Cyanotic Congenital Heart Disease

1

• Cyanotic Congenital Heart Disease
• Awni Al- Madani., MD
• FSCAI, FACC

2
CYANOSIS IN CHILDREN

• Central cyanosis
• Cyanosis of the tongue,mucous membranes and
  peripheral skin, it is necessary to have gt3g/dl
  of reduced Hb to have it.
• Peripheral cyanosis
• It is visible only in the skin of the extremities
  with normal arterial saturation due to vasomotor
  instability,ex. cold environment.

3
CAUSES OF CENTRAL CYANOSIS

• Congenital Heart Disease
•  
•     1) Cyanosis with PBF 2) Cyanosis
  with PBF
• a)      TOF
  a) D-TGA
• b)      Pulm. Atresia b)
  DORV
• c)      Tricuspid Atresia c)
  TAPVC
• d)      Critical PS
  d) Truncus arteriosus
•  

4
CAUSES OF CENTRAL CYANOSIS

• B) LUNG DISEASE D) CNS
  DEPRESSION
•  
• a)      RDS
  a) IVH
• b)      Pneumonia b)
  Perinatal asphyxia
• c)      Pneumothorax c)
  Heavy maternal sedation
• d)      Pleural effusion
• e)      Diaphragmatic hernia
• f)       T.E.Fistula
•  C) PERSISTENT PULMONARY E) MISCELLANOUS
• HYPERTENSION
• 
  a) shock sepsis
• 
  b) Hypoglycemia
• (PFC syndrome)
  c) Methemoglobinemia
• 
  d) Neuromuscular conditions
• 
  ( Werdnig Hoffman)

5
RADIOLOGICAL FEATURES

• CXR may exclude non cardiac causes of cyanosis
  e.g. RDS. . Meconium aspiration, Diaphgramatic
  hernia, Pneumothorax
• Pulmonary Vascular Markings
• Decreased
  Increased
• Heart Size
  Heart Size
• Normal Increased
  Increased
• ( Boot shaped) ( Wall-to-Wall)
  
• TOF Ebstein
  ( egg-on-end)
• 
  D-TGA
• Aortic Arch \ Mediastinum
  Abdominal Situs

6
ECG

• RV dominace on ECG is normal in the newborn.
•  
• Left axis deviation with LVH strongly suggests
• Tricuspid Atresia.
• Left axis deviation in a newborn may also
  indicate
• AV canal
• ( However , AV canal is usually an
  acyanotic
• form of heart disease).
•  

7
Blood gases response to 100 O2

• Always try to obtain ABG from RIGHT radial
  artery.
• Low PH may indicate sepsis, circulatory shock or
  
• severe hypoxia
•  
• High pCO 2 may indicate CNS or pulmonary
  disease.
•  
• Hyperoxia Test 100 O2 by hood for 10 minutes.
•  
• pO2 gt 150 torr pulmonary disease.
• pO2 lt 100 torrcyanotic heart disease

8
Transposition of the great arteries ( TGA)

• ? ASD ? VSD? PS
• PE
• Single accent. S2
• VSD murmur
• CXR
• (egg on a string)
• Increase CTR
• Increase PVM
• ECG
• RVH

9
TGA Management

• Medical
• PGE1
• O2 (3L/minute)
• Correct
• acidosis ,hypoglycemia.
• electrolyte disturbances.
• Transcatheter
• BAS
• Surgical
• Arterial switch (Jatene operation)
• at 7-15 days
• Atrial switch ( Senning operation)
• at 6-9 months

10
Tetralogy of Fallot

• ? Degree of PS
• Physical examination
• Single accent. S2
• ESM
•  
• CXR CTR normal
• Decrease PVM
•  
• ECG RVH

11
Cyanotic Spells

• Spasm of decrease SVR
  crying
• RVOT
•  
• Increase R..L shunting
• Increase systemic venous return
  DecreaseO2
  
  
  Increase CO2
  
  
  Decrease pH
  
  
• Tachypneea
•  

12
Cyanotic Spells

• Increase systemic vascular resistance
• 
  Squat/Knee chest
  position
• 
  Ketamine 1-2mg/kg
  IV
• 
  Neosynephrine
  0.02mg/kg IV
• 
  
• Tachycardia
  Propranolol 0.1mg/ Kg IV
• Release of infundibular spasm
  
•  
• Irritability Morphine 0.2mg/ Kg  
  S.C or IM
• Hypoxia
  Oxygen
•  
• Dehydration
  Volume
• Acidosis
  NaHco3 1mEq/ Kg IV

13
TOF management

• Medical
• Correct iron deficiency anemia
• Correct polycythemia
• B-Blocker
• Surgical
• Palliative Blalock-Taussig shunt
• for small PAs
• Definitive Total correction

14
Pulmonary Atresia/VSD

• ? PDA or Bronchial collaterals
• ? PAs size? Confluent or not.
• PE
• Cyanosis at birth
• S2 single
• No murmur
• CXR ( Like TOF)
• Normal CTR/ ?PVM
• ECG
• RAD/RVH

15
Pulmonary Atresia/VSD Management

• Medical ? PDA dependent
• PGE1
• Surgical
• Palliativeshunt
• Definitive staged repair (Unifocalization)
• RV-PA conduit

16
Pulmonary atresia with intact septum

• RV size? Coronary sinusoids present or not.
• PE
• Severe cyanosis/ tachypnea
• S2 single
• No murmur
• CXR
• RAE
• Decrease PVM
• ECG
• RAE, LVH

17
Pulmonary atresia with intact septum

• Medical
• PGE1 Radiofrequency perforation of PV.
• Surgical
• Open pulmonary valvotomy palliative
  Blallock-shunt
• (in the neonatal period).
• If good RV size Biventricular repair (at 1
  year of age).
• If small RV size sinusoids RVDCC
• BT- shunt in neonatal period
• Bidirectinol Glenn (at 6 months of age).
• Total Cavo Pulmonary Connection (TCPC)
• (at 4 years of age)

18
Ticuspid Atresia

• ? PS ? TGA? VSD
• PE
• Single S2
• VSD murmur
• ? CHF
• ECG
• - Superior QRS axis
• - RAE LVH

19
Tricuspid Atresia Management

• Medical
• Inadequate pulmonary flow
• PGE1
• BAS ( balloon atrial septostomy )
• Increase pulmonary blood flow (e.g.sizable VSD)
• Pulmonary artery banding
• Surgical
• Palliative shunt
• Blalock-Taussig shunt
• Bidirectional Glenn
• Definitive repair
• TCPC ( Total Cavo Pulmonary
  Connection)

20
Total Anomalous Pulmonary Venous Connection (
TAPVC)

• ? Site ? Obstructed
• PE
• ( Large ASD)
• CHF
• Wide split S2
• ESM
• Diastolic rumble( overflow at TV)
• CXR
• (snow man appearance)
• Increase CTR
• Increase PVM
• ECG
• RAE
• RVH

21
Total Anomalous Pulmonary Venous Connection (
TAPVC)
22
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC, supracardiac via vertical vein
23
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC, to coronary sinus
24
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC, Infradiaphragmatic
25
Treatment

• Correct acidosis
• Antifailure
• Surgery
• Anastomosis of
• Common Pulmonry Vein to the left atrium

26
Persistent Truncus Arteriosus

• Increase pulm. blood flow
• Increase pulm. vascular resistance
• PE
• RV tap
• S2 single
• Ejection click
• Diastolic murmur
• CXR
• Increase CTR
• Increae PVM
• ECG
• RVH
•  

27
Persistent Truncus Arteriosus

• Medical
• Antifailure therapy
• Surgical
• Wait till PVR drops
• Total correction at 2-3 months

28
Ebstein Anomaly

• ? PS
• PE
• Mild to severe cyanosis
• S2 wide split
• TR murmur
• Hepatomegaly
•  CXR
• Balloon shape
• Increase CTR
• Decrease PVM
• ECG
• RAE
• RBBB
• WPW? SVT?

29
Ebstein Anomaly

• 2D-Echo-Doppler

30
Ebstein management

• Medical
• Mild nothing
• CHF Diuretics
• Severe cyanosis
• PGE1
• SVT Inderal
• Surgical
• Palliative shunt
• Definitive repair
• If good RV size Danielson repair
• Carpentier repair
• If small RV----- Univentricular repair strategy

31
Single Ventricle

• ?Pulm. Blood flow
• No PS
• Lt-? Rt shunt
• Like large VSD
•  
• ?Pulm. Blood flow
• PS
• Rt-?Lt shunt
• Like Fallot

32
Single Ventricle Management

• With ?pulm. Blood flow
• Medical PGE1
• Surgical
•  
• - Blalock shunt
• - Bidirectional Glenn
• - Total Cavo Pulmonary Connection
• (TCPC)

33

• Thank You

34
(No Transcript)
35
Precordial Activity

• Visible precordial impulse
• Prominent feature of
• D-TGA
• TAPVR
• Vein of Galen
  aneurysm
•  
• RV impulse not palpable
• Tricuspid
  atresia
•  
• Thrill
• Only cyanotic newborn with
  a thrill Tricuspid atresia
•  

36
AUSCULTATION

• HEART SOUNDS
•  
• S1 is normally accentuated in newborns
•  
• S2 split is normally heard as a slurring rather
  than a distinct split.
• S2 is single in many cyanotic lesions especially
  in, D-TGA, TOF.
•  
• S2 is widely split in TAPVR, Critical PS.
•  

37
MURMURS

• SYSTOLIC EJECTION MURMURS
• May be heard in the first hours of life.
• Usually due to ventricular obstruction e.g.
  AS., PS., TOF
•  
• DIASTOLIC MURMURS
• Rarely heard in newborns
• Early diastolic murmurs heard in Truncus
  arteriosus ,
• TOF with absent pulmonary valve
•  
• Continuous
• Continuous murmurs are caused by AV fistulas
• (not PDA)
• ABSENT
• Silent hearts often characteristic of
  Tricuspid atresia , Pulmonary atresia D-TGA.
•  

38
EJECTIONS CLICKS

• Deformity of semilunar valves
• 1) Pulmonary PS
• 2) Aortic Critical
  AS
• CoA
•  
• Dilatation of great vessel
• 1) PULMONARY ARTERY PS
• 
  HLHS
•  
• 2) ASC. AORTA Truncus
  arteriosus
• 
  TOF

39
Right to Left Shunting(Tetralogy physiology)

• QS gt QP
• Atrial Ventricular Great vessels
  Pulm microcirc.
• (ASD) (VSD)
• Tricuspid Sub PS
  
• Atresia Critical PS
•  

40
USEFUL HINTS

• Large male baby with rapid, shallow abdominal
  breathing
• D-TGA
• Upper body blue, lower body pink seen in
  D-GAPDA.COA
•  
• Only cyanotic newborn who has a thrill
  Tricuspid atresia.
•  
• Ejection click is often heard in Severe PS,
  HLHS
• Systolic ejection murmurs in first hours of life
  TOF, PS, AS
•  
• Silent heart characteristic of D-TGA,
  Pulmonary atresia.
•  
• Pulse oximetry ABG should be obtained from the
  RIGHT arm.
• ECG showing LEFT axis deviation Tricuspid
  atresia

41
Ebstein Anomaly

• ? PS
• PE
• Mild to severe cyanosis
• S2 wide split
• TR murmur
• Hepatomegaly
•  CXR
• Balloon shape
• Increase CTR
• Decrease PVM
• ECG
• RAE
• RBBB
• WPW? SVT?

42
Total Anomalous Pulmonary Venous Connection (
TAPVC)
43
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC, supracardiac via vertical vein
44
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC to coronary sinus
45
Total Anomalous Pulmonary Venous Connection (
TAPVC)
TAPVC, Infradiaphragmatic
46
Persistent Truncus Arteriosus

• Increase pulm. blood flow
• Increase pulm. vascular resistance
• PE
• RV tap
• S2 single
• Ejection click
• Diastolic murmur
• CXR
• Increase CTR
• Increae PVM
• ECG
• RVH
•  

47
Persistent Truncus Arteriosus

• Medical
• Antifailure therapy
• Surgical
• Wait till PVR drops
• Total correction at 2-3 months

48
Total Anomalous Pulmonary Venous Connection (
TAPVC)

• ? Site ? Obstructed
• PE
• ( Large ASD)
• CHF
• Widesplit S2
• ESM
• Diastolic rumble( overflow at TV)
• CXR
• (snow man appearance)
• Increase CTR
• Increase PVM
• ECG
• RAE
• RVH

49
TOF absent pulmonary valve

• PE To fro murmur
• Respiratory symptoms( wheezy chest)
• CXR
• CTR normal
• Dilated PA segments
• Hyperinflated lungs
• ECG RVH
• Management Total correction with reduction
  pulmonary angioplasty  

50
TGA ?? coronaries
51
Double Outlet RV
52
Double Outlet RV

• ? position of VSD ?PS
•  
• Sub aortic VSD no PS like large VSD
• 
  CHF
• Subaortic VSDPS
• 
  like Fallot
• Sub-pulmonic VSD (Tassig-Bing)
•  
• 
  like TGA
• Doubly commited VSD

53
CYANOSIS IN CHILDREN

• Cyanosis depends not only on O2 saturation but
  also on absolute concentration of reduced Hb.
• Fetal Hb Neonates have serious reduction in pO2
  before cyanosis is clinically apparent.
•  DIFFERENTIAL CYANOSIS
• Upper body PINK, lower body BLUE
• PRE-ductal COA ( RV supplying Dao via PDA)
• Upper body Blue, lower Pink
• D-TGA with PDA and COA ( LV supplying Dao via
  PDA).