lt atrium Lt ventricle pulmonary artery
Systemic & pulmonary circulation separate
survival possible only if there isASD,VSD,PDA
Classification
A) With intact ventricular septum mixing site is
atrial communication PFOB) with VSD with/without pul stenosis
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Physiologically corrected type
Rt atrium morphologically inverted left
ventricle pulmonary artery
Lt atrium morphologically inverted Rt
ventricle aorta
Route of blood flow is normal
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C /F with intact VS
Cyanotic at birth
Interatrial mixing poor (PFO) rapid breathing,congestive failure due to hypoxia within 1st wk of life
CCF
S1 normal
S2- single
Ejection systolic murmur grade 1-2/6
CXR egg on side appearance,plethoric lung field
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With VSD
Good mixing at ventricular level, large
pulmonary blood flow cyanosis milder
CCF at 4-10 wks
Exam cyanosis,CCF
S1- Normal
S2 single Murmur ejection systolic grade 2-4/6
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Diagnosis
CXR- egg on side appearance ,cardiomegaly
with narrow base, plethoric lung field
ECG without VSD RAD,RVH
ECG with VSD RAD, biventricular
hypertrophy
Cardiac catheterization
Angiocardiography
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Medical management
Control CCF
Balloon atrial septotomy by cardiac catheterization -
Inter-atrial septum opened
Definitive repair Jatenes switch operation -
removal of aorta and pulmonary artery from their
origins and re-attached to the correct ventricles
Less preferred atrial switch operation mustard or
senning
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Corrected TGA
Normal route of blood flow
Commonly associated with other anomalies
98% - symtoms are due associated anomalies:
1.VSD with/without PS
2.Lt sided Ebsteins anomaly of tricuspid valve
3.Atrioventricular conduction abnormalities
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Truncus Arteriosus
Truncus fails to divide completely during fetal life,leaving a connection between the aorta andpulmonary arteries
Mixed oxygenated and de-oxygenated blood exits theheart and enters the systemic circulation
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Truncus Arteriosus
Single artery arises from the heart, supplying both aortaand pulmonary artery.
VSD below the truncal valve allows mixing of right and leftventricular blood
Degree of cyanosis is variable Presents with progressive heart failure
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Truncus Arteriosus
Medical Management
Digoxin and Diuretics
Surgical Repair
Usually required by 2-3 months of age
VSD is closed
PA trunk is separated from truncus
Conduit created between RV and PA using a
valved graft
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TAPVR
The pulmonary veins, instead of being connected to
the left atrium , are connected to the right atrium
or superior vena cava, and return oxygenated blood
to the right side of the heart.
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Total Anomalous Venous Return
Uncommon CCHD
Cyanosis
CCF at age 4-10 wks Murmur : pul ejection systolic + tricuspid flow
murmur
Continuous venous hum audible at upper leftor rt sternal border or in suprasdternal notch
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Diagnosis
CXR- snowman or figure of 8 configuration
ECG RAD,RVH,
ECHO- demonstrate abnormal course of pulveins
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Total Anomalous Venous Return
Control of CCF, pul infections
The only accepted treatment is surgery
Surgical connection is made between pulmonaryvenous confluence and the LA
Connection to systemic venous circulation is
ligated.
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Hypoplastic Left Heart
Fatal without early surgical
intervention
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Treatment- continued
General procedure for cyanotic heart lesionsinvolves a systemic to PA shunt.
Procedure known as the Blalock-Taussig shunt
Uses a small Gore-Tex shunt to connect eitherleft or right subclavian to left or right branch PA.
Allows partially desaturated blood to enter PA,increasing pulmonary blood flow and oxygenation
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CCHD with PA HTN
This group is named Eisenmenger syndrome
severe PA HTN resulting in RL shunt at
atrial, ventricular or pulmonary arterial level
Eisenmenger complex severe PA HTN with
VSD resulting in RL shunt
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Eisenmenger's syndrome named by Dr. Paul
Wood after Dr. Victor Eisenmenger, who first
described the condition in 1897.
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Hemodynamics
LR shunt in the heart causes:-
- increased flow through PA
- High O2 saturation in PA
- Hyperreactive pul vasculature Pul
vascular obstructive Ds PA HTN
PA HTN causes increased pressures in the right
side of the heart and reversal of the shunt into R Lshunt
R L shunt with VSD & PDA decompresses rt
ventricle RV has only concentric hypertrophy with
no increase in size ( no heave)
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R L shunt with ASD RVH +dilatation rtven failure
R L with ASD or VSD mixing of blood
reaches ascending aorta distributed towhole systemic circulation equal cyanosis
R L with PDA mixed blood directeddownwards to descending aorta (junction isdistal to lt Subclavian artery cyanosis +clubbing of toes only (differential cyanosis)
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Examination
Cyanosis
Clubbing
Fatigue Effort intolerance
Dyspnea
h/o recurrent chest infection
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Sounds
S1- normal
S2- ASD- wide fixed split
VSD- single PDA- normally split
Murmurs
Pulmonary regurgitation ( graham steel) Ejection systolic
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Investigations
CXR- prominance of pul artery,heart size
normal to large
ECG RVH ECHO-
Cardiac catherization-bi-directional shunt
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Treatment
Heart-lung transplant is required to fully treat
the syndrome
If this option is not available - treatment is
palliative-
Anticoagulants
Pulmonary vasodilators
Antibiotic prophylaxis to prevent endocarditis
Phlebotomy to treat polycythemia
Maintaining proper fluid balance
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