Transcript CYANOSIS - cardiologycmc.in

APPROACH TO CYANOSIS
Definition
Bluish discolouration of skin or
mucous membrane caused by
excess amounts of reduced
hemoglobin or abnormal
hemoglobin
4gm of reduced Hb in capillaries
required for cyanosis to be apparent
Mechanism
caused by absolute increase in reduced Hb,higher the Hb –
greater tendancy towards cyanosis
25
20
20
15
15
TOTAL Hb
RED-Hb
10
5
5
5
NORMAL
POLYCYTHEMIA
5
5
0
ANEMIA
• In severe anemia , greater systemic arterial
desaturation required for cyanosis to be
evident
• In polycythemia even lesser systemic arterial
oxygen saturation may result in clinical
cyanosis
• If fetal Hb is high, tissue hypoxia may occur
even if cyanosis is mild( arterial PaO2 low)
central
peripheral
CAUSE
ARTERIAL BLOOD
DESATURATION OR
ABNORMAL Hb
CUTANEOUS
VASOCONSTRICTION DUE
TO LOWCO
CONDITIONS
Seen in R-L shunt,
impaired pulmonary
function, abnormal Hb
exposure to cold air or
water and abnormally
greater extraction ofO2
from normally saturated
blood
SITES
conjunctiva,palate,tongue,i limited to ears,nose,cheeks
nner side of lips& cheeks
outer side of lips hands
feet&digits
certainly central if
associated with clubbing
and polycythemia,
probably central if it
deepens on effort
clubbing is absent
CARDIOVASCULAR
DUCT-INDEPENDENT
MIXING LESIONS
TGA, TAPVC,TA
DUCT-DEPENDANT PBF
TOF,EBSTEINS,TricusidAtre
DUCT-Dependant SBF
HLHS,IAA,CoA,Critical AS
L-RSHUNT &PUL EDEMA
SV States
PRIMARY LUNG DISEASE
AIRWAY OBSTRUCTION
EXTRINSIC LUNG COMPRESSION
PULMONARY AV MALFORMATION
PPHN
CNS DYSFUNCTION
HEMATOLOGIC
MISCELLANEOUS
HYPOGLYCEMIA,
METABOLICACIDOSIS
SEPSIS,
HYPOTHERMIA,
SHOCK
Diagnostic ladder
Clinical evaluation
Physical examination
History
Simple lab investigation
PO, CXray & ECG
Hb conc &peripheral blood filim
Echo
Invasive cardiac evaluation
•
MATERNAL HISTORY
DIABETES- TTN,RDS,HYPOGLYCEMIA
ASTHMA -TTN
POLYHYDRAMNIOS - TEF
PIH – IUGR,POLYCYTHEMIA,HYPOGLYCEMIA
LABOUR& DELIVERY
PROM –SEPSIS,PNEUMONIA
CHORIOAMNIONITIS- SEPSIS
C-SECTION- TTN,RDS,PPHN
NEWBORN
ONSET AT BIRTH- TTN,RDS,MAS,CDH,
ONSET –HRS AFTER BIRTH- CCHD,aspiration,TEF
CLUES BASED ON ONSET
1st week
D TGA
Pulmonary Atresia
Tricuspid atresia
Ebstein
Critical PS
> 1 week
TOF TGA
Admixture lesions
TAPVC
SV
DORV
Truncus
Examined in neutral thermal enviornment
Away from blue phototherapy lights
Asses capillary refill time- <2 sec
Barrel shaped chest –post term-MAS
Bell shaped thorax – neurologic abnormalities
Scaphoid abdomen-CDH
Look for nasal flaring,grunting & retractions
Palpate brachial & femoral pulses
BP- all four extrimities
SBP-gradient quite specific for arch abnormality
Not sensitive
S2- split in 80% by 48 hours
Differential cyanosis
CCHD in Newborns:
Clues based on presentation
Cyanosis
No Resp Distress
TGA
DDPC
Cyanosis
+
Resp Distress
TAPVC
obstructed
Shock
Differential
cyanosis
DDSC
CCHD in Newborns:
Clues Based on S2 split
S2
normal
single
fixed
DDPC
TGA
DDSC
TAPVC
Excludes
Cardiac
cause
CCHD in newborns:
Clues based on Murmurs
• Murmurs have poor sensitivity( < 50%)
No Murmur
TAPVC with obstruction
DDPC( closed PDA)
ESM
TGA, DDPC, DDSC
Continuous murmurs DDPC with collaterals
(are)
Diastolic murmur
(to & fro)
TOF- absent pulmonary
valve
cardiac
respiratory
HEART RATE
FASTER
FAST
RESPIRATORY RATE
FAST
FASTER
GRUNT
ABSENT
PRESENT
MURMUR
PROMINENT
CAN BE PRESENT
CHF
PRESENT
LESSLIKELY
ABNORMAL PULSE
YES
NO
RESPONSE TO O2
NOT MUCH
GOOD
Pulse oximetry
Standard of care for all infants with respiratory
distress & cyanosis
Accurate& reliable method of monitoring o2
saturation in infants noninvasively
Pulse oximeter probes on R hand & lower
extremity
Aim for 02 saturation of 90-95% by pulse
oximetry
PPHN- suspected –aim for higher o2 saturation
HYPEROXIA TEST
• ADMINISTER 100% O2 FOR 15 MINUTES
• ASSES O2 OF UPPER LIMB LOWER LIMB
• ABG
• TCMO
• PO
-
YES
YES
NO
HYPEROXIA TEST
GIVE 100% O2
ASSES PO2
PO2>200
PO2<150
NO CCHD
LIKELY CCHD
PASS
FAIL
150-200
?CCHD WITH PBF OR PPHN
PAO2 <50MM
C X RAY
CARDIOMEGALY
NO CARDIOMEGALY
PULMONARY VASCULARITY
EBSTEINS
PBF
PUL EDEMA
PBF
TGA + IVS
TAPVR With OBSTRN TA with PA orPS
PA WITH IVS
CRITICAL PS
TOF & TOF + PA
Those with decreased PBF &normal or slightly
increased heart size differentiated by there
QRS axis on ECG & MURMUR + or –
TA with PS or PA – SUPERIOR QRS AXIS ( 0 to-90)
Critical PS & PA with IVS- 0 to 90 degree
Differentiated by loud pul ejection murmur
TOF
&
TOF with PA - QRS 90 to 180
Pulmonary
continuous murmur
Stenosis murmur
Prostaglandin (PGE1) Infusion
Neonates –fail hyperoxia test
High
Signs& symptoms of CHD or
likelihood
Present in shock within 1st 3 wk of life of CHD
PGE1 administration –open ductus arteriosus
Depending on lesion - PBF or SBF or improves
Intercirculatory mixing- improves hypoxemia
&metabolic acidosis
Neonate with shock or CHF in 1st few weeks of life ductdependant SBF untilproven otherwise
• PG sensitive lesions:
Cyanosis + murmur or
mild/no cyanosis + abnormal pulses
• Can be withheld in a relatively stable child
( SaO2 > 70%; no acidosis)
• Target SaO2 >80%; pO2 around 45-50, normal
pH
• Once diagnosis is confirmed, it is ideal to start
PGE1 before transport.
WORSENING AFTER PGE1 – obstruction to blood flow out of pulmonary veins
• Always given as continous IV infusion.
• Start at 0.05-0.1μg/kg/min, can be reduced to
0.005 - 0.01µg/kg/min once duct is opened( ^
SaO2)
• Available as 500 μg vial
• Trade name: Alpostin/Prostin
• Cost: Rs 5000/- per ampoule
• One vial will last 2-3 days for a 3Kg baby
• Efficacy  with  age, less effective after 2
weeks of life, not effective after 4 weeks
• Adverse reactions more common in
premature& LBW infants
• Apnea –typically in 1st few hrs ,tachycardia,
bradycardia, fever, NEC, seizures,
thrombocytopenia,
• Continous cardiorespiratory monitoring
• PGE1 – peripheral vasodilation –
hypotension& cutaneous flushing
Separate IV line should be secured
Hypotension treated by
10-20ml/kg bolus of NS,RL,5%albumin
Remeasure ABG,reasses capillary refill&
vitalsigns within 15 to 30 min of starting PGE1
infusion
Principles of managment
Intial stabilisation – airway management
reliable venous access – umblical vein
Arterial line to monitor BP,acid-base,o2
Volume resucitation,inotropic support &
correction of metabolic acidosis
Blood glucose & sepsis workup-cyanosis+circ
collpse
Non invasive delineation of anatomic defectECHO
Evaluation & treatment of additional organ
system-pulmonary,renal,hepatic&CNS
Evaluation of additional congenital defects
Genetic evaluation – if indicated
Cardiac Cathetrisation
Surgical managment
• CCHD is an important differential diagnosis in
neonate presenting with cyanosis after birth.
• Clinical evaluation with CXR and Hyperoxia test
excludes CHD in most cases.
• Echocardiography recommended in all doubtful
cases.
• Prior stabilization and a monitored transport to
tertiary center ensures a optimal pre-operative
state.
• Early intervention with very encouraging results is
realistic for most forms of critical CHD in
newborns
r
CCHD derived from heterogeneous group of
conditions
May have pulmonic stenosis ,PAHor NL pulm
pressure with out PS
PBF may be NL,INCREASED or DECREASED
Decreased PBF may be due to Pulmonic stenosis or
PAH
Anomalies where free mixing of systemic and
venous blood occurs severity of cyanosis
determined by pulmonary blood flow
Thymus regresses very fast in cyanotic patients
CCHD
With
out
PS
With
PS
With
VSD
NO
VSD
PBF
PA
pressu
re NL
PBF
PA
pressure
elevated
PULM
VENOUS
OBSTRN
This classification result in six sub group of
cyanotic patients
PS without VSD
PS + large VSD
Increased PBF with or without PAH(transposition
physiology
PAH withdecreased PBF(Eisenmengerphisiology)
PAHdue to pul venous obstruction
NL or MILD elevated pul pressure without PS
PS without VSD
(cyanosis due to R toL shunt at atrial level&
cardiomegaly)
Triad-cyanosis,cardiomegaly,ischemic lung fields on
Cxray
Dominant a in JVP
Cardiomegaly
Parasternalimpulse
Widely split 2nd HS p2 late &soft
3rd &4th HS
Pulm ejection systolic murmur,TR murmur
Severe or critical pure PS with failing RV,Ebstein’s
PS with VSD (Fallot’s physiology)
Prominent a wave in jvp
NL heart size
Mild parasternal impulse
Systolic thrill uncommon
Single 2nd sound(widely split with inaudible
pulmonic sound)
ESM
Clear diastolic period
Ischemic lungs in xray without cardiomegaly
D/D of fallots physiology
Fallot’stetrology(commonest >2yrs )
TGA
TRICUSPID ATRESIA
SV
DORV
correctedTGA
AVCD
If no rvh on ecg;posssibilities-rv hypoplastic
&small,rv absent,PA not connected to RV
Tricuspid atresia,hypoplasticRV with or without
straddling TV, & single ventricle
Increased pulmonary blood flow with or without
PAH(transposition physiology)
Symptomatic in Neonate
Cyanosis-mild to severe
Failure to thrive& gain weight
CCF
Cardiomegaly –in2-3 wks of life
2nd sound single,s3 gallop,insignificant systolic
murmur
Cardiomegaly with Incrs pulm vasculatureon xray&
thymicshadow absent
Anomalies with cyanosis and increased PBF
TGA
DORV without PS
TA with Incrs PBF (largeVSD+ NO PS)
Persistent Truncus
SV without PS
TAPVC
Cyanosis with PAH and Diminished PBF
(eisenmenger- defnd as nonreactive PAH
resulting in a R to L shunt at atrial,ventricular or
great artery level)
Characteristics
H/O frequent chest infections in infancy
Cyanosis present from birth and appear late
Jvp-prominent a wave
No cardiomegaly or thrill(except when shunt at
atrial level)
No PSH
Constant EC of PAH
2nd sound is palpable,pulmonary component
accentuated
Systolic murmur in pulmonary area is
insignificant or absent
Pulmonary and/or TR murmurs may be present
EISENMENGER SYNDROME-DIFFERENTIATION
ASD
VSD
PDA
CYANOSIS
UNIFORM
UNIFORM
DIFFERENTIAL
CARDIOMEGALY
PRESENT
ABSENT
ABSENT
PARASTERNAL IMPULSE
HEAVING
MILD
MILD
2nd SOUND
WIDE FIXED
SPLIT
SINGLE
NORMALLY
SPLIT
TR
COMMON
RARE
RARE
ASC AORTA IN C Xray
NORMAL
NORMAL
LARGE
PAH-DIFFERENTIATION
HYPERKINETIC
OBSTRUCTIVE
HEART SIZE
LARGE
NORMAL(except ASD)
PARASTERNAL IMPULSE
HYPERKINETIC
FORCIBLE &HEAVING-ASD,
MILD IN VSD&PDA
CLICK OF PAH
ABSENT
PRESENT
2nd SOUND(P2accentuated in
both)
ASD-WIDE&FIXED,VSDWIDE&VARIABLE,PDAPARADOXICALLY SPLIT
ASD-WIDEFIXED
VSD-SINGLE
PDA-NORMAL
Shunt murmur
LOUD
SHORT OR ABSENT
Flow murmur
PRESENT
ABSENT
Patients with PAH due to Pulmonary Venous
hypertension(HLHS&TAPVC with obstruction)
Generally present in neonatal period
Severe cyanosis,CHF,S3 gallop,no
cardiomegaly,absence of significant murmurs,
Cxray –NL sized heart with severe PVHcausing GROUND GLASS appearance
2d echo- mitralatresia,aortic atresia,pulmonary
venous obstruction, hypoplastic LV
Cyanosis without PS & PA pressure normal
Heterogeneous group of anomalies
TAPVC- features of 2 ASD but with
cyanosis,figure of 8
Single atrium-mostly associated with polysplenia
SVC entering LA
Pulmonary AV fistula
Cyanosis
+diagnos
tic
approac
h
Booming p2
Cardiomegaly
Dex
troc
ardi
a
Dext
roca
rdia
com
plex
Levoc
ardia
Prominent
pulmonary
conus
Poor femoral
pulse&
With
viscer
al
Large brachiopoplipteal
situsin
versus
Systolic
pressure
gradient
Peripheral
vascular
pruning
Large&r
elativel
y silent
heart
Huge
RA
oligemi
c lung
fields
Giant p
wave
Syndrome
of
Levocardi
a with
Visceral
situs
inversus
Coarctation
syndrome
Ebstein’s
anomaly
Eisenmein
ger
syndrome
Prescence
of Howelljolly bodies
Asplenia
syndrome
Abnormal rhythm& CHD
• L- TGA
- Heart Block ; SVT
• Ebstein - SVT
Cyanosis&RVH
NL axis/RAD
Plethora
TGA
TAPVC
DORV
Oligemia
TOF
PA
PS+ASD&/OR
VSD
PS+TGA/DORV/
DOLV