Cyanotic Heart Disease (TOF)
Q&A COUNT: 32*
Relevant findings to this case:
History:
Child was playing in morning actively (or) Child had a fever (or) Child cried for some underlying LRI (or) Child was defecating in morning (these are triggers increasing sympathetic activity)
following which
+
she/he became BLUISH* and developed immediate loss of consciousness and fell down (or) became BLUISH* and suffered a seizure or upward rolling of eyes and fell down unconciousness (cyanotic spell)
Other history:
A/N history - any drug intake
Any family history?
H/O seizures, H/O nausea, vomiting, any history suggestive of focal neuro deficit - can indicate brain abscess
To rule out CCF:
1. No H/O suck rest suck cycle - no CCF
2. No H/O Forehead sweating - no CCF
3. No dyspnea - no CCF
4. No orthopnoea, PMD history - no LVF
5. No H/O recurrent LRI
Examination:
1. Edema absent, pan digital clubbing + cyanosis
2. External markers of CCF and Congenital heart disease/down's syndrome should be inspected!
3. CVS - Apical impulse normal and location of apex based on age; Murmurs depending on underlying disease (ESM in left 3rd/4th parasternal space); loud P2 indicates pulmonary hypertension (in our cases, its usually not seen)
NADAS CRITERIA FOR HEART DISEASE in children
Major criteria:
1. Systolic murmur of grade 3 or more
2. Diastolic murmur
3. Cyanosis
4. Congestive cardiac failure
Minor criteria:
1. Systolic murmur of grade 1 or 2
2. Abnormal S2
3. Abnormal Chest X Ray
4. Abnormal ECG
5. Abnormal Blood pressure
*Any 1 major criteria or 2 minor criteria - diagnostic of heart disease in children
TETRALOGY OF FALLOT - Name 4 components:
1. Right ventricular outlet obstruction (Pulmonary stenosis)
2. This makes RV to pump hard causing "Right Ventricular hypertrophy)
3. VSD
4. Over-riding of aorta
Name some Cyanotic Heart Diseases
T - Tetralogy of Fallot
T - Tricuspid atresia
T - Transposition of Great Vessels (TGA)
T - Total Anomalous Pulmonary Venous Connection (TAPVC)
Pulmonary artery hypertension, Eisenmenger syndrome and Ebstein anomaly
HISTORY:
Q&A COUNT: 32*
Relevant findings to this case:
History:
Child was playing in morning actively (or) Child had a fever (or) Child cried for some underlying LRI (or) Child was defecating in morning (these are triggers increasing sympathetic activity)
following which
+
she/he became BLUISH* and developed immediate loss of consciousness and fell down (or) became BLUISH* and suffered a seizure or upward rolling of eyes and fell down unconciousness (cyanotic spell)
Other history:
A/N history - any drug intake
Any family history?
H/O seizures, H/O nausea, vomiting, any history suggestive of focal neuro deficit - can indicate brain abscess
To rule out CCF:
1. No H/O suck rest suck cycle - no CCF
2. No H/O Forehead sweating - no CCF
3. No dyspnea - no CCF
4. No orthopnoea, PMD history - no LVF
5. No H/O recurrent LRI
Examination:
1. Edema absent, pan digital clubbing + cyanosis
2. External markers of CCF and Congenital heart disease/down's syndrome should be inspected!
3. CVS - Apical impulse normal and location of apex based on age; Murmurs depending on underlying disease (ESM in left 3rd/4th parasternal space); loud P2 indicates pulmonary hypertension (in our cases, its usually not seen)
NADAS CRITERIA FOR HEART DISEASE in children
Major criteria:
1. Systolic murmur of grade 3 or more
2. Diastolic murmur
3. Cyanosis
4. Congestive cardiac failure
Minor criteria:
1. Systolic murmur of grade 1 or 2
2. Abnormal S2
3. Abnormal Chest X Ray
4. Abnormal ECG
5. Abnormal Blood pressure
*Any 1 major criteria or 2 minor criteria - diagnostic of heart disease in children
TETRALOGY OF FALLOT - Name 4 components:
1. Right ventricular outlet obstruction (Pulmonary stenosis)
2. This makes RV to pump hard causing "Right Ventricular hypertrophy)
3. VSD
4. Over-riding of aorta
Name some Cyanotic Heart Diseases
T - Tetralogy of Fallot
T - Tricuspid atresia
T - Transposition of Great Vessels (TGA)
T - Total Anomalous Pulmonary Venous Connection (TAPVC)
Pulmonary artery hypertension, Eisenmenger syndrome and Ebstein anomaly
HISTORY:
(Q) Mechanism of cyanotic or tet or hypoxic spells
(A) A child crying continuously in the early morning, then turns bluish - can also indicate cyanotic spell
Crying (as a trigger) -- increases sympathetic activity -- causing pulmonary spasm - this increases intensity of RIGHT to Left shunt -- Tachypnoea develops -- leads to cyanosis and hypoxia
Other triggers can be - Any illness of baby making them cry, early morning defecation, playing
(Q)How cyanotic spell and febrile seizures presentation mimic each other?
(A) After an onset of fever... a cyanotic disease baby gets LOSS OF TONE, FROTHING OF MOUTH and UPWARD ROLLING OF EYES.
This is also a CYANOTIC SPELL (not seizures)
(Q)Age of onset for cyanotic heart disease:
(A) If newborn - it can be a complex heart disease like Truncus arteriosus and TGA.
TOF never develops in newborn/ early neonate
Also, cyanotic spells in TOF takes 4-6 months age to occur.
Also TOF never produces heart failure to manifest soon.
(Q)Usual Age required to develop a cyanotic spell
(A) 4-6 months
(Q)Complications of TOF
(A) Based on their age:
<2 years - Dehydration, abscess, Tachypnoea
>2 years - Thrombotic/ bleeding complications
Long standing TOF can cause Cerebral abscess!
EXAMINATION:
(Q) Pandigital clubbing - Cause
(A) Tetraology of Fallot
(Q)Age to develop clubbing
(A) 4-6 months
(Q)Which finger usually develops clubbing?
(A) Only thumb usually develops clubbing
(Q) Inference about Acrocyanosis
(A) Common and normal in newborn - then subsides
(Q) Name the external markers of Congenital Heart disease
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| This picture is taken from Aruchamy textbook of Paediatrics |
(Q) Signs of cardiac failure
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| This picture is taken from OP Ghai textbook of Paediatrics |
(Q) What is apical impulse?
Lowermost and Outermost point of definite cardiac impulse, which gives maximum thrust to the palpating finger
(Q) How apical impulse position varies with age?
INFANCY - In the left 4th Intercostal space, just "lateral" to the midclavicular line
(then)
UPTO 5 YEARS - In the left 5th Intercostal space, along the midclavicular line
(then)
AFTER 5 YEARS & ADULTS - In the left 5th Intercostal space, just "medial" the midclavicular line
(then)
UPTO 5 YEARS - In the left 5th Intercostal space, along the midclavicular line
(then)
AFTER 5 YEARS & ADULTS - In the left 5th Intercostal space, just "medial" the midclavicular line
(Q) Murmurs in different congenital heart diseases?
ASD - ESM in pulmonary area + MDM in tricuspid area
VSD - PSM, left sternal border (at 3rd and 4th IC spaces)
PDA - Continuous Murmur (in left 2nd IC space - in Gibson's area)
TOF - ESM (Left 3rd and 4th IC space)
TOF with cyanotic spell - ESM murmur decreases with intensity or becomes absent*
VSD - PSM, left sternal border (at 3rd and 4th IC spaces)
PDA - Continuous Murmur (in left 2nd IC space - in Gibson's area)
TOF - ESM (Left 3rd and 4th IC space)
TOF with cyanotic spell - ESM murmur decreases with intensity or becomes absent*
(Q) What happens to ESM murmur during cyanotic spell of TOF?
(A) No murmurs heard during cyanotic spell
(Q) TOF causes VSD's Pan systolic murmur to not occur - why?
(A) TOF alongwith large VSD causes EQUAL PRESSURE in both chambers - there is no shunting occurs.
(Q) Second heart sound in TOF
(A) No P2 due to Pulmonary stenosis
Single and loud S2 due to over-riding of aorta (loud because aorta is more anterior towards chest wall than usual)
MANAGEMENT:
(Q) What is Hyperoxia Test?
Hyperoxia test involves measuring arterial oxygen saturation before and after supplying 100% oxygen for 10 minutes.
It is done in a case of cyanosis.
If cyanosis is due to poor lung ventilation, then condition gets improved by this test.
If cyanosis is due to congenital heart disease (Right to Left shunt), the condition will not improve despite supplying oxygen as the blood do not come to lung at all , for perfusion!
(Q) What is Hyperoxia Test?
Hyperoxia test involves measuring arterial oxygen saturation before and after supplying 100% oxygen for 10 minutes.
It is done in a case of cyanosis.
If cyanosis is due to poor lung ventilation, then condition gets improved by this test.
If cyanosis is due to congenital heart disease (Right to Left shunt), the condition will not improve despite supplying oxygen as the blood do not come to lung at all , for perfusion!
(Q) RBC count in TOF
(A) Polycythemia actually occurs, but presence of HbF prevents polycythemia to manifest
There will be relative iron deficiency due to Polycythemia. So, give IRON.
(Q) Chest, ECG and Echo findings in TOF
ECG - Right axis deviation with RVH, T wave inversion in right precordial leads, V1 may show pure R waves
CXR - Normal sized heart with upturned apex (denoting RVH), absent right pulmonary artery segment (COEUR EN SABOT appearance), aorta enlarged, oligemic pulmonary fields
Echo - VSD, aorta over-riding, RVH, RV outlet obstruction revealed
CXR - Normal sized heart with upturned apex (denoting RVH), absent right pulmonary artery segment (COEUR EN SABOT appearance), aorta enlarged, oligemic pulmonary fields
Echo - VSD, aorta over-riding, RVH, RV outlet obstruction revealed
(Q) What are the defence mechanisms against a cyanotic spell?
(A) 2-5 years: Squatting or Mom will fold leg against the chest
After 5 years: Squatting equivalents
(Q) Name some Squatting equivalents
1) Knee chest position
2) Sitting with legs drawn underneath
3) Cross the leg when standing
4) Lying down
5) Sitting on chair with flexed legs
6) Baby sitting on mother's hip with legs flexed
(Q) Name some Squatting equivalents
1) Knee chest position
2) Sitting with legs drawn underneath
3) Cross the leg when standing
4) Lying down
5) Sitting on chair with flexed legs
6) Baby sitting on mother's hip with legs flexed
(Q) How squatting or squatting equivalents stop a cyanotic spell - Mechanism?
(Q) Treatment of Cyanotic Spell
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| This picture is taken from OP Ghai textbook of Paediatrics |
(Q) Drug of choice to prevent CYANOTIC SPELL
(A) PROPRANOLOL or Beta blockers (Metaprolol)
(Q) How Beta blocker prevents Cyanotic Spell - Mechanism?
(A) Propranolol decreases symapthetic response and reduces pulmonary spasms. It also increases systemic vascular resistance. Both decreases Right to Left shunt and hence cyanosis.
(Q) Ultimate/definite treatment for TOF
(A) Surgical closure of VSD and relief of Right ventricular outlet obstruction*
Palliative surgery can be done initially followed by definite surgery later.
*IE prophylaxis is given during these surgeries
(Q) Name some Palliative surgeries
Palliative surgeries are
Palliative surgery can be done initially followed by definite surgery later.
*IE prophylaxis is given during these surgeries
(Q) Name some Palliative surgeries
Palliative surgeries are
1. Blalock Taussig shunt* (subclavian artery - pulmonary artery anastamosis)
2. Balloon dilation of pulmonary valve
3. Stenting of patent arterial duct
4. Other surgeries - Waterston, Glenn, Gore-tex, Pott's surgery
(Q) Names of definite surgery
Brock's procedure and Rastelli's operation
2. Balloon dilation of pulmonary valve
3. Stenting of patent arterial duct
4. Other surgeries - Waterston, Glenn, Gore-tex, Pott's surgery
(Q) Names of definite surgery
Brock's procedure and Rastelli's operation