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Presenter:
Dr. Anindita Bose
FCPS student
Neonatology, BSMMU.
Dr. Mostafa Kamal
Resident ( Year 2)
Paediatric Nephrology, BSMMU
Particulars of the patient
• Name : B/O Tania
• Age : 10 Days
• Sex : Male
• Address : West Dewbhog,
N Gonj
• Date of admission : 04/02/19
• Date of examination : 04/02/19
• Informant : Father
Chief complaints
H/O Delayed cry after birth
Multiple birth defects
History of present illness
Mother Tania, 31 years old, para: 4-1(IUD), having blood
group B(+) ve, fathers blood group B(+)ve, was on irregular
antenatal check up (total 3 visits) and was duly immunized
against tetanus. At 20 weeks of gestation, she was first
diagnosed as 20 weeks of alive pregnancy which was
unplanned. About 12 weeks before diagnosis of
pregnancy, she was diagnosed as coronary artery disease
(Double vessel disease) and hypertension. CABG was done
under GA on 30.05.2018 and treated with clopidogrel,
History of present illness cont.
aspirin, atorvastatin, frusemide, spironolactone,
metoprolol, riboflavin, ferrous sulphate, folic acid. She was
known case of DM for last 5 years and was being treated
irregularly with insulin, linagliptin, glicazide and continued
upto 20 weeks of pregnancy, her HbA1C was 7.4% . After
diagnosis of pregnancy she continued aspirin, bisoprolol,
insulin. She had no H/O of APH, PROM, Hypothyroidism,
fever with rash.
History of present illness cont.
At 38+4 weeks of gestation, she admitted at private
hospital, LUCS was done due to less fetal movement,
previous C/S & cardiac surgery. A male baby weighing
2300gm was delivered on 25/01/19. Baby was found
meconium stained & didn’t cry immediately after birth.
After laryngeal suction, bag mask ventilation was given,
then baby cried. APGAR score was 4/10 & 8/10 @ 1min &
5mins respectively.
History of present illness cont.
Soon after birth baby developed respiratory distress in the
form of tachypnea, chest retraction. Baby was shifted to
NICU of private hospital for delayed crying followed by
respiratory distress and multiple congenital anomalies. At
PNA day 10, baby referred to BSMMU for further evaluation
and management.
Antenatal History
At 20 weeks of gestation, she was diagnosed 20 weeks
of alive pregnancy. During this pregnancy mother
didn’t receive any ANC upto 20 weeks of gestation,
then was on regular ANC & was duly immunized
against tetanus.
At 8 weeks of pregnancy she was diagnosed as
coronary artery disease, CABG was done
She was diagnosed case of DM for last 5 years
She also diagnose case as hypertention.
Natal History
A male baby delivered by LUCS weighing
2300gm on 25/01/19, cried after giving bag
mask ventilation for 1 min, APGAR score
4/10, 8/10 at 1st and 5th min
Postnatal History
Soon after birth baby developed respiratory
distress in the form of tachypnea, chest indrawing
Immunization History
 Not started yet.
Feeding History
OG feeding with expressed breast milk
Drug History
 Antibiotic: Ceftazidime, Amikacin,
Meropenem, Vancomycin
 Frusemide injection
Family History
He is the 3rd issue of non-consanguineous
parents. Other sibs are good in health
Socio-economic History
He belongs to a middle socio-economic
background, father is a businessman, mother
is a homemaker and their monthly income is
around 35000 tk
General Examination
•Appearance: Alert
•Anicteric
•Pink on 2L/min O2
•SPO2: 93% in 2L/min O2
•Reflex-Activities: good
•CRT: 2 sec
•Pulse volume : good
•CBG: 4.5 mmol/L
•Temperature: 36.8C
R/R : 54/min
H/R : 290 beats/min
Fontanelle : Open, not bulged
 Back and spine : Normal
 Genitalia- Normal male pattern.
 Anus: Patent
 Congenital anomaly: Anotia (right),
Bilateral club foot
Anotia
Bilateral club foot
Anthropometry
Weight : At Birth 2300 g (between 25th & 50th
centile) At admission 2290 gm
Length : 44 cm (between 25th & 50th centile)
OFC : 32 cm (between 25th & 50th centile)
Systemic Examination
Cardiovascular System Examination
• Inspection:
• Visible apex beat -Absent
• Palpation
• Apex beat: left 4th intercostal space
• Left parasternal heave, thrill, palpable P2-Absent
• Auscultation:
• Heart Rate : 290 b/min
• Heart Sound: first and second heart sounds are
audible in all four areas of precordium.
• Murmur: Systolic, grade 3/6, left 2nd ICS with no
radiation
GIT System Examination
• Abdomen : Soft, not distended
• Liver – 4 cm from right costal Margin
along the mid-clavicular line, margin
sharp, smooth surface
• Bowel sound –present
• Umbilicus: Healthy
Respiratory System Examination
• RR-54 br/ min
• Chest is normal in size and shape
• No grunting, chest indrawing
• Bilateral equal good air entry
• Breath sound vesicular, no added
sound.
Nervous system
• Conscious, alert
• Posture normal
• Primitive reflexes were Good
• Tone normal
• Deep tendon reflexes normal.
Nervous system
Facial nerve examination:
 Loss of wrinkle in right side
 Eyelids on right side could
easily be opened
 Loss of nasolabial fold on
right side
 Mouth deviated to left side
during crying
Suggestive of right sided lower
motor type facial nerve palsy
 Other systemic examination reveals no
abnormality.
Salient feature
S/O Tania, outborn, 3rd issue of non-consanguineous parents, got
admitted in NICU at his 10 days of age due to delayed cry after
birth followed by respiratory distress, multiple congenital
anomalies. Mother Tania, 31 years old lady, Para: 4-1(IUD), having
blood group B(+)ve, was on irregular ANC upto 20 weeks when
she was diagnosed with 20weeks alive pregnancy. About 12
weeks before diagnosis of pregnancy, CABG was done under GA
due to coronary artery disease with hypertention. She was known
case of DM for last 5 years. She had no H/O of APH, PROM,
Hypothyroidism, fever with rash.
Salient feature
Regarding drug history of mother during this pregnancy, for
heart disease clopidogrel, aspirin, atorvastatin, frusemide,
spironolactone, metoprolol; for DM insulin, linagliptin,
glicazide. After diagnosis of pregnancy she continued aspirin,
bisoprolol, insulin. At 38+4 weeks of gestation, LUCS was
done due to less fetal movement, previous C/S & cardiac
surgery. A male baby weighing 2300gm was delivered.
Salient feature cont.
Baby was found meconium stained & didn’t cry
immediately after birth. After laryngeal suction and bag
mask ventilation baby cried. APGAR score was 4/10 &
8/10 @ 1 min & 5 mins respectively. Baby was admitted in
a private NICU for delayed crying followed by respiratory
distress and multiple congenital anomalies. There baby
was treated with O2 inhalation, injectable antibiotics,
frusemide. At PNA day 10, baby was referred to BSMMU
for further evaluation and management.
Salient feature cont.
On examination, baby was alert, anicteric, pink on 2L/M O2,
reflex activities were good, well perfused, euglycemic,
normothermic, SPO2 – 93% on 2L/min O2, RR- 54/m, HR –
290/min. Congenital anomaly- Anotia, Bilateral club foot.
Anthropometrically the baby was appropriate for gestational age.
CVS examination murmur present, Systolic, grade 3/6 left 2nd ICS
with no radiation. Nervous system examination revealed right
sided lower motor type facial nerve palsy. On abdominal
examination, liver is 4 cm. Other Systemic examination reveals
nothing significant.
Provisional Diagnosis
????
Provisional diagnosis
Term (38+4 wks), low birth weight (2300gm),
AGA, IDM, Perinatal asphyxia (HIE-1), Congenital
heart disease, tachyarrythmia, multiple
congenital anomalies (right sided anotia, right
sided lower motor type facial palsy, bilateral club
foot), suspected sepsis
Investigation ( Outside BSMMU)
Test 26/01/19 02.02.19
CBC Hb –20.1 g/dl
TC- 11320/cmm,
N= 47%, L= 40.6%
Platelet- 1,50,000/cmm
Hb –21.3 g/dl
TC- 10490/cmm,
N= 51%, L= 37.6%
Platelet- 81,000/cmm
Blood C/S No growth
Electrolyte Na 140 144
K 4.07 4.22
CL 95 100
T-CO2 24.5 24.8
S. Calcium 8.8
Mg 0.69mmol/L
TSH 0.5
Investigation (BSMMU)
Test 05/02/19 06.02.19
CBC Hb –19.8 g/dl
TC- 9000/cmm, N= 52%, L= 40%
Platelet- 1,50,000/cmm
Hct – 57.6%
PBF – Anisopoikilocytosis with target cell
IT Ratio- 0.09
CRP- 30.6
Blood CS No growth
Electrolyte Na 135 137
K 4.2 4.3
CL 95 100
T-CO2 31.5 24.8
S. Calcium 8.9mg/dl
S. Creatinine 0.79 0.6
Mg 1.6 mg/dL
Management after admission (8PM)
• O2 inhalation
• OG feeding 10ml/2hrly
• Infusion 10% DBS
• Inj Meropenem
• Inj Vancomycin
• Inj Frusemide
• Inj Digoxin
• Thermal care
Follow up at 10PM (04.02.2019)
 O/E:
 HR 280-290/min
 BP- 83/46(62)
 RR -56/min
 CRT – 2 sec
 Heart - S1+S2+ M
 Lungs – BL equal air entry, no added sound
 Assesssment: tachyarrythmia most probably SVT
 Plan: Continuous ECG monitoring
S- 72-82
D- 46-54
M- 55-63
ECG pic
Cont…
Vagal stimulation by
Ice cold compression on face
Persisting Tachycardia (HR-270-280)
Plan: Cardiology consultation
Inj Adenosine
Follow Up: 05.02.19 at 9AM
Subjective Objective Assessment Plan & Measures Taken
Tachycardia
persisting
Pink in 2L/min O2
Reflex activity- good
CRT -2 sec
R/R – 54 br/min
H/R- 280b/min
BP- 83/46(62)
SPO2- 98% in 2L/min O2
Lung- B/L equal air entry
Heart- S1+S2+ M
Abdomen- soft, not distended,
liver 2cm
Umbilicus- Healthy
CBG- 6 mmol/L
Temp- 98°F
Urine- 2.7 ml/kg/hr
Meconium- passed
Stable
S-72-82
D-46-54
M-55-63
• Cardiology consultation
• Inj Adenosine
• Echocardiography
Give Inj Adenosine
HR -150-156/M
Echocardiography
 Transposition of great artery (d-TGA), PA arising from LV,
Aorta arising from RV. Large inlet VSD (Size-6.8mm) with
bidirectional shunt. Large ostium secundum ASD (size-
7.5mm) with L-R shunt. Large PDA with bidirectional shunt.
Both dynamic and fixed obstruction of LVOT. Dilated
coronary sinus. Myxomatous change to septal leaflet ant
leaflet of tricuspid valve
Follow up on 06.02.2019
At 3.30AM
HR- 288/M
-Ensure Digoxin maintainance dose
At 9pm
HR- 250-260 b/m
MX- Inj Adenosine
HR-120-130b/min
Club foot Mx(Orthopedic consultation)
 Type: Flexible variety of Congenital Talepes Equino-
varus (Bilateral)
 Plan:
 Manipulation
 Follow up & Serial plaster after 1 month
Follow Up: 07.02.19 at 10AM
Subjective Objective Assessment Plan & Measures Taken
Unusual weight
gain (80gm)
Pink in air
SPO2- 90% in air
Reflex activity- good
Fontanelle- Open, normal
CRT -2 sec
R/R – 58 br/min
H/R- 120-130 b/min
BP- 92/53(63)
Lung- B/L equal air entry
Heart- S1+S2+ M
Abdomen- soft, not
distended, liver 2cm
Umbilicus- Healthy
CBG- 6 mmol/L
Temp- 98°F
Urine- 2.5 ml/kg/hr
Stool- 4 times
Static
S-72-82
D-46-54
M-55-63
-Decreased fluid
-Increased feed
-Inj MgSO2 (Mg -0.6)
• At 6PM, 10PM SVT
develop > Spontaneous
resolved
Follow Up: 09.02.19 at 10AM
Subjective Objective Assessment Plan & Measures Taken
Repeated
episode of SVT
Pink in air
Reflex activity- good
Fontanelle- Open, normal
CRT -2 sec
R/R – 42 br/min
H/R- 120 b/min
BP- 92/53(63)
SPO2- 84% in air
Lung- B/L equal air entry
Heart- S1+S2+ M
Abdomen- soft, not distended,
liver 2cm
Umbilicus- Healthy
CBG- 6 mmol/L
Temp- 98°F
Urine- 2.5 ml/kg/hr
Stool- 4 times
Static
S-72-82
D-46-54
M-55-63
• Cardiac Consultation
• Tab Amiodarone
(5mg/kg/day) started
from 5PM
• At 8.30PM & 10.30PM
Baby developed SVT
(spontaneously
resolved)
Follow Up: 10.02.19, 1AM
 Subjective- Sudden bradycardia (HR-70/min),
Desaturation (SPO2-74%)
 Objective-
 Pink in air
 Reflex activity-poor
 CRT - 4 sec
 R/R – Irregular
 H/R- gradually decreased to 50/m
 Pulse volume - weak
 Heart – S1+S2+M
 Assessment : Cardiac arrest
 Management :
Positioning, suction, stimulation and bag mask
ventilation , CPR started
2 shot of Inj Adrenaline was given (10unit/kg)
Baby having gasping respiration, heart rate 90b/min
Mechanical ventilation with SIMV mode in following
method - Rate-40, Pressure – 14/5, FiO2- 60%
- Dopamin, Dobutamine were added
 After that
 Pink in air
 Reflex activity-Improving than previous
 CRT - 2 sec
 R/R – 45/m
 H/R- 132/m
 Pulse volume – good
 Lungs – bilateral good air entry
 Heart – S1+S2+M
 Pupil – reacting to light
 Plan : Chest X-ray
ABG
ABG: pH-7.37
PCO2- 34
PO2- 45
HCO3- 19.4
BE- -5
Follow Up: 10.02.19 at 10AM
Subjective Objective Assessment Plan & Measures Taken
One episode of
SVT at 4.30AM
(Mx by Tab.
Amiodarone)
Pink in MV
F-40, P-14/5,Fio2-.60%
SPO2- 93%
Reflex activity- Moderate
Fontanelle- Open, normal
CRT -2 sec
R/R – 42 br/min
H/R- 140 b/min
BP- 97/63(75)
Lung- B/L equal air entry
Heart- S1+S2+ M
Abdomen- Distended, liver
3cm
Girth-29cm
CBG- 9 mmol/L
Temp- 98°F
Urine- 3.2 ml/kg/hr
Stool- 3 times
Static
S-72-82
D-46-54
M-55-63
• Decreased setup
FiO2 50%
PIP 12
• Weaning off Dopamin
• Decreased 10% to 7.5%
dextrose
• Send septic workup,
S. Electrolyte (Refused)
At 9PM wean from MV to
5L/M o2
Follow Up: 11.02.19 at 10AM
Subjective Objective Assessment Plan & Measures Taken
One episode of
SVT at 10.30PM
(Mx by Inj
Adenosine)
Pink in 3L O2
SPO2- 93%
Reflex activity- Moderate
Fontanelle- Open, normal
CRT -2 sec
Temp- 37°c
R/R – 52 br/min
H/R- 240 b/min
BP- 88/58(67)
Lung- B/L equal air entry
Heart- S1+S2+ M
Abdomen- Distended, liver 3cm
Girth-29cm
CBG-6.3
Urine- 3.2 ml/kg/hr
Stool- 3 times
Static
S-72-82
D-46-54
M-55-63
• Referred to NHF
Final diagnosis
Term (38+4 wks), low birth weight (2300gm), AGA,
IDM, Perinatal asphyxia (HIE-1), Congenital heart
disease (d-TGA, Large VSD, Large ASD, Large PDA),
Supraventricular tachycardia, multiple congenital
anomalies (right sided anotia, right sided lower
motor type facial palsy, bilateral club foot)
Our patient
S/O Tania
A baby with multiple congenital malformations
(unilateral anotia, facial nerve palsy, bilateral
club foot and congenital heart disease)
Presented with tachycardia
Heart failure
Sepsis
Excluded
ECG monitoring
Tachyarrhythmia
Most common cause of tachyarrhythmia in
newborn
Supraventricular tachycardia
(SVT)
Supraventricular Tachycardia
(SVT)
SVT
• An abnormal arrhythmia arising above or
within bundle of His.
• It includes essentially all forms of paroxysmal
or incessant tachycardia except ventricular
tachycardia.
• It is the most common abnormal tachycardia
in infancy and childhood.
TYPES
SITE RHYTHM
Sinus node Sinus tachycardia Always secondary; usually
non-cardiac cause e.g. sepsis,
fever, pain, hypovolaemia,
respiratory failure, anaemia,
fluid overload, ionotropes
Atrium Atrial flutter
Atrial fibrillation
Atrial tachycardia
Rare
Very rare
Uncommon
AV junction AV re-entry tachycardia
AV nodal re-entry
tachycardia
Commonest form in newborn
rare
Below AV node His bundle tachycardia
(junctional ectopic
tachycardia)
Associated with abnormal
hearts/cardiomyopathy;
Usually postoperative
RISK FACTORS
 Bimodal age distribution
• One peak at infancy
• Another peak is in between 8-12
years (Calabro ` MP, Cerrito M, Luzza F,
Oreto G (2008) Supraventricular tachycardia
in infants: Epidemiology and clinical
management. Curr Pharm Des 14(8):723–
728 )
 No significant gender differences
were present at younger ages
( Anand RG, Rosenthal GL, Van Hare GF,
Snyder CS (2009) Is the mechanism of
supraventricular tachycardia in pediatrics
influenced by age, gender or ethnicity?
Congenit Heart Dis 4(6):464–468)
Our patient:
 presented in
neonatal period
 Male baby
PRESENTATION
Antenatal:
• Fetal SVT (fetal tachycardia, less fetal
movement)
• Non- immune hydrops fetalis, if started early
in pregnancy
Post natal:
Asymptomatic
Tachycardia (HR: 180-300/min)
• Abrupt onset and cessation
• May occur during rest
• May be precipitated by acute infection
• Attacks may persist from seconds to hours
Heart failure
If tachycardia goes unrecognized for a long time
Shock
Our patient:
Tachycardia
with
Sudden onset &
spontaneous
remission;
Some episodes
occurred during
sleep; persisted
for minutes to
hours
Causes of SVT
• Any form of structural heart disease
• Myocardial tumors
• Myocarditis
• Electrolyte disturbances
• Indwelling right arterial lines
In our patient:
Congenital heart
defect
(murmur+)
ASSOCIATED CARDIAC
DEFECTS/SYNDROME
According to Tripathy A, Black GB (2014) Factors associated with
the occurrence and treatment of supraventricular tachycardia in a
pediatric congenital heart diseaes cohort. Pediatr cardiol 35:368-
373
• 6–7 % of children with CHD have SVT
• ASD secundum (14.3 %)
• VSD (6 %)
• Pulmonary stenosis (5.4 %)
• PDA (5.3 %)
• Pulmonary hypertension (2.4 %)
Continued….
• Two or more concomitant CHDs were present in
8.6 % of SVT patients.
• A smaller percentage (7.2 %) of patients were
diagnosed with severe/complex (cyanotic) CHDs,
i.e., tetralogy of Fallot, transposition of the great
arteries, double-outlet right ventricle (DORV),
common ventricle, truncus arteriosus,
atrioventricular canal/endocardial cushion defect
(AV/ ECD), Ebstein’s anomaly, hypoplastic left
heart syndrome (HLHS), or total anomalous
pulmonary venous return
Our patient had associated CHD
• TGA
• A Large inlet VSD (6.8 mm)
• A Large ostium secundum ASD (7.5 mm)
• A Large PDA
• Posterior deviation of conal septum producing
dynamic Left Ventricular Outflow Tract
Obstruction
This patient also had some other
congenital anomalies
• Unilateral anotia
• Facial nerve palsy
• Bilateral club foot
There was no
reported case of
SVT found to be
associated with
these congenital
malformations
DIAGNOSIS
• Clinical
• ECG
• Echocardiogram
ECG
Our Patient
SVT
Echocardiography
Congenital heart disease
PRINCIPLES OF MANAGEMENT
Conversion of rhythm:
Vagal maneuvers: Ice pack to the face until
conversion or max. 10 seconds
Adenosine, if vagal maneuver fails
Control of rate:
Propranolol (if there are no signs of heart failure)
Digoxin
Amiodarone
Management of emergency/complication:
Digoxin (if there are signs of heart failure or
propranolol has failed) after ensuring not
hypokalaemic and no WPW syndrome
Electrical cardioversion by synchronous DC
cardioversion: if the baby is in shock
Maintenance therapy:
Once sinus rhythm is restored, maintenance
therapy with whatever agent restored, is
usually given for 3- 6 months
Surgical correction
Artificial pacing
Radiofrequency catheter ablation
Our patient was treated with
Vagal maneuver:
• Ice pack to the face
Inj. Adenosine
Inj. Digoxin
Tab. Amiodarone
How Adenosine was given
 Dose: 0.1 mg/kg/dose
 Prerequisite for giving Inj.
Adenosine:
• A large fresh cannula as close to
heart as possible
• 3-way channel
 Procedure: rapid IV bolus
followed by saline flush with
continuous ECG recording
COMPLICATION
• Heart failure
• Shock
• Cardiac arrest
• Myocardial dysfunction
• Mitral regurgitation
• Dilated cardiomyopathy Our patient:
Cardiac arrest
PROGNOSIS
• Usually excellent, in absence of structural cardiac
disease or cardiomyopathy
• WPW syndrome has small risk of sudden cardiac
death
• One fifth will recur after treatment
• When SVT starts in first months of life, it resolves
in 80% cases within the first year; If SVT starts
later in life, spontaneous remission may occur in
only 15-20% cases
(Vignati G, Annoni G (2008) Characterization of supraventricular tachycardia in
infants: Clinical and instrumental diagnosis. Curr Pharm Des 14(8):729–735)
Our patient:
Prognosis is
guarded
FOLLOW UP
Clinical
Investigations:
• Digoxin level
• CBC
• Electrolytes
• ECG
Newborn with Congenital Heart Disease and Multiple Anomalies

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Newborn with Congenital Heart Disease and Multiple Anomalies

  • 1. Presenter: Dr. Anindita Bose FCPS student Neonatology, BSMMU. Dr. Mostafa Kamal Resident ( Year 2) Paediatric Nephrology, BSMMU
  • 2. Particulars of the patient • Name : B/O Tania • Age : 10 Days • Sex : Male • Address : West Dewbhog, N Gonj • Date of admission : 04/02/19 • Date of examination : 04/02/19 • Informant : Father
  • 3. Chief complaints H/O Delayed cry after birth Multiple birth defects
  • 4. History of present illness Mother Tania, 31 years old, para: 4-1(IUD), having blood group B(+) ve, fathers blood group B(+)ve, was on irregular antenatal check up (total 3 visits) and was duly immunized against tetanus. At 20 weeks of gestation, she was first diagnosed as 20 weeks of alive pregnancy which was unplanned. About 12 weeks before diagnosis of pregnancy, she was diagnosed as coronary artery disease (Double vessel disease) and hypertension. CABG was done under GA on 30.05.2018 and treated with clopidogrel,
  • 5. History of present illness cont. aspirin, atorvastatin, frusemide, spironolactone, metoprolol, riboflavin, ferrous sulphate, folic acid. She was known case of DM for last 5 years and was being treated irregularly with insulin, linagliptin, glicazide and continued upto 20 weeks of pregnancy, her HbA1C was 7.4% . After diagnosis of pregnancy she continued aspirin, bisoprolol, insulin. She had no H/O of APH, PROM, Hypothyroidism, fever with rash.
  • 6. History of present illness cont. At 38+4 weeks of gestation, she admitted at private hospital, LUCS was done due to less fetal movement, previous C/S & cardiac surgery. A male baby weighing 2300gm was delivered on 25/01/19. Baby was found meconium stained & didn’t cry immediately after birth. After laryngeal suction, bag mask ventilation was given, then baby cried. APGAR score was 4/10 & 8/10 @ 1min & 5mins respectively.
  • 7. History of present illness cont. Soon after birth baby developed respiratory distress in the form of tachypnea, chest retraction. Baby was shifted to NICU of private hospital for delayed crying followed by respiratory distress and multiple congenital anomalies. At PNA day 10, baby referred to BSMMU for further evaluation and management.
  • 8. Antenatal History At 20 weeks of gestation, she was diagnosed 20 weeks of alive pregnancy. During this pregnancy mother didn’t receive any ANC upto 20 weeks of gestation, then was on regular ANC & was duly immunized against tetanus. At 8 weeks of pregnancy she was diagnosed as coronary artery disease, CABG was done She was diagnosed case of DM for last 5 years She also diagnose case as hypertention.
  • 9. Natal History A male baby delivered by LUCS weighing 2300gm on 25/01/19, cried after giving bag mask ventilation for 1 min, APGAR score 4/10, 8/10 at 1st and 5th min
  • 10. Postnatal History Soon after birth baby developed respiratory distress in the form of tachypnea, chest indrawing
  • 12. Feeding History OG feeding with expressed breast milk
  • 13. Drug History  Antibiotic: Ceftazidime, Amikacin, Meropenem, Vancomycin  Frusemide injection
  • 14. Family History He is the 3rd issue of non-consanguineous parents. Other sibs are good in health
  • 15. Socio-economic History He belongs to a middle socio-economic background, father is a businessman, mother is a homemaker and their monthly income is around 35000 tk
  • 17. •Appearance: Alert •Anicteric •Pink on 2L/min O2 •SPO2: 93% in 2L/min O2 •Reflex-Activities: good •CRT: 2 sec •Pulse volume : good •CBG: 4.5 mmol/L •Temperature: 36.8C
  • 18. R/R : 54/min H/R : 290 beats/min Fontanelle : Open, not bulged  Back and spine : Normal  Genitalia- Normal male pattern.  Anus: Patent  Congenital anomaly: Anotia (right), Bilateral club foot
  • 21. Anthropometry Weight : At Birth 2300 g (between 25th & 50th centile) At admission 2290 gm Length : 44 cm (between 25th & 50th centile) OFC : 32 cm (between 25th & 50th centile)
  • 23. Cardiovascular System Examination • Inspection: • Visible apex beat -Absent • Palpation • Apex beat: left 4th intercostal space • Left parasternal heave, thrill, palpable P2-Absent • Auscultation: • Heart Rate : 290 b/min • Heart Sound: first and second heart sounds are audible in all four areas of precordium. • Murmur: Systolic, grade 3/6, left 2nd ICS with no radiation
  • 24. GIT System Examination • Abdomen : Soft, not distended • Liver – 4 cm from right costal Margin along the mid-clavicular line, margin sharp, smooth surface • Bowel sound –present • Umbilicus: Healthy
  • 25. Respiratory System Examination • RR-54 br/ min • Chest is normal in size and shape • No grunting, chest indrawing • Bilateral equal good air entry • Breath sound vesicular, no added sound.
  • 26. Nervous system • Conscious, alert • Posture normal • Primitive reflexes were Good • Tone normal • Deep tendon reflexes normal.
  • 27. Nervous system Facial nerve examination:  Loss of wrinkle in right side  Eyelids on right side could easily be opened  Loss of nasolabial fold on right side  Mouth deviated to left side during crying Suggestive of right sided lower motor type facial nerve palsy
  • 28.  Other systemic examination reveals no abnormality.
  • 29. Salient feature S/O Tania, outborn, 3rd issue of non-consanguineous parents, got admitted in NICU at his 10 days of age due to delayed cry after birth followed by respiratory distress, multiple congenital anomalies. Mother Tania, 31 years old lady, Para: 4-1(IUD), having blood group B(+)ve, was on irregular ANC upto 20 weeks when she was diagnosed with 20weeks alive pregnancy. About 12 weeks before diagnosis of pregnancy, CABG was done under GA due to coronary artery disease with hypertention. She was known case of DM for last 5 years. She had no H/O of APH, PROM, Hypothyroidism, fever with rash.
  • 30. Salient feature Regarding drug history of mother during this pregnancy, for heart disease clopidogrel, aspirin, atorvastatin, frusemide, spironolactone, metoprolol; for DM insulin, linagliptin, glicazide. After diagnosis of pregnancy she continued aspirin, bisoprolol, insulin. At 38+4 weeks of gestation, LUCS was done due to less fetal movement, previous C/S & cardiac surgery. A male baby weighing 2300gm was delivered.
  • 31. Salient feature cont. Baby was found meconium stained & didn’t cry immediately after birth. After laryngeal suction and bag mask ventilation baby cried. APGAR score was 4/10 & 8/10 @ 1 min & 5 mins respectively. Baby was admitted in a private NICU for delayed crying followed by respiratory distress and multiple congenital anomalies. There baby was treated with O2 inhalation, injectable antibiotics, frusemide. At PNA day 10, baby was referred to BSMMU for further evaluation and management.
  • 32. Salient feature cont. On examination, baby was alert, anicteric, pink on 2L/M O2, reflex activities were good, well perfused, euglycemic, normothermic, SPO2 – 93% on 2L/min O2, RR- 54/m, HR – 290/min. Congenital anomaly- Anotia, Bilateral club foot. Anthropometrically the baby was appropriate for gestational age. CVS examination murmur present, Systolic, grade 3/6 left 2nd ICS with no radiation. Nervous system examination revealed right sided lower motor type facial nerve palsy. On abdominal examination, liver is 4 cm. Other Systemic examination reveals nothing significant.
  • 34. Provisional diagnosis Term (38+4 wks), low birth weight (2300gm), AGA, IDM, Perinatal asphyxia (HIE-1), Congenital heart disease, tachyarrythmia, multiple congenital anomalies (right sided anotia, right sided lower motor type facial palsy, bilateral club foot), suspected sepsis
  • 35. Investigation ( Outside BSMMU) Test 26/01/19 02.02.19 CBC Hb –20.1 g/dl TC- 11320/cmm, N= 47%, L= 40.6% Platelet- 1,50,000/cmm Hb –21.3 g/dl TC- 10490/cmm, N= 51%, L= 37.6% Platelet- 81,000/cmm Blood C/S No growth Electrolyte Na 140 144 K 4.07 4.22 CL 95 100 T-CO2 24.5 24.8 S. Calcium 8.8 Mg 0.69mmol/L TSH 0.5
  • 36. Investigation (BSMMU) Test 05/02/19 06.02.19 CBC Hb –19.8 g/dl TC- 9000/cmm, N= 52%, L= 40% Platelet- 1,50,000/cmm Hct – 57.6% PBF – Anisopoikilocytosis with target cell IT Ratio- 0.09 CRP- 30.6 Blood CS No growth Electrolyte Na 135 137 K 4.2 4.3 CL 95 100 T-CO2 31.5 24.8 S. Calcium 8.9mg/dl S. Creatinine 0.79 0.6 Mg 1.6 mg/dL
  • 37. Management after admission (8PM) • O2 inhalation • OG feeding 10ml/2hrly • Infusion 10% DBS • Inj Meropenem • Inj Vancomycin • Inj Frusemide • Inj Digoxin • Thermal care
  • 38. Follow up at 10PM (04.02.2019)  O/E:  HR 280-290/min  BP- 83/46(62)  RR -56/min  CRT – 2 sec  Heart - S1+S2+ M  Lungs – BL equal air entry, no added sound  Assesssment: tachyarrythmia most probably SVT  Plan: Continuous ECG monitoring S- 72-82 D- 46-54 M- 55-63
  • 40. Cont… Vagal stimulation by Ice cold compression on face Persisting Tachycardia (HR-270-280) Plan: Cardiology consultation Inj Adenosine
  • 41. Follow Up: 05.02.19 at 9AM Subjective Objective Assessment Plan & Measures Taken Tachycardia persisting Pink in 2L/min O2 Reflex activity- good CRT -2 sec R/R – 54 br/min H/R- 280b/min BP- 83/46(62) SPO2- 98% in 2L/min O2 Lung- B/L equal air entry Heart- S1+S2+ M Abdomen- soft, not distended, liver 2cm Umbilicus- Healthy CBG- 6 mmol/L Temp- 98°F Urine- 2.7 ml/kg/hr Meconium- passed Stable S-72-82 D-46-54 M-55-63 • Cardiology consultation • Inj Adenosine • Echocardiography Give Inj Adenosine HR -150-156/M
  • 42. Echocardiography  Transposition of great artery (d-TGA), PA arising from LV, Aorta arising from RV. Large inlet VSD (Size-6.8mm) with bidirectional shunt. Large ostium secundum ASD (size- 7.5mm) with L-R shunt. Large PDA with bidirectional shunt. Both dynamic and fixed obstruction of LVOT. Dilated coronary sinus. Myxomatous change to septal leaflet ant leaflet of tricuspid valve
  • 43. Follow up on 06.02.2019 At 3.30AM HR- 288/M -Ensure Digoxin maintainance dose At 9pm HR- 250-260 b/m MX- Inj Adenosine HR-120-130b/min
  • 44. Club foot Mx(Orthopedic consultation)  Type: Flexible variety of Congenital Talepes Equino- varus (Bilateral)  Plan:  Manipulation  Follow up & Serial plaster after 1 month
  • 45. Follow Up: 07.02.19 at 10AM Subjective Objective Assessment Plan & Measures Taken Unusual weight gain (80gm) Pink in air SPO2- 90% in air Reflex activity- good Fontanelle- Open, normal CRT -2 sec R/R – 58 br/min H/R- 120-130 b/min BP- 92/53(63) Lung- B/L equal air entry Heart- S1+S2+ M Abdomen- soft, not distended, liver 2cm Umbilicus- Healthy CBG- 6 mmol/L Temp- 98°F Urine- 2.5 ml/kg/hr Stool- 4 times Static S-72-82 D-46-54 M-55-63 -Decreased fluid -Increased feed -Inj MgSO2 (Mg -0.6) • At 6PM, 10PM SVT develop > Spontaneous resolved
  • 46. Follow Up: 09.02.19 at 10AM Subjective Objective Assessment Plan & Measures Taken Repeated episode of SVT Pink in air Reflex activity- good Fontanelle- Open, normal CRT -2 sec R/R – 42 br/min H/R- 120 b/min BP- 92/53(63) SPO2- 84% in air Lung- B/L equal air entry Heart- S1+S2+ M Abdomen- soft, not distended, liver 2cm Umbilicus- Healthy CBG- 6 mmol/L Temp- 98°F Urine- 2.5 ml/kg/hr Stool- 4 times Static S-72-82 D-46-54 M-55-63 • Cardiac Consultation • Tab Amiodarone (5mg/kg/day) started from 5PM • At 8.30PM & 10.30PM Baby developed SVT (spontaneously resolved)
  • 47. Follow Up: 10.02.19, 1AM  Subjective- Sudden bradycardia (HR-70/min), Desaturation (SPO2-74%)  Objective-  Pink in air  Reflex activity-poor  CRT - 4 sec  R/R – Irregular  H/R- gradually decreased to 50/m  Pulse volume - weak  Heart – S1+S2+M  Assessment : Cardiac arrest
  • 48.  Management : Positioning, suction, stimulation and bag mask ventilation , CPR started 2 shot of Inj Adrenaline was given (10unit/kg) Baby having gasping respiration, heart rate 90b/min Mechanical ventilation with SIMV mode in following method - Rate-40, Pressure – 14/5, FiO2- 60% - Dopamin, Dobutamine were added
  • 49.  After that  Pink in air  Reflex activity-Improving than previous  CRT - 2 sec  R/R – 45/m  H/R- 132/m  Pulse volume – good  Lungs – bilateral good air entry  Heart – S1+S2+M  Pupil – reacting to light  Plan : Chest X-ray ABG ABG: pH-7.37 PCO2- 34 PO2- 45 HCO3- 19.4 BE- -5
  • 50.
  • 51. Follow Up: 10.02.19 at 10AM Subjective Objective Assessment Plan & Measures Taken One episode of SVT at 4.30AM (Mx by Tab. Amiodarone) Pink in MV F-40, P-14/5,Fio2-.60% SPO2- 93% Reflex activity- Moderate Fontanelle- Open, normal CRT -2 sec R/R – 42 br/min H/R- 140 b/min BP- 97/63(75) Lung- B/L equal air entry Heart- S1+S2+ M Abdomen- Distended, liver 3cm Girth-29cm CBG- 9 mmol/L Temp- 98°F Urine- 3.2 ml/kg/hr Stool- 3 times Static S-72-82 D-46-54 M-55-63 • Decreased setup FiO2 50% PIP 12 • Weaning off Dopamin • Decreased 10% to 7.5% dextrose • Send septic workup, S. Electrolyte (Refused) At 9PM wean from MV to 5L/M o2
  • 52. Follow Up: 11.02.19 at 10AM Subjective Objective Assessment Plan & Measures Taken One episode of SVT at 10.30PM (Mx by Inj Adenosine) Pink in 3L O2 SPO2- 93% Reflex activity- Moderate Fontanelle- Open, normal CRT -2 sec Temp- 37°c R/R – 52 br/min H/R- 240 b/min BP- 88/58(67) Lung- B/L equal air entry Heart- S1+S2+ M Abdomen- Distended, liver 3cm Girth-29cm CBG-6.3 Urine- 3.2 ml/kg/hr Stool- 3 times Static S-72-82 D-46-54 M-55-63 • Referred to NHF
  • 53. Final diagnosis Term (38+4 wks), low birth weight (2300gm), AGA, IDM, Perinatal asphyxia (HIE-1), Congenital heart disease (d-TGA, Large VSD, Large ASD, Large PDA), Supraventricular tachycardia, multiple congenital anomalies (right sided anotia, right sided lower motor type facial palsy, bilateral club foot)
  • 54.
  • 55. Our patient S/O Tania A baby with multiple congenital malformations (unilateral anotia, facial nerve palsy, bilateral club foot and congenital heart disease) Presented with tachycardia Heart failure Sepsis Excluded
  • 56. ECG monitoring Tachyarrhythmia Most common cause of tachyarrhythmia in newborn Supraventricular tachycardia (SVT)
  • 58. SVT • An abnormal arrhythmia arising above or within bundle of His. • It includes essentially all forms of paroxysmal or incessant tachycardia except ventricular tachycardia. • It is the most common abnormal tachycardia in infancy and childhood.
  • 59. TYPES SITE RHYTHM Sinus node Sinus tachycardia Always secondary; usually non-cardiac cause e.g. sepsis, fever, pain, hypovolaemia, respiratory failure, anaemia, fluid overload, ionotropes Atrium Atrial flutter Atrial fibrillation Atrial tachycardia Rare Very rare Uncommon AV junction AV re-entry tachycardia AV nodal re-entry tachycardia Commonest form in newborn rare Below AV node His bundle tachycardia (junctional ectopic tachycardia) Associated with abnormal hearts/cardiomyopathy; Usually postoperative
  • 60. RISK FACTORS  Bimodal age distribution • One peak at infancy • Another peak is in between 8-12 years (Calabro ` MP, Cerrito M, Luzza F, Oreto G (2008) Supraventricular tachycardia in infants: Epidemiology and clinical management. Curr Pharm Des 14(8):723– 728 )  No significant gender differences were present at younger ages ( Anand RG, Rosenthal GL, Van Hare GF, Snyder CS (2009) Is the mechanism of supraventricular tachycardia in pediatrics influenced by age, gender or ethnicity? Congenit Heart Dis 4(6):464–468) Our patient:  presented in neonatal period  Male baby
  • 61. PRESENTATION Antenatal: • Fetal SVT (fetal tachycardia, less fetal movement) • Non- immune hydrops fetalis, if started early in pregnancy
  • 62. Post natal: Asymptomatic Tachycardia (HR: 180-300/min) • Abrupt onset and cessation • May occur during rest • May be precipitated by acute infection • Attacks may persist from seconds to hours Heart failure If tachycardia goes unrecognized for a long time Shock Our patient: Tachycardia with Sudden onset & spontaneous remission; Some episodes occurred during sleep; persisted for minutes to hours
  • 63. Causes of SVT • Any form of structural heart disease • Myocardial tumors • Myocarditis • Electrolyte disturbances • Indwelling right arterial lines In our patient: Congenital heart defect (murmur+)
  • 64. ASSOCIATED CARDIAC DEFECTS/SYNDROME According to Tripathy A, Black GB (2014) Factors associated with the occurrence and treatment of supraventricular tachycardia in a pediatric congenital heart diseaes cohort. Pediatr cardiol 35:368- 373 • 6–7 % of children with CHD have SVT • ASD secundum (14.3 %) • VSD (6 %) • Pulmonary stenosis (5.4 %) • PDA (5.3 %) • Pulmonary hypertension (2.4 %)
  • 65. Continued…. • Two or more concomitant CHDs were present in 8.6 % of SVT patients. • A smaller percentage (7.2 %) of patients were diagnosed with severe/complex (cyanotic) CHDs, i.e., tetralogy of Fallot, transposition of the great arteries, double-outlet right ventricle (DORV), common ventricle, truncus arteriosus, atrioventricular canal/endocardial cushion defect (AV/ ECD), Ebstein’s anomaly, hypoplastic left heart syndrome (HLHS), or total anomalous pulmonary venous return
  • 66. Our patient had associated CHD • TGA • A Large inlet VSD (6.8 mm) • A Large ostium secundum ASD (7.5 mm) • A Large PDA • Posterior deviation of conal septum producing dynamic Left Ventricular Outflow Tract Obstruction
  • 67. This patient also had some other congenital anomalies • Unilateral anotia • Facial nerve palsy • Bilateral club foot There was no reported case of SVT found to be associated with these congenital malformations
  • 71.
  • 72. PRINCIPLES OF MANAGEMENT Conversion of rhythm: Vagal maneuvers: Ice pack to the face until conversion or max. 10 seconds Adenosine, if vagal maneuver fails Control of rate: Propranolol (if there are no signs of heart failure) Digoxin Amiodarone
  • 73. Management of emergency/complication: Digoxin (if there are signs of heart failure or propranolol has failed) after ensuring not hypokalaemic and no WPW syndrome Electrical cardioversion by synchronous DC cardioversion: if the baby is in shock
  • 74. Maintenance therapy: Once sinus rhythm is restored, maintenance therapy with whatever agent restored, is usually given for 3- 6 months Surgical correction Artificial pacing Radiofrequency catheter ablation
  • 75. Our patient was treated with Vagal maneuver: • Ice pack to the face Inj. Adenosine Inj. Digoxin Tab. Amiodarone
  • 76. How Adenosine was given  Dose: 0.1 mg/kg/dose  Prerequisite for giving Inj. Adenosine: • A large fresh cannula as close to heart as possible • 3-way channel  Procedure: rapid IV bolus followed by saline flush with continuous ECG recording
  • 77.
  • 78. COMPLICATION • Heart failure • Shock • Cardiac arrest • Myocardial dysfunction • Mitral regurgitation • Dilated cardiomyopathy Our patient: Cardiac arrest
  • 79. PROGNOSIS • Usually excellent, in absence of structural cardiac disease or cardiomyopathy • WPW syndrome has small risk of sudden cardiac death • One fifth will recur after treatment • When SVT starts in first months of life, it resolves in 80% cases within the first year; If SVT starts later in life, spontaneous remission may occur in only 15-20% cases (Vignati G, Annoni G (2008) Characterization of supraventricular tachycardia in infants: Clinical and instrumental diagnosis. Curr Pharm Des 14(8):729–735) Our patient: Prognosis is guarded
  • 80. FOLLOW UP Clinical Investigations: • Digoxin level • CBC • Electrolytes • ECG